Residency In-service

Training Exam
Discussion and
Reference Manual
2002 Test Dates • March 1, 2, 3

AMERICAN ACADEMY OF NEUROLOGY

Executive Office
1080 Montreal Avenue
St. Paul, MN 55116
Direct RITE questions to:
Phone: 651-695-2706
Fax: 651-695-2791
E-mail: lwiener@[Link]
 2002 AAN RITE Discussion & Reference Manual

EXAM OBJECTIVES
The Residency In-service Training Examination (RITE) is sponsored by the American Academy of
Neurology (AAN) in order to achieve the following:

* To provide residents the opportunity to assess their knowledge in neurology and

neuroscience;
* To sharpen residents' knowledge base by identifying areas for potential growth;
* To serve as a tool for further education in neurology by providing the references and
discussions for each item.

This examination is not designed to be a certifying or qualifying examination, and its use in that
manner is vigorously discouraged by both the Residency Examination Subcommittee and the AAN
Board of Directors.

FREQUENTLY CITED REFERENCES

Adams RD, Victor M. Principles of neurology. 5th ed. New York: McGraw-Hill Co., 1994.

American Psychiatric Association. Psychiatric diagnosis and the diagnostic and statistical manual of
mental disorders. 4th ed. DSM-IV, September 1997.

Bakshi R, Lindsay BD, Kinkel PR. Brain magnetic resonance imaging in clinical neurology. In: Joynt
RJ, Griggs RC, editors. Clinical neurology. Philadelphia: Lippincott, Williams & Wilkins, 1998.

Benarroch EE, Westmoreland BF, Daube JR, et al. Medical neurosciences - an approach to anatomy,
pathology and physiology by systems and levels. 4th ed. New York: Lippincott, Williams & Wilkins,
1999.

Bradley WG, Daroff RB, Fenichel GM, et al, editors. Neurology in clinical practice. 3rd ed. Boston:
Butterworth-Heinemann, 2000.

Brazis PW, Masdeu JC, Biller J. Localization in clinical neurology. 3rd ed. Boston: Little, Brown,
1996.

Brown WF, Bolton CF, editors. Clinical electromyography, 2nd ed. Boston: Butterworth-Heinemann,
1993.

Chiappa KH. Evoked potentials in clinical medicine. 3rd ed. New York: Lippincott Raven, 1997.

Cummings JL, Benson DF. Dementia: a clinical approach. Boston: Butterworth-Heinemann, 1992.

Dubowitz V. Muscle disorders in childhood. Philadelphia: WB Saunders, Co., 1995.

Feinberg TE, Farah MJ. Behavioral neurology and neuropsychology. New York: McGraw-Hill, 1997.

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Fuller GN, Burger PC. Central nervous system. In: Sternberg SS, editor. Histology for pathologists. 2nd
ed. Philadelphia: Lippincott-Raven, 1997.

Fuller GN, Goodman JC. Practical review of neuropathology. Philadelphia: Lippincott, Williams &
Wilkins, 2001.

Graham DI, Lantos PL. Greenfield's neuropathology. 6th ed. London: Arnold, 1997.

Greenberg JO. Neuroimaging: a companion to Adams and Victor's principles of neurology. New York:
McGraw-Hill, Co., 1999.

Kandel ER, Schwartz JH, Jessel TM. Principles of neural science. 4th ed. New York: McGraw-Hill,
2000.

Kimura J. Electrodiagnosis in disease of nerve and muscle. 3rd ed. New York: Oxford University Press,
2001.

Kleihues P, Cavenee WK, editors. Pathology and genetics of tumors of the nervous system. 2nd ed.
New York: Oxford University Press, 2000.

Menkes JH, Sarnat HB, editors. Child neurology. 6th ed. Philadelphia: Lippincott, Williams & Wilkins,
2000.

Niedermeyer E, Lopes da Silva F. Electroencephalography. 4th ed. Baltimore: Lippincott, Williams &
Wilkins, 1998.

Osborne A. Diagnostic neuroradiology. St. Louis: CV Mosby, Inc., 1994.

Parent A. Carpenter's human neuroanatomy. 9th ed. Baltimore: Williams & Wilkins, 1996.

Pearl PL, Holmes GL. Absence seizures. In: Pellock JM, Dodson WE, Bourgeois BFD, editors.
Pediatric epilepsy: diagnosis and therapy. 2nd ed. New York: Demos, 2001.

Puzantian T, Stimmel G. Review of psychotropic drugs. New York: McMahon, 2001.

Solbrig MV, Healy JF, Jay CA. Infection of the nervous system. A. Bacterial infections. In: Bradley
WG, Daroff RB, Fenichel GM, et al, editors. Neurology in clinical practice. 3rd ed. Boston:
Butterworth-Heinemann, 2000.

Volpe JJ. Neurology of the newborn. 4th ed. Philadelphia: WB Saunders, 2001.

Yock DH. Imaging of CNS disease: a CT and MR teaching file. Baltimore: Mosby, 1991.

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Question(s) 1: Pharmacology/Chemistry Question(s) 4: Behavioral

Discussion: Discussion:
Vigabatrin is an irreversible inhibitor of the Sedation with antidepressants is usually
GABA (gamma-aminobutyric acid) dose dependent with higher doses causing
transaminase, which is especially effective more sedation. Mirtazapine is unique in that
in treatment of infantile spasms. Despite its it has less sedation when a dose greater
favorable pharmacokinetic profile, vigabatrin than 15 mg/d is administered.
has not been approved in the United States
because it produces visual field defects, Reference:
particularly progressive constriction with
Puzantian T, Stimmel G. Review of
nasal sparing. This has been attributed to
psychotropic drugs. New York: McMahon,
selective vulnerability of cells of the inner
2001.
retina that use GABA as a neurotransmitter.
_____________________________
Question(s) 5: Physiology
Reference:
Discussion:
French JA. Vigabatrin. Epilepsia
Electrocerebral silence can be seen in
1999;40(Suppl 5):71S-80S.
patients with a drug overdose or intoxication
_____________________________ who later recover. The recordings should be
Question(s) 2: Neuroradiology done at a sensitivity of 2 micro-V/mm and an
Discussion: impedance of greater than 100 ohms, and
The enhancement described is most using long inter-electrode distances.
characteristic of meningioma. Typically, in
glioblastoma and arteriovenous Reference:
malformation, the enhancement occurs in
American EEG Society guidelines for EEG
the arterial phase and by the venous phase,
recordings, evoked potentials, and
the enhancement has disappeared. The
polysomnography. Journal of Clin
other lesions often do not show tumor blush
Neurophysiol 1994;11:2-110.
on angiography.
_____________________________
Question(s) 6: Anatomy
Reference:
Discussion:
Greenberg JO. Neuroimaging: a companion
The mammillothalamic tract ends in the
to Adams and Victor's principles of
anterior nuclear group of the thalamus.
neurology. New York: McGraw-Hill, Co.,
1999.
_____________________________ Reference:
Question(s) 3: Pharmacology/Chemistry Benarroch EE, Westmoreland BF, Daube
JR, et al. Medical neurosciences - an
Discussion: approach to anatomy, pathology and
Calpain-3 is a muscle specific calcium-
physiology by systems and levels. 4th ed.
activated neutral protease. Mutations
New York: Lippincott, Williams & Wilkins,
producing deficiency of this enzyme produce
1999.
an autosomal recessive form of limb-girdle
_____________________________
muscular dystrophy (LGMD2A). Other
LGMDs are due to dysferlinopathy
(LGMD2B) and sarcoglycanopathies
(LBMD2C-2F).

Reference:
Tsao CY, Mendell JR. The childhood
muscular dystrophies: making order out of a
chaos. Sem Neurol 1999;19:9-23.
_____________________________

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Question(s) 7: Pathology Question(s) 10: Physiology

Discussion: Discussion:
Intraventricular hemorrhage in premature The afferent limb of the blink reflex is carried
infants originates from rupture of the fragile via the trigeminal nerve ipsilateral to the
blood vessels in the subependymal germinal stimulus. The efferent limb is transmitted
matrix. The vascular germinal matrix spawns through bilateral facial nerves. A pattern of
the neuroectodermal constituents of the absent R1 and ipsilateral R2 response on
brain and involutes at maturity. In the stimulation of one side and absent
premature, this structure is still present, and contralateral R2 to stimulation of the
in the face of hemodynamic instability or opposite side is consistent with a lesion of
hypoxemia may hemorrhage. the facial nerve.

Reference: Reference:
Nelson JS, Parisi JE, Schochet SS. Kimura J. Electrodiagnosis in disease of
Principles and practice of neuropathology. nerve and muscle. 3rd ed. New York: Oxford
St. Louis: Mosby, 1993. University Press, 2001.
_____________________________ _____________________________
Question(s) 8: Pathology Question(s) 11: Physiology
Discussion: Discussion:
Although increased internal nuclei are seen Valproic acid is the drug of choice for
in many myopathies, this alteration is patients with absence and tonic-clonic
particularly noteworthy in myotonic seizures and the 3Hz spike wave pattern on
dystrophy in which up to 30% of myofibers the EEG. Ethosuximide, while effective for
may bear internal nuclei. As in all muscle absence seizures, will not effectively treat
biopsies, care must be taken not to sample the generalized tonic clonic seizures.
the muscle too close to a tendinous insertion Carbamazepine and phenytoin are less
as this area is normally rich in internal effective against absence seizures.
nuclei.
Reference:
Reference: Levy R, Mattson R, Meldrum B.
Graham DI, Lantos PL. Greenfield's Antiepileptic drugs. 4th ed. New York:
neuropathology. 6th ed. London: Arnold, Raven Press, 1995.
1997. _____________________________
_____________________________ Question(s) 12: Pharmacology/Chemistry
Question(s) 9: Pharmacology/Chemistry Discussion:
Discussion: The aim of treatment of severe hypertension
Seizures and myoclonus are associated with in neurologic catastrophes associated with
use of high doses of meperidine. The increased intracranial pressure is to reduce
excitability is due to a metabolite the blood pressure to safe levels avoiding
normeperidine, which has twice the CNS further increase in intracranial pressure. The
stimulant effect of meperidine. drugs of choice include labetalol, esmolol,
and enalapril. Clonidine may be used in
some cases. Vasodilators, such as calcium
Reference: channel blockers or nitrites, are best
Jaffe JH, Martin W. Opioid analgesics and avoided as they may increase intracranial
antagonists. In: Guilman AG, Rall TW, Nies pressure in the setting of reduced systemic
AS, et al, editors. Goodman and Gilman's blood pressure, thus reducing cerebral
the pharmacological basis of therapeutics. perfusion pressure in areas with poor
8th ed. New York: Pergamon Press, cerebral autroregulation.
1990;485-521.
_____________________________

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Reference: Question(s) 16: Clinical Adult

Wijdicks EFM. The clinical practice of critical Discussion:
care neurology. Philadelphia: Lippincott- Oral potassium in repeated doses is
Raven, 1997. recommended for an attack of hypokalemic
_____________________________ periodic paralysis.
Question(s) 13: Clinical Adult
Discussion: Reference:
Sudden death is more often a manifestation Victor M, Ropper A. Adams and Victor's
of cardiac disease than of neurologic principles of neurology. 7th ed. New York:
disease. McGraw-Hill, Co., 2001.
_____________________________
Reference: Question(s) 17: Pathology
Bradley WG, Daroff RB, Fenichel GM, et al, Discussion:
editors. Neurology in clinical practice. 3rd The presence of demyelination with
ed. Boston: Butterworth-Heinemann, 2000. inclusion bodies in oligodendrocytes is
_____________________________ characteristic of progressive multifocal
Question(s) 14: Clinical Adult leukoencephalopathy (PML). The astrocytes
in the areas of demyelination may be large
Discussion:
and contain atypical bizarre nuclei. PML is
Lesions of the fifth lumbar nerve root
caused by infection of oligodendroglia with
produce denervation that is evident clinically
the JC virus, which is a member of the
in toe extensor, foot dorsiflexor, and foot
papova group of DNA viruses. The virus
invertor (posterior tibial) muscles.
receives its name from the initials of the first
Involvement of paraspinous muscles
patient from whom it was isolated and has
(electromyographically) confirms that the
no relationship to Jakob-Creutzfeldt disease
site of the lesion is at the level of the nerve
(more commonly termed Creutzfeldt-Jakob
root.
disease, CJD), which is a prion disorder, not
a viral disease.
Reference:
Adams RD, Victor M. Principles of Reference:
neurology. 5th ed. New York: McGraw-Hill
Graham DI, Lantos PL. Greenfield's
Co., 1994.
neuropathology. 6th ed. London: Arnold,
_____________________________ 1997.
Question(s) 15: Pharmacology/Chemistry _____________________________
Discussion: Question(s) 18: Anatomy
REM sleep behavior disorder (RBD) occurs
Discussion:
in patients with neurodegenerative
The short head of the biceps femoris is the
disorders, such as Parkinson's disease and
only muscle proximal to the knee innervated
multiple system atrophy. Clonazepam (0.5-
by the peroneal division of the sciatic nerve.
1.0 mg at bedtime) is very effective in
controlling both the behavior and the dream
components of RBD. Reference:
Delagi EF, Perotto A. Anatomic guide for the
Reference: electromyographer 1980;172.
Schenck CH, Mahowald MW. REM sleep _____________________________
parasomnias. In: Aldrich MS, editor.
Neurologic clinics - sleep disorders II.
Philadelphia: WB Saunders, 1996.
_____________________________

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Question(s) 19: Pathology retina. Methotrexate toxicity causes a

necrotizing leukoencephalopathy, phenytoin
Discussion: damages Purkinje cells (either primarily or
Hemorrhage and necrosis of the central core from intermittent hypoxia associated with
of the spinal cord usually results from repeated seizures), taxol toxicity causes a
traumatic subluxation injury of the cervical dose-dependent peripheral neuropathy with
spine. Rarely, hematomyelia can also result painful dysesthesias, and vincristine also
from intraspinal vascular malformations. causes a neuropathy, usually initially a
sensory neuropathy.
Reference:
Graham DI, Lantos PL. Greenfield's Reference:
neuropathology. 6th ed. London: Arnold, Graham DI, Lantos PL. Greenfield's
1997. neuropathology. 6th ed. London: Arnold,
_____________________________ 1997.
Question(s) 20: Clinical Adult _____________________________
Discussion: Question(s) 23: Neuroradiology
Although the pattern of recovery following a Discussion:
stroke may be variable, the earliest evidence Enhancement in the internal auditory canal
of a return of neurological function is usually is a non-specific finding as seen in many
an increase in tone. This generally occurs conditions, in addition to acoustic
before improvement in strength or a return schwannoma.
of reflexes.

Reference:
Reference: Mark AS. The vestibulocochlear cistern.
DeLisa JA, Gans BM. Rehabilitation Neuroimaging Clin N Am 1993;3:153-70.
medicine: Principles in practice. 2nd ed.
_____________________________
Philadelphia: Lippincott, 1993;458.
_____________________________ Question(s) 24: Pharmacology/Chemistry
Question(s) 21: Clinical Adult Discussion:
Several growth/differentiation factors have
Discussion: been shown to increase survival of specific
Hypnic headache is a rare nocturnal neuronal groups. The neurotrophin family
headache disorder affecting the elderly. including nerve growth factor (NGF), brain
Women are more often affected than men. derived neurotrophic factor (BDNF) and
Attacks of pain may be unilateral or bilateral neurotrophin-3 (NT-3) act via receptor
and are usually not accompanied by tyrosine kinase. For example, NGF, acting
autonomic symptoms. The headaches occur via trkA receptors, increases survival of
two to four hours after sleep onset and last nociceptive, sympathetic, and basal
15 to 30 minutes, occasionally hours. Some forebrain cholinergic neurons. Glial derived
patients have several attacks per night. neurotrophic factor (GDNF) acts via a
receptor complex that includes the
Reference: transmembrane tyrosine kinase Ret and is
Dodick DW, Mosek AC, Campbell JK. The critical for development of some nociceptive,
hypnic ("alarm clock") headache syndrome. parasympathetic, enteric, and dopaminergic
Cephalgia 1998;18:152-156. substantia nigra neurons. Ciliary
_____________________________ neurotrophic factor (CNTF) is a member of
the cytokine family that acts via a receptor
Question(s) 22: Pathology complex that may also recognize interleukin-
Discussion: 6. CNTF increases survival of motor
Chloroquine toxicity is associated with long- neurons and parasympathetic neurons.
term administration of the drug at doses Activation of the cytokine class receptor by
greater than 200 mg/day; the primary target
of damage is skeletal muscle, with less
frequent involvement of cardiac muscle or

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CNTF accounts for the severe side effects Reference:

observed when this agent was administered Klass DW, Westmoreland BF.
in clinical trials for amyotrophic lateral Electroencephalography: general principles
sclerosis. and adult electroencephalograms. In: Daube
JR, editor. Clinical neurophysiology.
Reference: Philadelphia: FA Davis Company, 1996;73-
103.
Mitsumoto H, Tsuzaka K. Neurotrophic
factors and neuromuscular disease: I. _____________________________
General comments, the neurotrophin family, Question(s) 28: Clinical Pediatrics
and neuropoietic cytokines. Muscle & Nerve Discussion:
1999;22:983-999. Patients with Lennox-Gastaut syndrome
_____________________________ have multiple types of seizures, such as
Question(s) 25: Pharmacology/Chemistry tonic, atypical absence, and atonic with age
of onset of one to eight years. The EEG
Discussion:
reveals slow spike-wave discharges, 1.5 to 2
Neuroleptic malignant syndrome is felt to
Hz. Most children are mentally retarded and
result from insufficient stimulation of
approximately 70% have an identifiable
dopamine receptors and is typically
cause for the retardation and epilepsy.
precipitated by treatment with high potency
Hypsarrythmia is an abnormal EEG pattern
neuroleptics. Dopamine agonist drugs, such
usually associated with infantile spasms in
as bromocriptine, have proven effective in
infants less than one year of age.
treating this potentially lethal condition.

Reference:
Reference:
1. Berg BO, editor. Principles of child
Johnston MV, MacDonald RL, Young AB.
neurology. New York: McGraw-Hill, 1996.
Principles of drug therapy in neurology.
Philadelphia: FA Davis, 1992. 2. Pellock JM, Dodson WE, Bourgeois BFD,
editors. Pediatric epilepsy: diagnosis and
_____________________________
therapy. 2nd ed. New York: Demos, 2001.
Question(s) 26: Pharmacology/Chemistry _____________________________
Discussion: Question(s) 29: Behavioral
Parkin parkinsonism is inherited in an
autosomal recessive manner and is Discussion:
characterized by early onset parkinsonism An anterior callosal lesion disconnects the
and dystonia. The responsible gene product left hand (right motor strip) from the verbal
is called Parkin and it is involved in protein left hemisphere, yielding an apraxia to
breakdown. It is a ubiquitin ligase and is verbal commands confined to the left hand
involved in the degradation of many proteins (alien hand syndrome).
including alpha-synuclein.
Reference:
Reference: Watson RT, Heilman KM. Callosal apraxia.
Shimura, et al. Familial Parkinson's disease Brain 1983;106:391-403.
gene product, Parkin, is a ubiquitin-protein _____________________________
ligase. Nature Genetics 2000;25:302-305.
_____________________________
Question(s) 27: Physiology
Discussion:
Breach rhythm is a finding seen in patients
with skull defect, which alters the
conductance between the brain and the
recording electrode over the scalp.

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Question(s) 30: Clinical Adult Question(s) 33: Pharmacology/Chemistry

Discussion: Discussion:
CNS toxoplasmosis is the most commonly The pedunculopontine nucleus (PPN)
encountered neurologic opportunistic receives inputs from the globus pallidus and
infection in AIDS patients. Pyrimethamine subthalamic nucleus, and projects to the
and sulfadiazine are of initial benefit in up to substantia nigra, thalamus, and medullary
90% of patients. The CSF profile can be reticulospinal neurons. The PPN contains
identical to that of HIV encephalopathy. one population of cholinergic and another
Blood and CSF serologies are not in general population of glutamatergic neurons. It has
diagnostically reliable. Most adults in the been implicated as a component of the
U.S. have antibodies indicative of prior mesencephalic locomotor region. There is
exposure to toxoplasmosis and cerebral loss of PPN neurons in Parkinson's disease,
involvement is thought to represent and this has been correlated to the gait
reactivation of a late primary infection. A abnormalities in this disorder.
course of empiric therapy for toxoplasmosis
is recommended in many cases. Reference:
Pahapill PA, Lozano AM. The
Reference: pedunculopontine nucleus and Parkinson's
Solbrig MV, Healy JF, Jay CA. Infection of disease. Brain 2000;123:1767-1783.
the nervous system. A. Bacterial infections. _____________________________
In: Bradley WG, Daroff RB, Fenichel GM, et Question(s) 34: Physiology
al, editors. Neurology in clinical practice.
Discussion:
3rd ed. Boston: Butterworth-Heinemann,
In diseases of the sensory system that
2000.
damage sensory fibers proximal to the
_____________________________ dorsal root ganglia, sensation is impaired
Question(s) 31: Anatomy but the sensory nerve action potential
Discussion: remains normal.
Innervation of the detrusor muscle is
parasympathetic (S2-S4). Reference:
Kimura J. Electrodiagnosis in disease of
Reference: nerve and muscle. 3rd ed. New York: Oxford
Benarroch EE, Westmoreland BF, Daube University Press, 2001.
JR, et al. Medical neurosciences - an _____________________________
approach to anatomy, pathology and Question(s) 35: Clinical Adult
physiology by systems and levels. 4th ed.
Discussion:
New York: Lippincott, Williams & Wilkins,
The most common neurologic manifestation
1999.
of Legionnaires' disease is delirium (found in
_____________________________ 30% of cases). Ataxia, transverse myelitis,
Question(s) 32: Physiology and CSF pleocytosis are less common
Discussion: manifestations.
F-responses can be used to assess the
entire length of the motor axon and can be Reference:
absent or delayed in demyelinating diseases
Solbrig MV, Healy JF, Jay CA. Infection of
of the peripheral nerve, such as in Guillain
the nervous system. A. Bacterial infections.
Barré syndrome.
In: Bradley WG, Daroff RB, Fenichel GM, et
al, editors. Neurology in clinical practice.
Reference: 3rd ed. Boston: Butterworth-Heinemann,
Kimura J. Electrodiagnosis in disease of 2000
nerve and muscle. 3rd ed. New York: Oxford _____________________________
University Press, 2001.
_____________________________

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Question(s) 36: Clinical Adult Reference:

Menza MA, Liberatore BL. Psychiatry in the
Discussion:
geriatric neurology practice. In: Riggs JE,
Episodic dyscontrol is used to identify short
editor. Neurologic clinics - the neurology of
periods of rage (violence) in an otherwise
aging. Philadelphia: WB Saunders, 1998.
peaceful individual. The patient often
expresses disbelief and/or remorse _____________________________
concerning the incident; some degree of Question(s) 39: Behavioral
intoxication (most often alcohol) is frequently Discussion:
but not invariably present. Anosodiaphoria may be seen with right
hemisphere lesions as in this patient. It is
Reference: indifference to their condition despite the
Rickler KC. Episodic dyscontrol. In: Benson recognition that they may have a
DF, Blumer D, editors. Psychiatric aspects hemiparesis and/or hemisensory deficit.
of neurologic disease. New York: Grune &
Stratton, 1982;2:49-72. Reference:
_____________________________ Heilman KM, Watson RT, Valenstein E,
Question(s) 37: Clinical Adult editors. Clinical neuropsychology. 2nd ed.
New York: Oxford University Press, 1985.
Discussion:
Partial seizures are those for which there is _____________________________
behavioral or EEG evidence of onset in a Question(s) 40: Clinical Adult
focal area of the brain limited to one lobe or Discussion:
hemisphere. The seizure focus determines ALS is a disorder of upper and lower motor
whether the seizure has sensory neurons. The associated dysarthria reflects
manifestations, motor manifestations or both flaccid and spastic features.
both. Partial seizures are classified as
complex when there is impairment of
consciousness. Partial complex seizures Reference:
may occur at any age. Partial complex Darley FL, Aronson AE, Brown JR.
seizures most often arise from the temporal Differential diagnostic patterns of dysarthria.
lobe but may also arise from other lobes of Journal of Speech and Hearing Research
the brain, particularly the frontal lobe. They 1969;12:246-269.
respond to a number of different _____________________________
anticonvulsants, though they tend to be Question(s) 41: Pharmacology/Chemistry
more refractory to medical treatment than
most other types of seizure. Discussion:
In patients with detrusor hyperreflexia
without outlet obstruction or urinary
Reference: retention, anticholinergic drugs, including
Engel J Jr. Seizures and epilepsy. oxybutynin, are the most appropriate
Philadelphia: FA Davis Co., 1989. treatment. If retention occurs, this should be
_____________________________ combined with intermittent self-
Question(s) 38: Behavioral catheterization.

Discussion:
Late-life delusional disorder is characterized Reference:
by the occurrence of non-bizarre delusions, Chancellor MB, Blaivas JG. Urological and
predominantly involving erotic, grandiose, sexual problems in multiple sclerosis. Clin
jealous or persecutory themes. Average Neurosci. 1996;2:189-195.
age of onset for women is 60-69 years and _____________________________
the minimum duration of symptoms is one
month.

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Question(s) 42: Pharmacology/Chemistry Question(s) 45: Pharmacology/Chemistry

Discussion: Discussion:
Malignant hyperthermia and central core Nimodipine and other dihydropyridines block
disease are autosomal dominant disorders calcium influx through L-type channels.
associated with mutations in the muscle These channels are involved in slow
ryanodine receptor (RYR). The RYR is the depolarization and excitation-contraction
calcium release channel of the sarcoplasmic coupling of the vascular smooth muscle.
reticulum of the skeletal muscle. Calcium influx through N- and P/Q channels
triggers neurotransmitter release.
Reference:
Quene KA, Healy JMS, Keating KE, et al. Reference:
Mutations in the ryanodine receptor gene in Murad F. Drugs used for the treatment of
central core disease and malignant angina: organic nitrates, calcium-channel
hyperthermia. Nature Genetics [Link]- blockers and adrenergic antagonists. In:
55. Gilman AG, Rall TW, Nies AS, et al, editors.
_____________________________ Goodman and Gilman's the pharmacological
Question(s) 43: Clinical Adult basis of therapeutics. 8th ed. New York:
Pergamon Press, 1990.
Discussion: _____________________________
Marburg's variant of multiple sclerosis is
among the more acute of the idiopathic Question(s) 46: Behavioral
demyelinating disorders. It may need to be Discussion:
included in the differential diagnosis of Carbamazepine and amitriptyline have both
stupor and coma. been used for emotional lability. However,
carbamazepine is the better choice in
persistent post-traumatic agitation because
Reference: it does not have the anticholinergic and mild
1. Young GB. Other inflammatory disorders. epileptogenic properties that amitriptyline
In: Young GB, Ropper AH, Bolton CF, has.
editors. Coma and impaired consciousness.
New York: McGraw-Hill, 1998.
2. Hogancamp WE, Noseworthy JH. Reference:
Demyelinating disorders of the central Shutter LA, Colohan AR. Post-traumatic
nervous system. In: Goetz CG, Pappert EJ, agitation: psychopharmacology and early
editors. Textbook of clinical neurology. management. The Neurologist 1999 Sept;
Philadelphia: WB Saunders, Co., 1999. 5(5):258-270.
_____________________________ _____________________________
Question(s) 44: Clinical Adult Question(s) 47: Physiology
Discussion: Discussion:
Duchenne's muscular dystrophy is An EEG pattern consisting of generalized,
associated with high creatine kinase (CK) bisynchronous periodic sharp-and-slow
levels. It is a sex-linked recessive disorder wave complexes recurring at 4- to 15-
resulting in abnormal amounts of a protein, second intervals is characteristic of
dystrophin, in muscle. subacute sclerosing panencephalitis.

Reference: Reference:
Brooke MH. A clinical view of Klass DW, Westmoreland BF.
neuromuscular disease. 2nd ed. Baltimore: Electroencephalography: general principles
Williams & Wilkins, 1986. and adult electroencephalograms. In: Daube
_____________________________ JR, editor. Clinical neurophysiology.
Philadelphia: FA Davis Company, 1996;73-
103.
_____________________________

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Question(s) 48: Clinical Adult classified as low grade (WHO grade I):
Discussion: meningothelial, fibrous, transitional,
Progressive myelopathy with chiefly motor psammomatous, angiomatous, microcystic,
signs, occurring in various endemic foci secretory, lymphoplasmacyte-rich, and
(Jamaica, South India, South Africa, metaplastic.
Colombia, Japan) is associated with the
retrovirus HTLV-I. Multiple sclerosis is Reference:
suggested by recurrent optic neuritis. Cycad 1. Kleihues P, Cavenee WK, editors.
consumption may be associated with Pathology and genetics of tumors of the
Guamanian ALS and the parkinsonism- nervous system. 2nd ed. New York: Oxford
dementia complex. University Press, 2000.
2. Fuller GN, Goodman JC. Practical review
Reference: of neuropathology. Philadelphia: Lippincott,
Adams RD, Victor M. Principles of Williams & Wilkins, 2001.
neurology. 5th ed. New York: McGraw-Hill _____________________________
Co., 1994. Question(s) 50: Pharmacology/Chemistry
_____________________________ Discussion:
Question(s) 49: Pathology Facial nerve palsy, frequently bilateral, is the
Discussion: most common neurological sign of
The potential for invasion of mesenchymal sarcoidosis. It is often accompanied by
tissues, including the dura (and superior parotid gland swelling. Loss of taste is also
sagittal sinus), cranial bones, muscles and common in conjunction with the facial
scalp, is an intrinsic property that is weakness and indicates that the lesion is
frequently seen to a greater or lesser extent above the exit of the chorda tympani.
in many ordinary (WHO grade I) Sarcoidosis is often responsive to
meningiomas. Invasion of these corticosteroid therapy.
mesenchymal tissues may complicate
surgical resection but does not constitute a Reference:
criterion for upgrading to atypical (WHO Silberberg DH. Sarcoidosis of the nervous
grade II) or anaplastic (WHO grade III) system. In: Aminoff MJ, editor. Neurology
meningioma. Brain invasion, in contrast, is and general medicine. New York: Churchill
much less commonly seen and its presence Livingston, 1989.
in the absence of prior surgical procedures _____________________________
warrants a diagnosis of atypical (WHO
grade II) meningioma. Highly vascular Question(s) 51: Pharmacology/Chemistry
meningiomas are sometimes referred to as Discussion:
angiomatous and have no association with Drugs that can raise carbamazepine levels
aggressive behavior. The older term include isoniazid, erythromycin, cimetidine,
"angioblastic meningioma" encompassed a calcium channel blockers (such as
heterogeneous group of vascular dural- verapamil), and propoxyphene.
based tumors that included angiomatous Carbamazepine levels are lowered by
meningioma, hemangiopericytoma and phenobarbital, phenytoin, and primidone.
hemangioblastoma; it is an imprecise and Warfarin, chlorpromazine, digoxin, and
obsolete term that should be avoided. Four gabapentin have no significant effect on
histologic subtypes merit upgrading based carbamazepine levels.
on their potential for early recurrence and/or
aggressive clinical behavior: clear cell (WHO
grade II), chordoid (WHO grade II), rhabdoid Reference:
(WHO grade III), and papillary (WHO grade MacDonald Rl. Seizure disorders and
III). In contrast, the remaining nine WHO- epilepsy. In: Johnston MV, MacDonald RL,
recognized meningioma subtypes are Young AB, editors. Principles of drug
therapy in neurology. Philadelphia: FA
Davis, 1992.
_____________________________

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Question(s) 52: Pharmacology/Chemistry Reference:

Davis LE. Fungal infections of the nervous
Discussion:
system. Neurol. Clin 1999;17:761-781.
Galantamine (Reminyl) is a drug recently
approved in the United States for treatment _____________________________
of Alzheimer's disease. Unlike donepezil, Question(s) 55: Pharmacology/Chemistry
rivastigmine, and tacrine, galantamine not Discussion:
only blocks acetylcholinesterase but also Paroxysmal kinesigenic dyskinesia is
activates presynaptic nicotinic receptors by characterized by asymetrical dystonic or
binding to an allosteric site of the molecule. choreic movements brought on by startle or
This may result in increased release of sudden movements. These attacks respond
neurotransmitters. well to low dose anticonvulsants.

Reference: Reference:
Francis PT, Palmer AM, Snape M, et al. The Sethi KD. Paroxysmal dyskinesias. The
cholinergic hypothesis of Alzheimer's Neurologist 2000 May;6(3):177-185.
disease: a review of progress. J Neurol _____________________________
Neurosurg Psychiatr 1999;66:137-147.
_____________________________ Question(s) 56: Physiology
Question(s) 53: Anatomy Discussion:
In secondary bilateral synchrony, the focal
Discussion: epileptiform discharge spreads to relevant
The foramen ovale transmits the mandibular subcortical and forebrain structures and then
division of the trigeminal (V) nerve. The to the contralateral cortex.
foramen rotundum transmits the maxillary
division of the trigeminal (V) nerve.
Reference:
Engel J Jr. Seizures and epilepsy.
Reference: Philadelphia: FA Davis Co., 1989.
Brazis PW, Masdeu JC, Biller J. Localization _____________________________
in clinical neurology. 3rd ed. Boston: Little,
Brown, 1996. Question(s) 57: Physiology
_____________________________ Discussion:
Question(s) 54: Pharmacology/Chemistry Inclusion body myositis produces a
characteristic pattern of weakness involving
Discussion: finger flexor and quadriceps muscles most
Fluconazole, together with itraconazole and severely. Muscle enzymes are normal or
ketoconazole, belongs to the triazole class minimally increased, and EMG studies
of antifungal drugs that inhibit the synthesis typically reveal an inflammatory myopathy.
of ergosterol, with the accumulation of The muscle biopsy finding most typical of
substitued sterols that interfere with the inclusion body myositis is vacuole formation
synthesis and permeability of the fungal cell surrounded by a basophilic rim.
membrane. Like other triazoles, fluconazole
inhibits cytochrome P450 - dependent liver
metabolism and consequently increases Reference:
plasma concentration of phenytoin, warfarin, Engel AG, Franzini-Armstrong C. Myology.
cyclosporine, zidovudine, oral hypoglycemic 2nd ed. New York: McGraw-Hill, 1994.
agents, and other drugs. Fluconazole is the _____________________________
most widely used oral agent for CNS fungal
infections. One of its advantages is that it
crosses the blood brain barrier well, with
CSF concentrations that are 50-90% of the
plasma. Its main disadvantage is that it has
a lower cure rate than amphotericin B for
most CNS fungal infections, including
cryptococcal meningitis.

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Question(s) 58: Physiology Question(s) 61: Pathology

Discussion: Discussion:
Median somatosensory evoked potentials in Malignant peripheral nerve sheath tumors
cervical spondylosis may show diminished most commonly arise in a neurofibroma,
amplitude of N13. Arnold-Chiari often of the plexiform type. They may also
malformation is associated with normal arise de novo in a normal nerve. Malignant
amplitude of N13 and increased N13-N20 transformation of a schwannoma or
interpeak latency. In amyotrophic lateral ganglioneuroma is rare. It is extremely rare
sclerosis, median SEP's are typically to find these malignant peripheral nerve
normal. sheath tumors involving or arising from
cranial nerves or cranial nerve
neurofibromas.
Reference:
Daube JR, editor. Clinical neurophysiology.
Philadelphia: FA Davis, 1996. Reference:
_____________________________ Graham DI, Lantos PL. Greenfield's
Question(s) 59: Neuroradiology neuropathology. 6th ed. London: Arnold,
1997.
Discussion: _____________________________
MRI does not involve gamma rays,
positrons, sodium or potassium. Only Question(s) 62: Physiology
protons are involved. Nuclei with paired Discussion:
electrons in the outer shell do not give an Vasopressin is secreted by cells of the
MR signal. supraoptic nuclei.

Reference: Reference:
Mazziotta JC, Gilman S. Clinical Brain Parent A. Carpenter's human
Imaging: principles and applications. neuroanatomy. 9th ed. Baltimore: Williams &
Philadelphia: FA Davis Co., 1992;39-70. Wilkins, 1996.
_____________________________ _____________________________
Question(s) 60: Pharmacology/Chemistry Question(s) 63: Neuroradiology
Discussion: Discussion:
Pantothenate kinase is essential to Cavernous hemangioma, one of the most
coenzyme A (CoA) synthesis. Deficiency of common malformations identified on MRI, is
CoA may lead to impaired membrane best visualized on gradient echo and T2-
biosynthesis, for example in rod weighted images with high field strength
photoreceptors, that degenerate in HSS. scanners. They typically have popcorn
Phosphopantothenate, the product of appearance with mixed signal core and
pantothenate kinase, is deficient, leading to hemosiderin rim. Pontine glioma, ruptured
cysteine accumulation, that in turn aneurysm, tuberculoma or venous angioma
predisposes to free radical production and do not contain calcium or hemosiderin rim.
lipid peroxidation in the presence of iron.
Reference:
Reference: Atlas S, Mark A, Fram E, et al. Vascular
Zhou B, Westaway SK, Levinson B, et al. A intracranial lesions: applications of gradient
novel pantothenate kinase gene (PANK 2) is echo imaging. Radiology 1988;169:455-61.
defective in Hallervorden-Spatz syndrome. _____________________________
Nature Genetics 2001;28:345-349.
_____________________________

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Question(s) 64: Pharmacology/Chemistry Question(s) 67: Pharmacology/Chemistry

Discussion: Discussion:
The serotonin syndrome consists of a Complex partial status epilepticus presents
combination of mental and behavioral with altered awareness and responsiveness.
changes, motor hyperactivity, and Automatisms may be present. EEG will
autonomic lability, that occurs following use demonstrate characteristic epileptiform
of potent serotomimetic agents alone or in discharges. This form of status epilepticus
combination with non-specific monoamine usually responds well to intravenous
oxidase inhibitors (MAOIs). This syndrome benzodiazepines, such as lorazepam, which
can occur in patients taking various must be followed by anticonvulsants
combinations of drugs, including serotonin effective in chronic seizure control, such as
precursors (e.g. tryptophan), serotonin carbamazepine.
reuptake inhibitors (e.g. fluoxetine,
sertraline, clomipramine, imipramine,
nortriptyline, trazodone), MAOIs (clorgyline, Reference:
phenelzine, tranylcypromine, iproniazid). Ramsay RE. Acute treatment of seizures.
In: Weiner WJ, editor. Emergent and urgent
neurology. Philadelphia: JB Lippincott,
Reference: 1992.
Bodner RA, Lynch T, Lewis L, et al. _____________________________
Serotonin syndrome. Neurology Question(s) 68: Physiology
1995;45:219-223.
_____________________________ Discussion:
In BAEP's, absence of wave I with intact
Question(s) 65: Clinical Adult wave V is most commonly due to peripheral
Discussion: hearing loss.
In barbiturate coma, ciliospinal and
oculovestibular reflexes may be absent, the
EEG may be flat, and there may be loss of Reference:
spontaneous respirations. Characteristically, Chiappa KH. Evoked potentials in clinical
the pupils remain reactive. medicine. 3rd ed. New York: Raven Press,
1997.
_____________________________
Reference:
Question(s) 69: Anatomy
Adams RD, Victor M. Principles of
neurology. 5th ed. New York: McGraw-Hill Discussion:
Co., 1994. The anterior choroidal artery arises from the
_____________________________ internal carotid artery distal to the origin of
the posterior communicating artery. It has a
Question(s) 66: Physiology long subarachnoid course, enters the inferior
Discussion: horn of the lateral ventricle through the
EMG needle examination evidence of choroidal fissure, and supplies the
fibrillation potentials and decreased amygdaloid complex, hippocampal
recruitment of motor units in the iliopsoas, formation, globus pallidus, and the
vastus medialis, adductor longus and vastus ventrolateral portion of the posterior limb
lateralis muscles without other abnormality and the entire retrolenticular portion of the
localizes the lesion to the lumbar plexus. internal capsule.

Reference: Reference:
Kimura J. Electrodiagnosis in disease of Parent A. Carpenter's human
nerve and muscle. 3rd ed. New York: Oxford neuroanatomy. 9th ed. Baltimore: Williams &
University Press, 2001. Wilkins, 1996.
_____________________________ _____________________________

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Question(s) 70: Anatomy Question(s) 73: Anatomy

Discussion: Discussion:
The inferior cerebellar peduncle connects The ciliary muscle receives post ganglionic
the medulla to the cerebellum and contains parasympathetic innervation via the 3rd
the dorsal spinocerebellar tract, (oculomotor) cranial nerve. The superior
cuneocerebellar tract, olivocerebellar tract, cervical ganglion provides the sympathetic
and the vestibulocerebellar tract. The innervation to the facial sweat glands,
trigeminocerebellar tract lies within the carotid artery, pineal gland and tarsal
superior cerebellar peduncle and would be muscle.
spared in a lesion confined to the inferior
cerebellar peduncle. Reference:
Reference: Low PA, editor. Clinical autonomic
disorders. 2nd ed. New York: Lippincott-
Brazis PW, Masdeu JC, Biller J. Localization
Raven, 1997.
in clinical neurology. 3rd ed. Boston: Little,
_____________________________
Brown, 1996.
_____________________________ Question(s) 74: Physiology
Question(s) 71: Pathology Discussion:
One of the most important technical aspects
Discussion: of recording evoked potentials is replication
Sturge-Weber syndrome is characterized by
of the waveforms.
facial vascular nevi in a trigeminal
distribution and leptomeningeal vascular
malformation of the occipital lobe. Some Reference:
patients also have ocular choroidal Chiappa KH. Evoked potentials in clinical
angiomas. medicine. 3rd ed. New York: Raven Press,
1997.
Reference: _____________________________
Graham DI, Lantos PL. Greenfield's Question(s) 75: Pharmacology/Chemistry
neuropathology. 6th ed. London: Arnold, Discussion:
1997. Demyelination results in dysregulation of
_____________________________ expression of voltage gated sodium
Question(s) 72: Clinical Adult channels in the axon. There is upregulation
of some sodium channel subtypes and
Discussion:
homogeneous redistribution of the sodium
Paroxysmal dyskinesias consist of episodes
channels along the axon. This allows
of involuntary movements and are classified
recovery of function, but at the expense of
according to phenomenology, duration of
abnormal axonal excitability.
attacks, and etiology. With paroxysmal
kinesigenic dyskinesia (paroxysmal
kinesigenic choreoathetosis) abnormal Reference:
involuntary movements lasting seconds to Waxman SG. Acquired channelopathies in
minutes occur abruptly after a sudden nerve injury and MS. Neurology 2001;
voluntary movement. With paroxysmal 56:1621-1627.
nonkinesigenic dyskinesia (paroxysmal _____________________________
dystonic choreoathetosis) attacks of
involuntary movements last minutes to hours
and occur spontaneously.

Reference:
Demirkiran M, Jankovic J. Paroxysmal
dyskinesias: clinical features and
classification. Ann Neurol 1995;113:1645-
1672.

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Question(s) 76: Clinical Adult necrosis in which the cell explodes, thereby
releasing its contents into its environment
Discussion: and provoking an inflammatory response.
Aphasia is a disorder of language, to be
distinguished from disorders of speech
(dysarthria, dysphonia) and disorders of Reference:
thought (e.g., dementia, confabulation, Graham DI, Lantos PL. Greenfield's
perseveration, agnosia). Aphasic utterances neuropathology. 6th ed. London: Arnold,
can involve nonexistent word forms called 1997.
neologisms. _____________________________
Question(s) 79: Pathology
Reference: Discussion:
Bradley WG, Daroff RB, Fenichel GM, et al. Toxoplasmosis is a special hazard in
Neurology in clinical practice. 2nd ed. patients with HIV infection and often
Boston: Butterworth-Heinemann, 1996. appears as multiple ring-enhancing lesions
_____________________________ on CT examination.
Question(s) 77: Pharmacology/Chemistry
Discussion: Reference:
Sudden sensorineural hearing loss (SSNHL) Graham DI, Lantos PL. Greenfield's
has an incidence of 1:3000. A study of 603 neuropathology. 6th ed. London: Arnold,
patients with this diagnosis compared the 1997.
outcomes in patients treated with IV _____________________________
pentoxifylline alone, IV glucocorticoids
Question(s) 80: Clinical Pediatrics
alone, and the combination of IV
pentoxifylline and IV glucorcoticoids. None Discussion:
of the therapies appeared to be harmful; the A study of 160 children from 36 weeks
combination of steroids and pentoxifylline gestation to 18 years of age with
produced more improvement at all radiographic confirmation of cerebral
frequencies than pentoxifylline alone, and sinovenous thrombosis found that 58
glucortocoids alone produced better results percent of children had seizures, 76 percent
than non-steroidal therapy. Antiviral therapy had diffuse neurologic signs, and 42 percent
for SSNHL has not been evaluated in this had focal neurologic signs. 43 percent of
fashion. patients were neonates, and 54 percent
were under one year of age. Risk factors
were present in 98 percent of cases, and
Reference: included acute systemic illness, head and
Alexiou C, Arnold W, Fauser C, et al. neck disorders and abnormal tests for
Sudden sensorineural hearing loss: does prothrombotic disorders (in 38 percent).
application of glucocorticoids make sense? Seizures at presentation and the presence
Arch Otolaryngol Head Neck Surg of venous infarcts predicted adverse
2001;127:253-258. neurologic outcomes.
_____________________________
Question(s) 78: Pathology Reference:
Discussion: DeVeber G, Andrew M, Adams C, et al.
Apoptosis is an active energy-dependent Cerebral sinovenous thrombosis in children.
process in which individual cells discretely N Engl J Med 2001;345:417-423.
die without provoking the hubbub of _____________________________
inflammation. Proteases, endonucleases
and lipases are activated, destroying the cell
from within. One hallmark of apoptosis is
fragmentation of the nucleus into chunks
called apoptotic bodies. Cellular and
organelle swelling are more common in

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Question(s) 81: Anatomy

Discussion: Reference:
The upper visual fields ultimately project to Kuhar MJ, et al. The dopamine hypothesis
the inferior lip of the contralateral calcarine of the reinforcing properties of cocaine.
sulcus. Trends Neurosci 1991;14:299-302.
_____________________________
Reference: Question(s) 85: Behavioral
Benarroch EE, Westmoreland BF, Daube Discussion:
JR, et al. Medical neurosciences - an Patients with REM behavior disorder have
approach to anatomy, pathology and lack of limb paralysis that normally
physiology by systems and levels. 4th ed. accompanies REM sleep. Therefore, they
New York: Lippincott, Williams & Wilkins, act out their dreams, which can often involve
1999. aggressive or violent behavior.
_____________________________
Question(s) 82: Physiology Reference:
Discussion: Schenck CH, Mahowald MW. REM sleep
A patient with a recent stroke and seizures parasomnias. In: Aldrich MS, editor.
would most likely show periodic lateralized Neurologic clinics - sleep disorders II.
sharp wave discharges on the EEG. Philadelphia: WB Saunders, 1996.
_____________________________
Reference: Question(s) 86: Anatomy
Daly DD, Pedley TA. Current practice of Discussion:
clinical electroencephalography. 2nd ed. The internal arcuate fibers from the dorsal
New York: Raven Press, 1990. column nuclei (cuneate and gracile nuclei)
_____________________________ decussate completely to form the medial
Question(s) 83: Anatomy lemniscus.
Discussion:
The anterior cingulate gyrus has an Reference:
inhibitory influence on the micturition reflex Benarroch EE, Westmoreland BF, Daube
and would be the most likely site of JR, et al. Medical neurosciences - an
impairment. approach to anatomy, pathology and
physiology by systems and levels. 4th ed.
New York: Lippincott, Williams & Wilkins,
Reference: 1999.
De Groat WC, Booth AM. Autonomic
_____________________________
systems to the urinary bladder and sexual
organs. In: Dyck PJ, Thomas PK, editors. Question(s) 87: Behavioral
Peripheral neuropathy. Philadelphia: WB Discussion:
Saunders, 1993. Digit span, which involves attentional
_____________________________ processes, immediate recall and ability to
Question(s) 84: Pharmacology/Chemistry sequence bits of information, may be
reduced following lesions of dorsolateral
Discussion: frontal cortex. Lesions of the fornix,
Cocaine, like amphetamine, binds to the mammillary bodies and medial dorsal
presynaptic dopamine transporter (DAT) nucleus of the thalamus, on the other hand,
inhibiting dopamine reuptake and increasing cause amnesia (impaired secondary
dopamine concentrations in the nucleus memory) without a reduction of digit span.
accumbens. This mechanism underlies the
acute reinforcing effects of these drugs of
addiction. Reference:
Fuster JM. The prefrontal cortex. 2nd ed.
New York: Raven Press, 1989.
_____________________________

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Question(s) 88: Clinical Pediatrics hyperintensities, loss of total white matter

Discussion: volume and preferential involvement of the
Hypoxic-ischemic encephalopathy is the frontal white matter that correlates with
most common cause of early seizures in observed neuropsychological deficits.
both premature and full-term infants.
Intracranial hemorrhage may occur anytime Reference:
during the first week and is a more common Filley CM, Kleinschmidt-DeMasters BK.
cause of seizures in premature infants than Toxic leukoencephalopathy. N Engl J Med
in term infants. Developmental 2001:345;425-432.
abnormalities of the brain may be _____________________________
associated with early or late seizures.
Kernicterus initially presents with hypotonia, Question(s) 91: Physiology
cerebral depression, and seizures are Discussion:
uncommon. Pyridoxine dependency is a A unilateral P100 abnormality indicates an
very rare cause of intractable seizures. ipsilateral lesion of the visual pathway
anterior to the optic chiasm such as a
unilateral demyelinating process or optic
Reference: nerve glioma. A tumor of the occipital lobe or
Volpe JJ. Neurology of the newborn. 4th ed. a thalamic hemorrhage would cause a
Philadelphia: WB Saunders, 2001. bilateral abnormality or little or no effect on
_____________________________ the P100 latency.
Question(s) 89: Physiology
Discussion: Reference:
A progressive increase in latency, duration, Chiappa KH. Evoked potentials in clinical
amplitude and area of motor and sensory medicine. 3rd ed. New York: Raven Press,
nerve action potentials accompanies a 1997.
physiologic decline in temperature. This is _____________________________
reversed by warming.
Question(s) 92: Clinical Adult
Discussion:
Reference: Epidural metastases (carcinoma, lymphoma,
Aminoff M. Electromyography in clinical myeloma) are the most common spinal
practice. 3rd ed. New York: Churchill tumors in the elderly.
Livingston, 1998.
_____________________________
Reference:
Question(s) 90: Pharmacology/Chemistry
Adams RD, Victor M. Principles of
Discussion: neurology. 4th ed. New York: McGraw-Hill,
Toxic leukoencephalopathy is associated 1989.
with exposure to therapeutic agents _____________________________
(particularly antineoplastic drugs), drugs of
abuse and environmental toxins. Question(s) 93: Clinical Adult
Hyperintensity of white matter on T-2 Discussion:
weighted sequences is characteristic, and Prosopagnosia (inability to identify faces
the diagnosis should not be made in the visually) is most often seen after bilateral
absence of such changes. Methotrexate and inferior occipito-temporal lesions affecting
carmustine are the anticancer drugs most both fusiform gyri. Similar bilateral lesions
commonly implicated in toxic (or a nondominant lesion) of the fusiform
leukoencephalopathy. Carbon monoxide and lingual gyri account for cerebral
poisoning typically produces demyelination achromatopsia. Palinopsia (recurrence of a
that begins days to weeks after exposure. visual image after diverting gaze or when
The mechanism is not known, although the stimulus object is withdrawn) occurs with
prolonged depression of oxygenation and occipito-temporal disease, often
circulation has been proposed. Alcoholism epileptogenic. Alexia without agraphia
produces a number of changes, including an (inability to read but with preserved writing
excessive number of white matter ability) occurs with combined lesions of the

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dominant occipital lobe and the inferior Reference:

splenium of the corpus callosum or with a Parent A. Carpenter's human
single lesion of the dominant occipito- neuroanatomy. 9th ed. Baltimore: Williams &
temporal paraventricular white matter Wilkins, 1996.
behind, beneath, and beside the occipital _____________________________
horn of the lateral ventricle. Alexia with
agraphia (central alexia) is usually due to an Question(s) 97: Anatomy
angular gyrus (parietal lobe) lesion. Discussion:
The suprachiasmatic nucleus of the
hypothalamus receives direct inputs from
Reference: the retina. This retino-hypothalamic tract
Brazis PW, Masdeu JC, Biller J. Localization mediates influences of the day-night light
in clinical neurology. 3rd ed. Boston: Little, cycle on the circadian pacemaker.
Brown, 1996.
_____________________________
Reference:
Question(s) 94: Pharmacology/Chemistry Parent A. Carpenter's human
Discussion: neuroanatomy. 9th ed. Baltimore: Williams &
3,4-diaminopyridine (3,4,-DAP) blocks a K+ Wilkins, 1996.
channel and increases acetylcholine _____________________________
release. It has been used for treatment of
Lambert-Eaton myasthenic syndrome. Question(s) 98: Anatomy
Discussion:
Unlike the relay and association nuclei of the
Reference: thalamus (dorsal thalamus), the reticular
McEvoy KM, Windebank AJ, Daube R, et al. nucleus (ventral thalamus) does not project
3-4 diaminopyrimidine in the treatment of to the cerebral cortex. It receives inputs
Lambert-Eaton myasthenic syndrome. from the cortex and projects to the other
NEJM 1989;321:1567-1572. thalamic nuclei, and is critical for
_____________________________ thalamocortical synchronization, particularly
Question(s) 95: Physiology generation of sleep spindles.
Discussion:
Absence seizures are characteristically seen Reference:
in children 5-15 years of age associated with Parent A. Carpenter's human
staring and may have automatisms such as neuroanatomy. 9th ed. Baltimore: Williams &
repetitive chewing movements. Similar Wilkins, 1996.
features may be seen in complex partial _____________________________
seizures, although absence seizures are
shorter and may occur more frequently. The Question(s) 99: Pathology
characteristic electroencephalogram during Discussion:
an absence seizure would show 3 Hz spike- Lesions associated with Friedreich ataxia
and-wave discharges. include degeneration of the posterior
columns, corticospinal tracts,
spinocerebellar tracts and dorsal roots.
Reference: Anterior motor neurons are not affected.
Niedermeyer E, Lopes da Silva F.
Electroencephalography. 4th ed. Baltimore:
Lippincott, Williams & Wilkins, 1998. Reference:
_____________________________ Graham DI, Lantos PL. Greenfield's
neuropathology. 6th ed. London: Arnold,
Question(s) 96: Anatomy
1997.
Discussion: _____________________________
The Babinski sign, an abnormal superficial
plantar reflex, is elicited by stroking the sole
of the foot, which is in the S1 dermatome.

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Question(s) 100: Anatomy Question(s) 103:

Discussion: Pharmacology/Chemistry
The most prominent efferent pathway from Discussion:
the amygdaloid nuclear complex is the stria The vainilloid receptor (VR1) is a member of
terminalis. Most of the fibers arise from the the transient receptor potential (TRP) family
corticomedial part of the nuclear complex. of cation channels, which are particularly
They arch along the medial border of the permeable to calcium. The VR1 receptor is
caudate near its junction with the thalamus. activated by capsaicin, noxious heat, and
The largest termination is the nucleus of the acid pH.
stria terminalis. This is located lateral to the
columns of the fornix and dorsal to the
anterior commissure. Other terminations Reference:
include the anterior hypothalamic nuclei and Caterina MJ, Julius D. The vainilloid
the medial preoptic area. receptor: a molecular gateway to the pain
pathway. Annu. Rev. Neurosci 2001;
24:487-517.
Reference: _____________________________
Benarroch EE, Westmoreland BF, Daube Question(s) 104: Physiology
JR, et al. Medical neurosciences - an
approach to anatomy, pathology and Discussion:
physiology by systems and levels. 4th ed. Botulism results in an impaired release of
New York: Lippincott, Williams & Wilkins, acetylcholine from the nerve terminal.
1999.
_____________________________ Reference:
Question(s) 101: Clinical Adult Engel AG, Franzini-Armstrong C. Myology.
Discussion: 2nd ed. New York: McGraw-Hill, 1994.
A lesion of the medial longitudinal fasciculus _____________________________
results in impaired adduction of the Question(s) 105: Behavioral
ipsilateral eye, and not a conjugate deviation Discussion:
of the eyes. Visual hallucinations may be experienced
but are uncommon in the dementia of
Reference: depression, Alzheimer's disease,
Miller NR. Walsh and Hoyt's clinical neuro- progressive supranuclear palsy, Pick's
ophthalmology. 4th ed. Baltimore: Williams disease, and normal pressure
& Wilkins, 1985. hydrocephalus. They are commonly
_____________________________ associated with diffuse Lewy body disease.
Question(s) 102: Clinical Adult
Discussion: Reference:
Takayasu's syndrome is a nonspecific Feinberg TE, Farah MJ. Behavioral
arteritis involving mainly the aorta and the neurology and neuropsychology. New York:
large arteries arising from its arch. Most of McGraw-Hill, 1997.
the patients have been young Asian _____________________________
females. The exact etiology has never been Question(s) 106: Behavioral
ascertained but an autoimmune mechanism Discussion:
has been suspected. Prosopagnosia results from bilateral
occipitotemporal junction lesions. It is often
Reference: associated with achromatopsia because of
Adams RD, Victor M. Principles of involvement of the fibers projecting from the
neurology. 4th ed. New York: McGraw-Hill, inferior lip of the occipital lobe.
1989.
_____________________________

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Reference: Question(s) 110: Neuroradiology

Tranel D. Disorders of color processing Discussion:
(perception, imagery, recognition, and The Chiari type I malformation is defined as
naming). In: Feinberg TE, Farah MJ, editors. congenital displacement of cerebellar tonsils
Behavioral neurology and neuropsychology. (with or without vermian displacement) into
New York: McGraw-Hill, 1997. the cervical spine canal. It is frequently
_____________________________ associated with narrow foramen magnum
Question(s) 107: Pathology and syringohydromyelia (syrinx).
Hydrocephalus is not a common finding.
Discussion:
More than 2-3 mm downward displacement
Normal pressure hydrocephalus may easily
of the tonsils is considered abnormal. The
be mistaken clinically for Binswanger's or
reference point is posterior lip of foramen
Alzheimer's disease, and the latter may co-
magnum. Herniation beyond C3 is rare. The
exist with normal pressure hydrocephalus
4th ventricle locates in its normal position in
but do not cause it. The two most common
the posterior fossa. Spinal dysraphism, open
antecedent conditions that may have
tentorial incisura and narrow tectum are
occurred years before the development of
associated with Chiari II (but not with Chiari I
normal pressure hydrocephalus and
malformation). Chiari I malformation is often
subarachnoid hemorrhage and head injury.
asymptomatic, especially if small. 2-3 mm
herniation is a very common finding in MRI.
Reference: The correct answer is syrinx.
Graham DI, Lantos PL. Greenfield's
neuropathology. 6th ed. London: Arnold, Reference:
1997.
Bakshi R, Lindsay BD, Kinkel PR. Brain
_____________________________ magnetic resonance imaging in clinical
Question(s) 108: Pathology neurology. In: Joynt RJ, Griggs RC, editors.
Discussion: Clinical Neurology. Philadelphia: Lippincott,
The predominant pathological lesion in Williams & Wilkins, 1998;1(4A):1-203.
patients with homocystinuria is _____________________________
thromboembolic disease. Involvement of Question(s) 111: Anatomy
cerebral blood vessels results in infarcts in
Discussion:
the cerebrum, cerebellum, midbrain, and
Cytoplasmic filaments are classified into
thalamus. Thrombi may also be seen in
three categories based on cross sectional
dural venous sinuses. Arterial walls of
diameter: thin (5-7 nm), intermediate (10-12
affected patients may show fibrous intimal
nm), and thick (16 nm). Thin filaments are
thickening.
composed of actin and thick filaments are
composed of myosin. There are several
Reference: different types of intermediate filaments,
Graham DI, Lantos PL. Greenfield's which differ in their protein composition. The
neuropathology. 6th ed. London: Arnold, intermediate filament proteins are: GFAP,
1997. neurofilament proteins, cytokeratins,
_____________________________ desmin, vimentin, and nestin. Microtubules,
which are composed of tubulin, are larger
Question(s) 109: Pathology than thick filaments, with a diameter of 24
Discussion: nm.
Examination of the cortex shows the
characteristic inclusion of myoclonic
epilepsy, the Lafora body. Reference:
Fuller GN, Goodman JC. Practical review of
neuropathology. Philadelphia: Lippincott,
Reference: Williams & Wilkins, 2001.
Graham DI, Lantos PL. Greenfield's _____________________________
neuropathology. 6th ed. London: Arnold,
1997.

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 2002 AAN RITE Discussion & Reference Manual

Question(s) 112: Behavioral Question(s) 115: Pathology

Discussion: Discussion:
Approximately 40-50% of Alzheimer's Autosomal dominant cerebral cavernous
disease patients will experience delusions malformation (CCM1) syndrome occurs in
during the course of their disease. Most Hispanic individuals and is characterized by
common are the persecutory delusions such intraparenchymal cavernous malformations
as theft or infidelity. About ten percent of that can produce seizures, impairment of
patients may experience hallucinations, function, or hemorrhage.
usually visual or auditory. Grandiose
delusions are rare, as are olfactory
hallucinations. Reference:
1. Online Mendelian Inheritance in Man
[Link]
Reference: [Link]
Cummings JL. Treatment of Alzheimer's 2. McKusick VA. Mendelian inheritance in
disease. Clinical cornerstone 2001;3(4):27- man: a catalog of human genes and genetic
39. disorders. 12th ed. Baltimore: Johns
_____________________________ Hopkins University Press, 1998.
Question(s) 113: Pathology _____________________________
Discussion: Question(s) 116: Pathology
Head injury patients with diffuse axonal Discussion:
injury are usually comatose from the time of Meningioangiomatosis may be a cause of
injury and recovery is minimal. seizures or may be an asymptomatic
Experimental studies have shown that the condition. Like multiple meningiomas,
histological hallmark of this condition - meningioangiomatosis is associated with
axonal spheroids or axonal retraction balls - neurofibromatosis type 2.
develop over a period of hours to days.
Reference:
Reference: Kleihues P, Cavenee WK, editors. Pathology
Graham DI, Lantos PL. Greenfield's and genetics of tumors of the nervous
neuropathology. 6th ed. London: Arnold, system. 2nd ed. New York: Oxford
1997. University Press, 2000.
_____________________________ _____________________________
Question(s) 114: Anatomy Question(s) 117: Anatomy
Discussion: Discussion:
The lower midbrain tegmentum contains the The nucleus tractus solitarius is the first
fibers of the superior cerebellar peduncle central relay station for both the
that decussate to reach the contralateral vasodepressor and cardioinhibitory
ventral lateral thalamic nucleus. components of the baroreflex. The dorsal
nucleus of the vagus and particularly the
nucleus ambiguus mediate the
Reference:
cardioinhibitory effect. Inhibition of neurons
Parent A. Carpenter's human of the ventrolateral medullary reticular
neuroanatomy. 9th ed. Baltimore: Williams & formation is involved in the vasodepressor
Wilkins, 1996. response.
_____________________________

Reference:
Benarroch EE, Westmoreland BF, Daube
JR, et al. Medical neurosciences - an
approach to anatomy, pathology and
physiology by systems and levels. 4th ed.
New York: Lippincott, Williams & Wilkins,
1999.

23
 2002 AAN RITE Discussion & Reference Manual

Question(s) 118: Anatomy in the cell, and the amino-terminal fragments

Discussion: containing the glutamine expansions form
The posterior cord splits into the radial and aggregates, visible as nuclear and
axillary nerves. cytoplasmic inclusions in the brain. The
mechanism of neurotoxicity of Htt is not
proven, but several observations have been
Reference: made. Mutant Htt activates caspase, thus
Benarroch EE, Westmoreland BF, Daube promoting apoptosis, and also reduces
JR, et al. Medical neurosciences - an transcription of brain-derived neurotrophic
approach to anatomy, pathology and factor (BDNF), which normally protects
physiology by systems and levels. 4th ed. striatal neurons from glutamate-induced
New York: Lippincott, Williams & Wilkins, excitotoxicity. Wild-type Htt has been shown
1999. to reduce the toxicity of mutant Htt in vivo.
_____________________________
Question(s) 119: Anatomy Reference:
Discussion: 1. Trottier Y, Mandel JL. Huntingtin - profit
The abducens nerve is contained in the and loss. Science 2001;293:445-446.
sheath of the internal carotid artery at the 2. Zaccato C, Ciannola A, Rigamonti D, et
level of the cavernous sinus. The al. Loss of Huntingtin-mediated BDNF gene
oculomotor (III), trochlear (IV) and transcription in Huntington's disease.
ophthalmic (VI) nerves are located in the Science 2001;293:493-498.
lateral wall of the cavernous sinus. The optic _____________________________
nerve occupies the optic foramen but not the Question(s) 122: Clinical Adult
cavernous sinus.
Discussion:
Spinal bulbar muscular atrophy (Kennedy’s
Reference: disease) is characterized by lower motor
Parent A. Carpenter's human neuron findings, especially affecting cranial
neuroanatomy. 9th ed. Baltimore: Williams & musculature associated with decreased
Wilkins, 1996. libido and gynecomastia. Female carriers
_____________________________ are asymptomatic. Muscle biopsy shows
Question(s) 120: Behavioral non-diagnostic neuropathic changes.
Testicular biopsy shows diminished
Discussion: spermatozoids with abnormal motility and
The triad of optic ataxia, ocular apraxia, and mild elevation of FSH and LH. Diagnosis is
simultanagnosia (Balint's syndrome) is by DNA analysis which shows expanded
usually the result of bilateral occipitoparietal CAG repeats translated into polyglutamine
junction lesions. repeats.

Reference: Reference:
Rafal RD. Balint syndrome. In: Feinberg Ashizawa T, Zoghbi HY. Diseases with
TE, Farah MJ, editors. Behavioral neurology trinucleotide repeat expansion. In: Appel SH,
and neuropsychology. New York: McGraw- editor. Current neurology. Volume 17.
Hill, 1997. Amsterdam: IOS Press, 1997.
_____________________________ _____________________________
Question(s) 121:
Pharmacology/Chemistry
Discussion:
Htt is a cytoplasmic protein, highly
expressed in the cortex and striatum, that
contains increased numbers of glutamine
residues in Huntington Disease (HD),
reflecting expansion of CAG repeats in the
Htt gene. Mutant Htt undergoes proteolysis

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Question(s) 123: nociceptive-specific and thermal inputs from

Pharmacology/Chemistry the posterior ventromedial nucleus, which is
the site of termination of spinothalamic
Discussion: pathways arising from lamina I of the dorsal
Astrocyte-derived lactic acid is the main horn.
source of energy to the neuron. During
periods of activity, the neuron releases
glutamate, which is taken up by the Reference:
astrocyte. Astrocyte uptake depends on the Parent A. Carpenter's human
Na,K, ATPase. The fall in ATP levels neuroanatomy. 9th ed. Baltimore: Williams &
activates glycolysis in the astrocyte, with Wilkins, 1996.
production of lactate, which is released. _____________________________
Lactate is taken up by the neuron and
Question(s) 127: Behavioral
converted to pyruvate via the lactate
dehydrogenase, thus providing a source for Discussion:
neuronal aerobic metabolism. Bilateral lesions of the amygdala result in
the Kluver-Bucy syndrome. This syndrome
is seen in herpes simplex encephalitis,
Reference: Pick's disease, anoxic-ischemic lesions in
Magistretti P, Pellerin L. Astrocytes coupled the anterior medial temporal lobes, and after
synaptic activity to glucose utilization in the bilateral temporal lobectomy. It is rarely, if
brain. News Physiol Sci 1999;14:177-182. ever, seen as a manifestation of Creutzfeldt-
_____________________________ Jakob, Alzheimer's or Huntington's disease.
Question(s) 124: Pathology
Discussion: Reference:
Target fibers, which are best seen with Mesulam M. Principles of behavioral and
trichrome or NADH stains, are characteristic cognitive neurology. Philadelphia: FA Davis
of acute denervation. Co., 1999.
_____________________________
Reference: Question(s) 128: Clinical Adult
Graham DI, Lantos PL. Greenfield's Discussion:
neuropathology. 6th ed. London: Arnold, Allodynia is the perception of a non-noxious
1997. stimulus as being painful.
_____________________________
Question(s) 125: Behavioral Reference:
Discussion: Kanner R. Pain management secrets.
Patients with AIDS dementia complex have Philadelphia: Hanley and Belfus, 1997;2.
a subcortical dementia with psychomotor _____________________________
slowing, difficulty concentrating, especially in Question(s) 129: Anatomy
conducting serial 7's, impaired reading, and
forgetfulness. Discussion:
The nucleus ambiguus contains motor
neurons that supply striated muscles of the
Reference: palate, pharynx and larynx.
Navia BA, Jordan BD, Price RW. The AIDS-
dementia complex. In: Clinical features.
Annals of Neurology 1986;19:517-524. Reference:
_____________________________ Parent A. Carpenter's human
neuroanatomy. 9th ed. Baltimore: Williams &
Question(s) 126: Anatomy Wilkins, 1996.
Discussion: _____________________________
The insular cortex receives inputs from the
parvocellular portion of the ventromedial
nucleus of the thalamus, relaying taste and
visceral sensation. In addition, it receives

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 2002 AAN RITE Discussion & Reference Manual

Question(s) 130: Question(s) 133: Clinical Adult

Pharmacology/Chemistry Discussion:
Discussion: Paroxysmal hemicrania is a disorder, more
Cerebral cysticercosis is a common cause of common in women, characterized by
focal seizures in the Hispanic population frequent (7 to 22 per day) episodes of
living in the southern United States. The unilateral severe but short-lasting (five to 45
acute lesions tend to resolve with formation minutes) headaches associated with
of a granuloma, and therefore expectant ipsilateral autonomic manifestations.
treatment with anticonvulsants is suggested. Indomethacin is the treatment of choice.
If the cyst and/or edema persist in the two to
three month follow-up CT or MRI then
cysticidal therapy is indicated. The drugs of Reference:
choice are praziquantel or albendazole. Goadsby PJ, Lipton RB. A review of
Dexamethasone is added to the treatment to paroxysmal hemicranias, SUNCT syndrome
reduce the inflammatory reaction induced by and other short-lasting headaches with
the acute destruction of the parasite. autonomic features, including new cases.
Brain 1997;120:193-209.
_____________________________
Reference:
Question(s) 134: Behavioral
Sotelo J. Cerebral cysticercosis. In: Johnson
RT, Griffin JW, editors. Current therapy in Discussion:
neurologic disease. 5th ed. St. Louis: CV Significant pathological dilatation of the
Mosby, 1997. cerebral ventricular system, especially of the
_____________________________ lateral and third ventricles, has been
demonstrated in schizophrenic patients.
Question(s) 131: Clinical Pediatrics
Discussion:
Hemiplegic cerebral palsy (CHP) is the most Reference:
common CP syndrome among children born Shelton RC, Weinberger DR. X-ray
at term and is most commonly the result of computerized tomography studies in
cerebral infarction. Scoliosis occurs in only schizophrenia: a review and synthesis. In:
10-20% of children with CHP, and is often Nasrableh HA, Weinbreger DR, editors.
mild. Likewise, general growth (height and Handbook of schizophrenia. Amsterdam:
weight) is not impaired, and the head Elsevier Science Publications, 1986;1.
circumference is normal in 85-90%. About _____________________________
20% of children with CHP have learning Question(s) 135: Clinical Adult
disabilities. Discussion:
Amitriptyline relieves pain, but can have
Reference: adverse cardiac effects. Sympathectomy
Neville B, Goodman R. Congenital relieves pain associated with reflex
hemiplegia. London: Mac Keith Press, 2000. sympathetic dystrophy. Gabapentin may
_____________________________ relieve neuropathic pain.
Question(s) 132: Behavioral
Discussion: Reference:
The nonfluent cortical aphasias include Halsey JH. Post-traumatic pain syndromes.
Broca's, global, mixed transcortical, and In: Rowland LR, editor. Merritt's textbook of
transcortical motor. neurology. Baltimore: Williams & Wilkins,
1995.
_____________________________
Reference:
Benson DF. Aphasia, alexia, and agraphia.
New York: Churchill Livingston, 1979.
_____________________________

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 2002 AAN RITE Discussion & Reference Manual

Question(s) 136: Anatomy

Discussion: Reference:
The "frontal eye field" in humans is located Parent A. Carpenter's human
in the caudal part of the middle frontal gyrus. neuroanatomy. 9th ed. Baltimore: Williams &
Wilkins, 1996.
_____________________________
Reference:
Parent A. Carpenter's human Question(s) 140: Clinical Pediatrics
neuroanatomy. 9th ed. Baltimore: Williams & Discussion:
Wilkins, 1996. Seizures, blindness, psychomotor
_____________________________ retardation are cardinal features of Tay-
Question(s) 137: Behavioral Sachs disease. Tay-Sachs disease is an
autosomal recessive disorder in which
Discussion: hexosaminidase A is deficient and
Clinical features of catatonia include hexosaminidase B is increased. The
psychosocial withdrawal, stupor, mutism, disorder is panethnic, but is more frequent in
immobility, generalized analgesia, posturing, Ashkenazy Jews. Prenatal diagnosis is now
waxy flexibility, mannerisms, rituals, available. Hepatosplenomegaly is not a
grimacing, and shoulder shrugging. feature of Tay-Sachs disease, but does
occur in Sandhoff disease (deficiency of
Reference: hexosaminidase A and B). Macrocephaly is
Philbrick KL, Rummans TA. Malignant frequent in GM2 gangliosidoses.
catatonia. J Neuropsychiatry Clin Neurosci
1994;6:1-13. Reference:
_____________________________ Brady RO, Patterson MC. Disorders of lipid
Question(s) 138: metabolism. In: Berg BO, editor. Principles
Pharmacology/Chemistry of child neurology. New York: McGraw-Hill,
Discussion: Co., 1996.
Refsum's disease is a potentially treatable _____________________________
neuropathy due to defective alpha oxidation Question(s) 141: Clinical Adult
of phytanic acid in the microsomes. Diet low Discussion:
in this branched fatty acid (less than 10 The most common visual field defect seen in
mg/d) and, in severe cases, plasma early compressive lesions of the optic nerve
exchange, can lead to improvement of the is a central scotoma in the ipsilateral eye.
neuropathy.

Reference:
Reference: Bradley WG, Daroff RB, Fenichel GM, et al,
Skjelda OH, Stokke O, Refsum S, et al. editors. Neurology in clinical practice. 3rd
Clinical and biochemical heterogeneity in ed. Boston: Butterworth-Heinemann, 2000.
conditions with phytanic acid accumulation.
_____________________________
J Neurol Sci 1987;77:87-96.
_____________________________ Question(s) 142: Neuroradiology
Question(s) 139: Anatomy Discussion:
There is almost 100% association of
Discussion: meningomyelocele with Chiari II (Arnold-
The anterior choroidal artery supplies the Chiari malformation) but not with Chiari I
lateral part of the medial segment of the (tonsillar herniation). Association of midline
globus pallidus, the target of pallidotomy in dysraphism is present, thoraco-lumbar
patients with Parkinson's disease. The kyphoscoliosis and diastematomyelia are
posterior communicating artery supplies the seen in approximately 30%.
medial part of the medial segment.

27
 2002 AAN RITE Discussion & Reference Manual

Reference: Reference:
El Gammel T, et al. MR imaging of Chiari II Kimura J. Electrodiagnosis in disease of
malformation. AJR 1988;150:163-170. nerve and muscle. 3rd ed. New York: Oxford
_____________________________ University Press, 2001.
Question(s) 143: Clinical Pediatrics _____________________________
Discussion: Question(s) 146:
Children with Tourette's syndrome very Pharmacology/Chemistry
frequently have an associated attention Discussion:
deficit disorder and obsessive-compulsive Tolcapone and entacapone increase the
disorder. Coprolalia is quite unusual in duration of clinical response with levodopa.
children. Most children with Tourette's do These drugs act by inhibiting COMT enzyme
not need medication and often symptoms in the periphery and decreasing conversion
subside after adolescence. Motor tics and of levodopa to 3-0-methyl dopa. Tolcapone
vocalizations can be suppressed for varying is associated with a small risk of liver
periods of time, which is helpful in damage and its use requires liver enzyme
distinguishing tics from chorea. The disorder monitoring. Both may result in severe
is felt to be an autosomal dominant disorder diarrhea and cause discoloration of urine.
but expression of the gene is very complex.
Reference:
Reference: Baas H, Beiske AG, Ghika J, et al. COMT
Fenichel GM. Clinical pediatric neurology. inhibition with tolcapone reduces the
Philadelphia: WB Saunders, Co., 1997. “wearing-off” phenomenon and levodopa
_____________________________ requirements in fluctuating parkinsonian
Question(s) 144: Clinical Adult patients. J Neurology Neurosurgery
Psychiatry 1997;63:421-428.
Discussion: _____________________________
Patients with spinal stenosis may have leg
pain on exertion, which is relieved by rest Question(s) 147: Clinical Adult
(neurogenic claudication). This pain is worse Discussion:
with back hyperextension, relieved by Rhinocerebral mucormycosis is a fulminant
leaning forward when walking (e.g. when disease involving the frontal and nasal
using a shopping cart), and often not cavities with direct extension to the cranial
present when riding a bicycle. Absent cavity. Typically, this disease occurs in
peripheral arterial pulses suggests vascular patients with uncontrolled diabetes and
claudication rather than neurogenic invades the juxtaposed cerebrum after local
claudication. spread in the nasal-orbital cavities.

Reference: Reference:
Brazis PW, Masdeu JC, Biller J. Localization Soloniuk DS, Moreland DB. Rhinocerebral
in clinical neurology. 3rd ed. Boston: Little, mucormycosis with extension to the
Brown, 1996. posterior fossa: case report. Neurosurgery
_____________________________ 1988;23:641-643.
Question(s) 145: Physiology _____________________________
Discussion: Question(s) 148: Anatomy
The H-reflex is the electrophysiological Discussion:
equivalent of the monosynaptic tendon The dermatome of the thumb is C6.
stretch reflex at the ankle.
Reference:
Parent A. Carpenter's human
neuroanatomy. 9th ed. Baltimore: Williams &
Wilkins, 1996.

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 2002 AAN RITE Discussion & Reference Manual

Question(s) 149:
Pharmacology/Chemistry Reference:
Discussion: Graham DI, Lantos PL. Greenfield's
Friedreich's ataxia is the most common neuropathology. 6th ed. London: Arnold,
autosomal recessive cerebellar ataxia. It is 1997.
due to an unstable expansion of the GAA _____________________________
repeat in the first intron in the frataxin gene
in chromosme 9q. Frataxin is located in the Question(s) 153: Behavioral
mitochondria, where it appears to exert a Discussion:
protective effect against iron accumulation Galantamine is a therapeutic agent for
and generation of reactive oxygen species. Alzheimer's disease that acts by both
Thus, it has been proposed that Friedreich's inhibiting AChE and allosterically modulating
ataxia is due to oxidative stress due to free nicotinic ACh receptors. Rivastigmine acts
radicals generated in iron-filled by inhibiting AChE and
mitochondria. butyrylcholinesterase. Choline
acetyltransferase is decreased in AD.
Chromosomes 1, 14, 19, 21 and possibly 12
Reference: have been associated with the genetics of
Pandolfo M. Friedreich's ataxia: clinical AD but not 3. Alpha secretase cleaves APP
aspects and pathogenesis. Sem Neurol normally. Beta and gamma secretase have
1999;19:311-321. been implicated in the production of toxic
_____________________________ beta amyloid.
Question(s) 150: Clinical Adult
Discussion: Reference:
Gastric resection is a cause of B12 Cummings JL. Treatment of Alzheimer's
deficiency due to loss of production of disease. Clinical cornerstone 2001;3(4):27-
intrinsic factor. 39.
_____________________________
Reference: Question(s) 154: Clinical Adult
Victor M, Ropper A. Adams and Victor's Discussion:
principles of neurology. 7th ed. New York: Crocodile tears, a sign of aberrant
McGraw-Hill, Co., 2001. regeneration, is most commonly seen in
_____________________________ association with Bell's palsy.
Question(s) 151: Physiology
Discussion: Reference:
Decerebrate rigidity is produced in response Bradley WG, Daroff RB, Fenichel GM, et al.
to a lesion in the brainstem below the level Neurology in clinical practice. 2nd ed.
of the red nucleus. Boston: Butterworth-Heinemann, 1996.
_____________________________
Reference:
Kandel ER, Schwartz JH, Jessel TM.
Principles of neural science. 4th ed. New
York: McGraw-Hill, 2000.
_____________________________
Question(s) 152: Pathology
Discussion:
The nucleus of Onufrowicz of the sacral cord
is spared in motor neuron disease. The
remaining motor nuclei are involved to
varying degrees in ALS.

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 2002 AAN RITE Discussion & Reference Manual

Question(s) 155: Pathology

Discussion: Reference:
Triethyltin produces severe white matter Brown WF, Bolton CF, editors. Clinical
edema due to accumulation of fluid in electromyography. 2nd ed. Boston:
vacuoles within myelin sheaths. There is Butterworth-Heinemann, 1993.
splitting of myelin along intraperiod lines. _____________________________
Other toxic causes of intramyelin edema
Question(s) 159: Anatomy
include hexachlorophene, 5-fluorouracil, and
lithium. Discussion:
The subfornical organ is a circumventricular
structure with no blood-brain-barrier.
Reference: Although the subcommissural organ is
Ellison D, Love S. Neuropathology a circumventricular in nature, it possesses a
reference text of CNS pathology. Chicago: capillary endothelium with tight junctions.
Mosby, 1998.
_____________________________
Reference:
Question(s) 156: Behavioral Parent A. Carpenter's human
Discussion: neuroanatomy. 9th ed. Baltimore: Williams &
A diagnosis of major depression requires the Wilkins, 1996.
presence of five out of nine depressive _____________________________
symptoms listed in DSM IV for at least two
Question(s) 160: Clinical Adult
weeks. The diagnosis suggests a biologic
pathophysiology and is usually an indication Discussion:
for pharmacologic intervention. The most common cause of an isolated
oculomotor (3rd) nerve palsy is an
intracranial aneurysm usually arising from
Reference: the junction of the internal carotid and
American Psychiatric Association. posterior communicating arteries. This is
Psychiatric diagnosis and the diagnostic and especially true if the (3rd) nerve palsy is
statistical manual of mental disorders. 4th "complete" and involves the pupil as well as
ed. DSM-IV, September 1997. the extraocular muscles. When the pupillary
_____________________________ light reflex is spared, ischemia to the
Question(s) 157: Anatomy oculomotor nerve is more likely and is most
commonly associated with underlying
Discussion: diabetes mellitus.
The anterior choroidal artery usually arises
from the internal carotid artery.
Reference:
Lee AG, Brazis PW. Clinical pathways in
Reference: neuro-ophthalmology. New York: Theime,
Parent A. Carpenter's human 1998.
neuroanatomy. 9th ed. Baltimore: Williams &
_____________________________
Wilkins, 1996.
_____________________________
Question(s) 158: Physiology
Discussion:
Motor conduction block, abnormal temporal
dispersion, slowed conduction velocities,
and prolonged late responses are the
electrodiagnostic features of demyelination.

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 2002 AAN RITE Discussion & Reference Manual

Question(s) 161: Pathology Question(s) 164:

Pharmacology/Chemistry
Discussion:
About 90% of patients with idiopathic Discussion:
syringomyelia have Chiari type I Levodopa crosses the blood-brain-barrier
malformation. None of the spinal cord via a transport system shared by neutral
degenerative disorders are strongly amino acids of the diet. Lipid soluble drugs
associated with syringomyelia, nor is readily pass through barrier without a
subacute combined degeneration. transport system.
Megalencephaly is not associated with
syringomyelia. Reference:
Reich SG, DeLong MR. Parkinson's
Reference: disease. In: Johnson R, editor. Current
Graham DI, Lantos PL. Greenfield's therapy in neurologic diseases. BC Decker
neuropathology. 6th ed. London: Arnold, Inc., 1990.
1997. _____________________________
_____________________________ Question(s) 165: Physiology
Question(s) 162: Discussion:
Pharmacology/Chemistry Lennox-Gastaut syndrome is characterized
Discussion: clinically by frequent generalized seizures in
Oxcarbazepine is a derivative of association with mental retardation and a
carbamazepine and shares many similarities slow spike-and-wave EEG pattern.
with carbamazepine, including its
mechanism of action, ability to induce Reference:
hepatic metabolism of oral contraceptives
Niedermeyer E, Lopes da Silva F.
and other drugs, risk of hyponatremia, and
Electroencephalography. 4th ed. Baltimore:
indication for treatment of partial epilepsy.
Lippincott, Williams & Wilkins, 1998.
Unlike carbamazepine, oxcarbazepine is
_____________________________
reduced to 10-monohydroxy-carbamazepine
and does not undergo oxidation to an Question(s) 166: Clinical Pediatrics
epoxide. This may explain its fewer side Discussion:
effects as compared to carbamazepine. The combination of dysconjugate, highly
variable nystagmus, head nodding and head
Reference: tilt without ophthalmologic abnormalities,
and with normal neuroimaging, is diagnostic
Holland KD. Efficacy, pharmacology, and
of spasmus nutans. Latent nystagmus is a
adverse effects of antiepileptic drugs. Neurol
jerk nystagmus that is evoked or enhanced
Clin 2001;19:313-345.
by covering one eye. Congenital nystagmus
_____________________________ is usually conjugate and suppressed by
Question(s) 163: convergence (in contrast to spasmus
Pharmacology/Chemistry nutans, that is typically increased by
Discussion: convergence). Opsoclonus describes
First episode demyelination in presumed chaotic, conjugate saccades, seen
multiple sclerosis should be treated with classically in the paraneoplastic syndrome
beta-interferon. associated with neuroblastoma. Whipple's
disease produces a convergence-
divergence nystagmus with associated
Reference: movements of the muscles of the head and
Jacobs LD, Beck RW, Simon JH, et al. neck (oculomasticatory myorhythmia).
Intramuscular interferon beta-1a therapy
initiated during a first demyelinating event in
multiple sclerosis. CHAMPS Study Group. Reference:
NEJM 2000;343(13):898-904. Leigh RJ, Zee DS. The neurology of eye
_____________________________ movements. 3rd ed. New York: Oxford
University Press, 1999.

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 2002 AAN RITE Discussion & Reference Manual

Question(s) 167:
Pharmacology/Chemistry Reference:
Discussion: Benarroch EE, Westmoreland BF, Daube
Abrupt withdrawal of CNS depressants, JR, et al. Medical neurosciences - an
such as barbiturates and benzodiazepines approach to anatomy, pathology and
that have been used at high doses for physiology by systems and levels. 4th ed.
prolonged periods of time can produce New York: Lippincott, Williams & Wilkins,
seizures and other manifestations of central 1999.
nervous system hyperexcitability that may _____________________________
be lethal. These effects are less common
Question(s) 171: Pathology
with drugs that have high binding to plasma
proteins and long half-life. Discussion:
Ammon's horn sclerosis is seen in chronic
temporal lobe epilepsy and may reflect
Reference: hypoxic or excitotoxic injury to this structure.
Jaffe JH. Drug addictions and drug abuse.
In: Gilman AG, Rall TW, Nies AS, et al,
editors. Goodman and Gilman's the Reference:
pharmacological basis of therapeutics. 8th Graham DI, Lantos PL. Greenfield's
ed. New York: Pergamon Press, 1990. neuropathology. 6th ed. London: Arnold,
_____________________________ 1997.
_____________________________
Question(s) 168: Anatomy
Question(s) 172: Pathology
Discussion:
The little toe's dermatome is S1. Discussion:
CSF oligoclonal bands are seen in nearly all
patients with subacute sclerosing
Reference: panencephalitis and 83-94 percent of
Parent A. Carpenter's human patients with definite multiple sclerosis.
neuroanatomy. 9th ed. Baltimore: Williams & They are also seen in 25-50 percent of
Wilkins, 1996. patients with neuroborreliosis, cryptococcal
_____________________________ meningitis, Guillain-Barre syndrome, and
Question(s) 169: Physiology other conditions. Many patients with
neuromyelitis optica fail to have oligoclonal
Discussion: bands in CSF.
Light sensitivity manifested by
photomyoclonus induced by photic
stimulation can occur with abrupt alcohol Reference:
withdrawal. Henry JB. Clinical diagnosis and
management by laboratory methods.
Philadelphia: Saunders, 1996.
Reference:
_____________________________
Niedermeyer E, Lopes da Silva F.
Electroencephalography. 4th ed. Baltimore: Question(s) 173:
Lippincott, Williams & Wilkins, 1998. Pharmacology/Chemistry
_____________________________ Discussion:
Question(s) 170: Anatomy Aspirin/sustained release dipyridamole
(Aggrenox®) is an effective antiplatelet
Discussion: therapy. The most frequent side effect that
The nucleus of the tractus solitarius (NTS) may limit its use is headache.
contains the first central neuron for the
baroreceptor afferents. Lesions involving the
NTS produce fluctuating hypertension Reference:
mimicking a pheochromocytoma. Hervey PS, Goa KL. Extended-release
dipyridamole/aspirin. [review] Drugs.
1999;58(3):469-75.
_____________________________

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Question(s) 174: Pathology Question(s) 177: Anatomy

Discussion: Discussion:
Laminar necrosis results from The pedunculopontine nucleus (PPN) is an
hypoxic/ischemic injury as may occur in a integral component of the motor circuit of the
large variety of conditions producing basal ganglia. It receives inputs from the
ischemia or hypoxia. Multiple sclerosis may globus pallidus and subthalamic nucleus
involve the cortex with focal demyelination and projects to reticulospinal medullary
best seen with myelin stains. Marchiafava- neurons that control locomotion. There is
Bignami disease is necrosis of the corpus depletion of PPN neurons in Parkinson's
callosum initially associated with disease.
consumption of red wine but subsequently
seen in malnourished, chronically ill
individuals. Hyponatremia is related to Reference:
central pontine myelinolysis. Parent A. Carpenter's human
neuroanatomy. 9th ed. Baltimore: Williams &
Wilkins, 1996.
Reference: _____________________________
Graham DI, Lantos PL. Greenfield's Question(s) 178:
neuropathology. 6th ed. London: Arnold, Pharmacology/Chemistry
1997.
_____________________________ Discussion:
The hypocretin/orexin system of the lateral
Question(s) 175: Pathology hypothalamus has been implicated in the
Discussion: mechanisms of narcolepsy. These neurons
Angioinvasive branching fungal hyphae are project to cholinergic and monoaminergic
characteristic of aspergillosis and cell groups involved in regulation of REM
mucormycisis. In contrast, in blastomycosis, sleep. Reduced CSF levels of hypocretin
coccidioidomycosis, cryptococcosis, and and reduced numbers of hypocretin cells
histoplasmosis rounded yeast forms are have been found in patients with narcolepsy.
seen.
Reference:
Reference: Silber MH, Rye DB. Solving the mysteries of
Fuller GN, Goodman JC. Practical review of narcolepsy: the hypocretins story [editorial].
neuropathology. Philadelphia: Lippincott, Neurology 2001;56:1616-18.
Williams & Wilkins, 2001. _____________________________
_____________________________ Question(s) 179: Behavioral
Question(s) 176: Behavioral Discussion:
Discussion: Problems with interpersonal relationships,
Contusion of the orbitofrontal cortex is fear of abandonment, waxing and waning
associated with social disinhibition. Apathy, between idealizing and devaluing people
depression and loss of task set is more close to them, impulsive outbursts of anger,
commonly seen in dorsolateral prefrontal suicidal gestures, associated depression,
lesions. Akinetic mutism is more commonly and often dissociative states are all
associated with medial frontal lesions. commonly seen in the patient with borderline
personality disorder.
Reference:
Malloy PF, Aloia M. Frontal lobe dysfunction Reference:
in traumatic brain injury. In: McAllister TW, American Psychiatric Association.
Green RL, guest editors. Tucker GJ, editor. Psychiatric diagnosis and the diagnostic and
Neurobehavioral consequences of traumatic statistical manual of mental disorders. 4th
brain injury. Seminars in clinical ed. DSM-IV, September 1997.
neuropsychiatry. Philadelphia: WB _____________________________
Saunders, 1998.
_____________________________

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Question(s) 180: Clinical Adult Question(s) 182: Pathology

Discussion: Discussion:
Human prion diseases include kuru, Critical care myopathy is an under-
sporadic Creutzfeldt-Jakob disease, recognized disorder characterized by
iatrogenic Creutzfeldt-Jakob disease, new selective loss of myosin from myofibers.
variant Creutzfeldt-Jakob disease, Also known as myosin losing myopathy, this
Gerstmann-Straussler-Scheinker disease, disorder is most commonly seen in critically
and fatal familial insomnia. ill patients treated with corticosteroids and
neuromuscular blockers.
Reference:
Mastrianni JA, Roos RP. The prion Reference:
diseases. Seminars in Neurology Ruff RL. Why do ICU patients become
2000;3:337-352. paralyzed? Ann Neurol 1998;43:154-155.
_____________________________ _____________________________
Question(s) 181: Pathology Question(s) 183: Physiology
Discussion: Discussion:
Zellweger's cerebro-hepato-renal syndrome Rapid, irregular, asynchronous movements
presents with hypotonia and cranio-facial of the legs and trunk occurring while
dysmorphic features and the brain shows standing is called orthostatic tremor or
widespread neuronal migration defects, "shaky legs syndrome". This disorder
especially pachygyria and cerebellar usually occurs in middle-aged or elderly
abnormalities. Wolman's disease, due to people and is characterized by feelings of
acid lipase deficiency, presents with unsteadiness in the legs and fear of falling
diarrhea, vomiting, failure to thrive, when standing. Walking is normal and
hepatosplenomegaly and adrenal symptoms are abolished by walking or
calcification, but minimal CNS abnormalities. sitting.
Farber's disease is characterized by painful
swelling of the joints and subcutaneous
nodules; neurons show stored material but Reference:
widespread migrational abnormalities are Brazis PW, Masdeu JC, Biller J. Localization
rarely found in the brain. Pompe's disease in clinical neurology. 3rd ed. Boston: Little,
is primarily a disorder of muscle and Brown, 1996.
presents with hypotonia, and while neurons _____________________________
and astrocytes may show increased storage Question(s) 184: Behavioral
of glycogen, there are no associated Discussion:
migrational disorders in the brain. Krabbe's Patients with dementia of the Alzheimer's
leukodystrophy demonstrates no migrational type most commonly have a fluent aphasia
disorders in the brain. with progressive emptiness in spontaneous
speech, preserved repetition, relatively
Reference: better oral comprehension and diminished
Graham DI, Lantos PL. Greenfield's reading comprehension with better ability to
neuropathology. 6th ed. London: Arnold, read aloud.
1997.
_____________________________ Reference:
Cummings JL, Benson DF. Dementia: a
clinical approach. Boston: Butterworth-
Heinemann, 1992.
_____________________________

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Question(s) 185: Physiology Question(s) 188: Physiology

Discussion: Discussion:
Low frequency sounds are best picked up by Children who develop infantile botulism
the apex of the basilar membrane. typically are normal at birth and develop
normally until the second to fifth month of
life. Hypotonia then develops, accompanied
Reference: by constipation. On examination the patient
Kandel ER, Schwartz JH, Jessel TM. is quite weak and areflexic. Compound
Principles of neural science. 4th ed. New muscle action potential recording in
York: McGraw-Hill, 2000. response to 50 Hertz stimulation produces a
_____________________________ diagnostic incrementing response.
Question(s) 186:
Pharmacology/Chemistry Reference:
Discussion: Kimura J. Electrodiagnosis in disease of
There are several families of neuroactive nerve and muscle. 3rd ed. New York: Oxford
peptides, that include the neurohypophyseal University Press, 2001.
hormones (vasopressin, oxytocin, _____________________________
neurophysin), opioids (opiocortins,
Question(s) 189: Clinical Adult
enkephalins, dynorphins, FMRFamide),
tachykinins (substance P, physalaemin, Discussion:
kassinin, uperolein, eledoisin, bombesin, Patients that have suffered severe head
substance K), secretins (secretin, glucagon, injury with basilar skull fracture are at
VIP, GIP, GHRF, peptide histidine increased risk for the development of
isoleucineamide), insulins (insulin, IGF 1 diabetes insipidus (DI). Serum sodium
and 2), somatostatins (somatostatins, should be followed carefully in patients who
pancreatic polypeptide) and gastrins are potential organ donors following severe
(gastrin, chlecystokinin). head injury since failure to recognize and
treat DI can render donor organs unusable
due to damage from hypotension and
Reference: hypernatremia.
Schwartz JH. Neurotransmitters. In: Kandel
ER, Schwartz JH, Jessell TM, editors.
Principles of neural science. 4th ed. New Reference:
York: McGraw-Hill, 2000;280-297. Narayan RK, Wilberger JE Jr., Povlishock
_____________________________ JT. Neurotrauma. New York: McGraw-Hill,
1996.
Question(s) 187: Clinical Pediatrics _____________________________
Discussion: Question(s) 190: Pathology
Infantile botulism usually presents between
3 and 18 weeks of age. The disease is Discussion:
caused by the C. botulinum toxin which In tabes dorsalis, the degeneration is
blocks acetylcholine release. Clinical confined to the dorsal columns, whereas in
features include constipation, hypotonia, AIDS myelopathy, amyotrophic lateral
areflexia, poor suck, impaired pupillary sclerosis, Friedreich's ataxia, and pernicious
response to light and ophthalmoplegia. The anemia, degeneration occurs in other tracts.
infants are often breast-fed. Diagnosis is
made by EMG with repetitive nerve Reference:
stimulation, causing an incremental
Graham DI, Lantos PL. Greenfield's
response and isolation of C. botulinum toxin
neuropathology. 6th ed. London: Arnold,
in the stool.
1997.
_____________________________
Reference:
Menkes JH, Sarnat HB, editors. Child
neurology. 6th ed. Philadelphia: Lippincott
Williams & Wilkins, 2000.

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Question(s) 191: Neuroradiology Question(s) 194:

Discussion: Pharmacology/Chemistry
The posterior cerebral artery supplies parts Discussion:
of the temporal lobe, parietal lobe, occipital Wernicke's encephalopathy may be
lobe, part of the thalamus, midbrain, choroid precipitated by a carbohydrate load in the
plexus and ependyma of third and lateral face of chronic, severe malnutrition. The
ventricles, but not the globus pallidus. characteristic clinical symptoms include
mental status changes, ophthalmoplegia,
nystagmus and ataxia. Parenteral thiamine
Reference: can produce dramatic improvement.
Caplan LR. Stroke: a clinical approach.
Butterworth-Heinemann, 1995.
_____________________________ Reference:
Question(s) 192: Mancall EL. Nutritional disorders of the
Pharmacology/Chemistry nervous system. In: Aminoff MJ, editor.
Neurology and general medicine. New
Discussion: York: Churchill Livingston, 1989.
Ethambutol is the drug most frequently _____________________________
associated with toxic optic neuropathy.
Intoxication most commonly occurs with Question(s) 195:
dosages of at least 25 mg/kg/d, and may be Pharmacology/Chemistry
more common in patients with renal Discussion:
tuberculosis. Visual loss is insidious and Mirtazapine is a new antidepressant drug
usually symmetric. Yellow-green that blocks the serotonin 5HT2A and the
dyschromatopsia is the earliest symptom. histamine H1 receptors. Blockade of H1
There is no treatment other than withdrawal receptors accounts for sedation, drowsiness,
of the drug. and weight gain that occur with this and
other antidepressants such as amitriptyline
and doxepin.
Reference:
Miller NR, Newman NJ. Walsh & Hoyt's
clinical neuro-ophthalmology: the essentials. Reference:
5th ed. Philadelphia: LIppincott Williams & Richelson E. Pharmacology of
Wilkins, 1999. antidepressants. Mayo Clin Proc. 2001:511-
_____________________________ 27.
Question(s) 193: Behavioral _____________________________
Discussion: Question(s) 196: Behavioral
Prosopagnosia (in which visual perception is Discussion:
intact but there is an impaired ability to Korsakoff amnestic syndrome causes
recognize the identity of the perceived figure impairment in declarative memory
by vision alone) is nearly always associated (anterograde amnesia) and forgetting of
with bilateral lesions involving the recent events (retrograde amnesia) with
occipitotemporal junctional area. sparing of motor memory and semantic
memory (memory for meaning of words).
Digit span remains normal in this syndrome.
Reference:
Trand D. Functional neuroanatomy. In:
Yuodfsky SC, Hales RE, editors. America Reference:
psychiatric press textbook of Squire LR. Memory and brain. New York:
neuropsychiatry, 1992. Oxford Press, 1987.
_____________________________ _____________________________

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Question(s) 197: Physiology Reference:

1. Graham DI, Lantos PL. Greenfield's
Discussion:
neuropathology. 6th ed. London: Arnold,
Periodic limb movements of sleep (nocturnal
1997.
myoclonus) occur during light sleep.
2. Esiri MM, Morris JH. The neuropathology
of dementia. Cambridge: Cambridge
Reference: University Press, 1997.
Niedermeyer E. Electroencephalography. _____________________________
4th ed. Philadelphia: Lippincott, Williams Question(s) 201: Pathology
and Wilkins, 1999.
_____________________________ Discussion:
Malignant hyperthermia is a potential
Question(s) 198: Behavioral complication seen in patients with central
Discussion: core myopathy associated with the
Depression is the most common psychiatric administration of certain anesthetic drugs
disorder in epilepsy; in one study depression such as succinylcholine and halothane.
accounted for 80% of psychiatric hospital
admissions of epileptics.
Reference:
Graham DI, Lantos PL. Greenfield's
Reference: neuropathology. 6th ed. London: Arnold,
Mendez MF, Cummings JL, Benson DF. 1997.
Depression in epilepsy. Arch Neurol _____________________________
1986;43:766-770. Question(s) 202: Physiology
_____________________________
Discussion:
Question(s) 199: Neuroradiology The short head of the biceps femoris muscle
Discussion: is the only muscle proximal to the knee to
Enhancing meninges is a nonspecific receive innervation via the peroneal division
finding, and can be seen with a variety of of the sciatic nerve.
findings, including granulomatous diseases
of the meninges, meningitis (usually
Reference:
bacterial more than viral), prior hemorrhage
or surgery, metastatic meningeal disease Kimura J. Electrodiagnosis in disease of
and idiopathic intracranial hypotension. It is nerve and muscle. 3rd ed. New York: Oxford
not seen in pseudotumor cerebri, in which University Press, 2001.
imaging studies are typically normal. _____________________________
Question(s) 203: Physiology
Reference: Discussion:
Lee S, et al, editors. Cranial MRI and CT. Large motor unit potentials result from
3rd ed. McGraw-Hill, 1992. collateral sprouting.
_____________________________
Question(s) 200: Pathology Reference:
Discussion: Kimura J. Electrodiagnosis in disease of
Choline acetyltransferase is the synthetic nerve and muscle. 3rd ed. New York: Oxford
enzyme for acetylcholine, and is a marker of University Press, 2001.
the axonal termini of cholinergic neurons. _____________________________
The neurons of the basal nucleus are the
major cholinergic projections to the cerebral
cortex, and with depopulation of this
projection nucleus, there is a corresponding
reduction in cortical choline
acetyltransferase.

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Question(s) 204: Anatomy Reference:

Misulis K. Spehlmann's evoked potential
Discussion:
primer. Amsterdam: Elsevier Science
The dorsal columns of the spinal cord
Publications, 1994.
constitute part of a highly specific sensory
pathway with respect to place. _____________________________
Question(s) 208: Pathology
Reference: Discussion:
Parent A. Carpenter's human Hallervorden-Spatz disase is a progressive
neuroanatomy. 9th ed. Baltimore: Williams & neurodegenerative disease with
Wilkins, 1996. neuroaxonal dystrophy, rust-brown
discoloration of globus pallidus and pars
_____________________________
reticularis of substantia nigra due to
Question(s) 205: Clinical Adult accumulation of iron-containing pigment,
Discussion: and an onset usually before age 15 years.
About 1% of patients with strokes due to Nasu-Hakola disease is characterized by
vertebrobasilar occlusive disease have repreated bone fractures, bone cysts, and
sudden bilateral hearing impairment. mineralization of the basal ganglia.

Reference: Reference:
Huang MH, Huang CC, Ryu SJ, et al. Graham DI, Lantos PL. Greenfield's
Sudden bilateral hearing impairment in neuropathology. 6th ed. London: Arnold,
vertebrobasilar occlusive disease. Stroke 1997.
1993;24:132-137. _____________________________
_____________________________ Question(s) 209: Clinical Adult
Question(s) 206: Pathology Discussion:
Discussion: The most common cognitive deficit after a
Rosenthal fibers are opaque, homogeneous, closed head injury is impairment of memory.
eosinophilic structures composed of
compressed GFAP intermediate filaments Reference:
that are commonly associated with slow
Capruso DX, Levin HS. Cognitive
growing low grade tumors, including pilocytic
impairment following closed head injury.
astrocytoma, pleomorphic
Neurology Clinics 1992;10(4):879.
xanthoastrocytoma, and ganglion cell
tumors. They may be seen adjacent to any _____________________________
chronic compressive process and are Question(s) 210: Pathology
abundant in Alexander disease (which in a Discussion:
large number of cases occurs secondary to Onion bulbs are the result of repeated
a GFAP gene mutation). episodes of demyelination and remyelination
and are composed of concentric rings of
Reference: Schwann cells. They are prominent in
1. Graham DI, Lantos PL. Greenfield's demyelinating neuropathies such as
neuropathology. 6th ed. London: Arnold, Charcot-Marie-Tooth disease.
1997.
2. Fuller GN, Burger PC. Central nervous Reference:
system. In: Sternberg SS, editor. Histology Graham DI, Lantos PL. Greenfield's
for pathologists. 2nd ed. Philadelphia: neuropathology. 6th ed. London: Arnold,
Lippincott-Raven, 1997. 1997.
_____________________________ _____________________________
Question(s) 207: Physiology
Discussion:
The P-9 of the somatosensory evoked
potential originates in the brachial plexus.

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Question(s) 211: Pathology

Discussion: Reference:
The current World Health Organization Kaplan HI, Sadock BJ. Pocket handbook of
(WHO 2000) criteria constitute one of three psychiatric drug treatment. 2nd ed.
grading systems for the fibrillary (diffuse) Baltimore: Williams & Wilkins, 1996.
astrocytomas that are currently used _____________________________
internationally. The other two systems are
Question(s) 214: Anatomy
the St. Anne/Mayo classification and the
modified Ringertz system. Under the WHO Discussion:
2000 criteria, diffuse astrocytomas that The central nucleus of the inferior colliculus,
exhibit nuclear atypia and marked mitotic medial geniculate body, nucleus of the
activity are classified as anaplastic trapezoid body, and superior olivary nucleus
astrocytomas (WHO grade III). If either are part of the auditory pathway. The
vascular proliferation or necrosis had been inferior olive is not.
present, the neoplasm would be upgraded to
glioblastoma (WHO grade IV), but these Reference:
features were not seen in this case. Parent A. Carpenter's human
neuroanatomy. 9th ed. Baltimore: Williams &
Reference: Wilkins, 1996.
1. Kleihues P, Cavenee WK, editors. _____________________________
Pathology and genetics of tumors of the Question(s) 215:
nervous system. 2nd ed. New York: Oxford Pharmacology/Chemistry
University Press, 2000.
Discussion:
2. Fuller GN, Goodman JC. Practical review Astrocytes contain glutamine synthase, and
of neuropathology. Philadelphia: Lippincott ATP-dependent enzyme that utilizes
Williams & Wilkins, 2001. glutamate and ammonia (NH3) to produce
_____________________________ glutamine. Therefore, astrocytes have two
Question(s) 212: Anatomy important functions (1) detoxification of NH3;
Discussion: and (2) providing glutamine to the neurons
A lateral medullary stroke spares the as a substrate for glutamate production by
hypoglossal nucleus, medullary pyramid and action of glutaminase.
the medial longitudinal fasciculus.
Reference:
Reference: Benarroch EE, Westmoreland BF, Daube
Brazis PW, Masdeu JC, Biller J. Localization JR, et al. Medical neurosciences - an
in clinical neurology. 3rd ed. Boston: Little, approach to anatomy, pathology and
Brown, 1996. physiology by systems and levels. 4th ed.
_____________________________ New York: Lippincott, Williams & Wilkins,
1999.
Question(s) 213: Behavioral _____________________________
Discussion: Question(s) 216: Physiology
Quetiapine is an effective antipsychotic with
very little dopaminergic blocking activity. Discussion:
Hence, it is useful in Parkinson's disease Triceps, anconeus and pronator teres (C6-
patients with hallucinations or delusions. It C7) have significant contributions from the
acts predominantly on serotonin receptors. C-7 root.
Risperidone, perphenazine, thioridazine,
and haloperidol all have more dopamine Reference:
blocking properties than quetiapine. Brazis PW, Masdeu JC, Biller J. Localization
in clinical neurology. 3rd ed. Boston: Little,
Brown, 1996.
_____________________________

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Question(s) 217: Pathology Reference:

Doody RS. Treatment of Alzheimer's
Discussion:
disease. The Neurologist 1997;3(6):333-
Anencephaly is convincingly associated with
343.
maternal folate deficiency and reduced rates
of this congenital abnormality occur in _____________________________
women given folate. The thymus is Question(s) 221: Physiology
enlarged in anencephalics due to lack of Discussion:
adrenal driven involution, which in turn Sleep spindles are generated by the
results from absence or derangement of the reticular nucleus of the thalamus.
hypothalamic-pituitary axis. The risk of
anencephaly in subsequent pregnancies is
5-7%, females are more commonly afflicted, Reference:
and there is no compelling evidence of a Niedermeyer E, Lopes da Silva F.
role of maternal exposure to toxins in the Electroencephalography. 4th ed. Baltimore:
genesis of this condition. Lippincott, Williams & Wilkins, 1998.
_____________________________
Reference: Question(s) 222: Clinical Adult
Graham DI, Lantos PL. Greenfield's Discussion:
neuropathology. 6th ed. London: Arnold, Normal people can markedly supress their
1997. vestibulo-occular reflexes and decrease
_____________________________ most kinds of nystagmus by fixating on an
Question(s) 218: Anatomy object. Failure of fixation to supress
nystagmus implies a central nervous system
Discussion: lesion.
The prosencephalon divides into the
diencephalon and the telencephalon. The
diencephalon gives rise to the thalamus and Reference:
hypothalamus. The telencephalon gives rise Baloh RW. Dizziness, hearing loss, and
to the striatum and cerebral cortex. tinnitus. Philadelphia: FA Davis, 1998;75.
_____________________________
Reference: Question(s) 223: Pathology
Parent A. Carpenter's human Discussion:
neuroanatomy. 9th ed. Baltimore: Williams & Tomaculous neuropathy (also known as
Wilkins, 1996. pressure sensitive neuropathy) is
_____________________________ characterized by focal hypermyelination with
Question(s) 219: Behavioral redundant myelin folds best seen on teased
nerve preparations. Afflicted individuals
Discussion: have susceptibility to pressure palsies
Nefazodone does not suppress REM sleep. following relatively trivial compression.
It actually may increase REM sleep.

Reference:
Reference: 1. Graham DI, Lantos PL. Greenfield's
Kaplan HI, Sadock BJ. Pocket handbook of neuropathology. 6th ed. London: Arnold,
psychiatric drug treatment. 2nd ed. 1997.
Baltimore: Williams & Wilkins, 1996. 2. Midroni G, Bilbao JM. Biopsy diagnosis of
_____________________________ peripheral neuropathy. Boston: Butterworth-
Question(s) 220: Behavioral Heinemann. 1995.
Discussion: _____________________________
The apolipoprotein E (APOE) genotype
associated with the greatest risk of
developing Alzheimer's disease is the 4/4
allele.

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Question(s) 224: Clinical Pediatrics Reference:

Berg BO, editor. Principles of child
Discussion:
neurology. New York: McGraw-Hill, 1996.
Night terrors (pavor nocturnus) in children
are an arousal during slow-wave sleep and _____________________________
characteristically occur during the first half of Question(s) 227: Pathology
the night 30 minutes after onset of sleep. Discussion:
The child often cries out, is agitated and is Patients with tuberous sclerosis have a
uncommunicative. Treatment is not variety of systemic and central nervous
necessary, but often the episodes are system tumors. In the brain, astrocytic
confused with nocturnal complex partial hamartomas called tubers and candle
seizures and sedative medication can gutterings are present. Subependymal giant
temporarily suppress the episodes. cell astrocytomas are true neoplasms arising
in the vicinity of the foramen of Monro.
Reference: These tumors may produce CSF
Pellock JM, Dodson WE, Bourgeois BFD, obstruction, and they may rarely undergo
editors. Pediatric epilepsy: diagnosis and malignant degeneration.
therapy. 2nd ed. New York: Demos, 2001.
_____________________________ Reference:
Question(s) 225: Behavioral Kleihues P, Cavenee WK, editors. Pathology
and genetics of tumors of the nervous
Discussion:
system. 2nd ed. New York: Oxford
Catatonia may be seen in a number of
University Press, 2000.
medical, neurological, and psychiatric
conditions. Of the psychiatric causes, _____________________________
bipolar disorder is most common. Patients Question(s) 228:
may present with a host of clinical features Pharmacology/Chemistry
including akinetic mutism, catalepsy, waxy Discussion:
flexibility, echopraxia and echolalia, The trigeminal ganglion cells, like other
utilization behavior, and despite extreme primary nociceptive neurons, contain
negativism may manifest automatic obedient glutamate and several neuropeptides,
behavior. When the patient is severely including substance P, neurokinin A, and
impaired, ECT is a treatment of choice. IV calcitonin gene related peptide. These
benzodiazepines such as lorazepam may peptides not only potentiate nociceptive
also be beneficial. transmission in the trigeminal nucleus
caudalis, but also elicit neurogenic
Reference: inflamation with vasodilation and edema.
Taylor M. The fundamentals of clinical Triptans, acting via presynaptic 5-HT1D
neuropsychiatry. New York: Oxford receptors, inhibit release of these
University Press, 1999. neuropeptides.
_____________________________
Question(s) 226: Clinical Pediatrics Reference:
Goadsby PJ, Edvinsson L. Sumatriptan
Discussion:
reverses the changes in calcitonin gene-
A partial third nerve palsy associated with
related peptide seen in the headache phase
headache in young children is most
of migraine. Ann Neurol 1993;33:48-56.
commonly due to ophthalmoplegic migraine.
Cerebral aneurysms presenting as a partial _____________________________
third nerve palsy would be extremely
unusual in young children. Myasthenia
gravis does not present with pain and
pupilary involvement would be unusual.
Guillain-Barre, Miller Fisher Syndrome, and
a brain stem tumor would have other
findings on careful neurologic examination.

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Question(s) 229: Physiology Question(s) 232:

Discussion: Pharmacology/Chemistry
The utricle and saccule of the vestibular Discussion:
system are associated with linear Like other projection neurons in relay
acceleration. systems of the brain, neurons of the nucleus
gracilis projecting to the thalamus use L-
glutamate.
Reference:
Kandel ER, Schwartz JH, Jessel TM.
Principles of neural science. 4th ed. New Reference:
York: McGraw-Hill, 2000. Benarroch EE, Westmoreland BF, Daube
_____________________________ JR, et al. Medical neurosciences - an
Question(s) 230: Behavioral approach to anatomy, pathology and
physiology by systems and levels. 4th ed.
Discussion: New York: Lippincott, Williams & Wilkins,
Hypoperfusion in the left internal carotid 1999.
artery distribution causes watershed infarcts _____________________________
affecting the borderzone area of the middle
and anterior cerebral arteries, and the Question(s) 233: Clinical Adult
posterior borderzone between the middle Discussion:
and posterior cerebral arteries. The patient's In benign paroxysmal positional vertigo,
aphasia is a mixed transcortical aphasia, there is a latent period before the
manifested by absent spontaneous speech development of vertigo and nystagmus
and impaired comprehension. Intact (vertical, torsional, or a combination) when
repetition is secondary to sparing of the the affected ear is in the down position on
presylvian area. Hallpike-Dix testing, and resolution of
symptoms within 60 seconds. The diagnosis
is based on history and physical, with no
Reference: need for further diagnostic testing in
Cummings JL. Clinical neuropsychiatry. New straightforward cases. Canalith repositioning
York: Grune & Stratton, Inc., 1985. treatment and cupololith repositioning
_____________________________ treatment both produce remission in 60-70%
Question(s) 231: Behavioral of cases, and improvement in an additional
Discussion: 20-30% of cases.
Factitious disorder is defined as a syndrome
of intentional production of psychological or Reference:
physical symptoms in the absence of Troost BT, Patton JM. Exercise therapy for
external incentives but in the presence of a positional vertigo. Neurology 1992;42:1441-
psychological need to assume the sick role. 1444.
When there are external incentives for the _____________________________
behavior then "malingering" is the likely
diagnosis. Question(s) 234: Anatomy
Discussion:
In the developing neural tube, the basal
Reference: plate becomes anterior horn gray matter and
American Psychiatric Association. the alar plate becomes the posterior horn
Psychiatric diagnosis and the diagnostic and gray matter.
statistical manual of mental disorders. 4th
ed. DSM-IV, September 1997.
_____________________________ Reference:
Parent A. Carpenter's human
neuroanatomy. 9th ed. Baltimore: Williams &
Wilkins, 1996.
_____________________________

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Question(s) 235: Physiology Question(s) 238: Physiology

Discussion: Discussion:
3 Hz slow wave activity can be seen as a Juvenile myoclonic epilepsy typically begins
normal finding during hyperventilation in a in adolescence. The EEG is characterized
child. Simple absence seizures are by frontally predominant, 4-6 Hz generalized
associated with 3 Hz spike and wave spike-and-wave discharges. Myoclonic and
discharges during hyperventilation. generalized seizures are the predominant
seizure types. Absence seizures occur in
approximately one-third of patients. The
Reference: disorder is often familial, and patients are
Daly DD, Pedley TA. Current practice of cognitively normal.
clinical electroencephalography. 2nd ed.
New York: Raven Press, 1990.
_____________________________ Reference:
Question(s) 236: Anatomy Wyllie E, editor. The treatment of epilepsy:
principles and practice. Philadelphia: Lea
Discussion: and Febiger, 1993.
Normal CSF pressure (lumbar) measured in _____________________________
recumbent position is 10 to 15 cm H20.
Question(s) 239: Physiology
Discussion:
Reference: With acute (less than 1 week) nerve root
Parent A. Carpenter's human compression, the only EMG abnormality
neuroanatomy. 9th ed. Baltimore: Williams & may be a reduced recruitment of motor unit
Wilkins, 1996. potentials.
_____________________________
Question(s) 237:
Reference:
Pharmacology/Chemistry
Brown WF, Bolton CF, editors. Clinical
Discussion: electromyography, 2nd ed. Boston:
Apoptosis is a mechanism of cell death that Butterworth-Heinemann, 1993.
may occur in many neurodegenerative _____________________________
disorders. It depends on activation of
caspases and is characterized by chromatin Question(s) 240: Pathology
condensation and cell desintegration in the Discussion:
absence of inflammation. Apoptosis may be When nerve cells undergo rapid death, the
triggered by exogenous signals such as cell bodies and proximal dendrites are
activation of "death receptors" by the Fas- phagocytized by cells derived from
ligand, by p53 in reponse of DNA damage, microglia, which give rise to macrophages.
or by release of mitochondrial cytochrome c,
which activates an apoptosis activating
factor (Apaf-1) and then the caspase Reference:
cascade. Release of cytochrome c is 1. Graham DI, Lantos PL. Greenfield's
inhibited by Bcl-2, one of the antiapoptotic neuropathology. 6th ed. London: Arnold,
members of the Bcl-2 family. Binding of the 1997.
proapoptotic member Bax to Bcl-2 prevents 2. Fuller GN, Burger PC. Central nervous
its protective effect and triggers apoptosis. system. In: Sternberg SS, editor. Histology
for pathologists. 2nd ed. Philadelphia:
Lippincott-Raven, 1997.
Reference: _____________________________
Thompson CA. Apoptosis in the
pathogenesis and treatment of disease.
Science 1995;267:1456-62.
_____________________________

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Question(s) 241: Question(s) 244: Physiology

Pharmacology/Chemistry Discussion:
Discussion: Polymorphic delta activity is produced by
Nifedipine and other calcium channel processes involving subcortical white matter.
blockers can cause increased weakness in
myasthenic patients. A variety of drugs Reference:
induce or worsen myasthenic symptoms
Daly DD, Pedley TA. Current practice of
through actions on pre- or post-synaptic
clinical electroencephalography. 2nd ed.
mechanisms. Certain antibiotics impair
New York: Raven Press, 1990.
transmitter release by interfering with
_____________________________
calcium flux. Other drugs affect metabolism
of acetylcholine or cholinergic receptor Question(s) 245: Clinical Adult
function. Bromocriptine, chloramphenicol, Discussion:
fluoxetine and gabapentin do not adversely Aseptic meningitis, cranial neuritis, and
affect the myasthenic patient. radiculoneuritis are the most characteristic
neurologic complications of the second
Reference: stage of Lyme disease.
Sanders DB, Howard JF Jr. Disorders of
neuromuscular transmission. In: Bradley Reference:
WG, Daroff RB, Fenichel GM, et al, editors. Solbrig MV, Healy JF, Jay CA. Infection of
Neurology in clinical practice. 3rd ed. the nervous system. A. Bacterial infections.
Boston: Butterworth-Heinemann, 2000. In: Bradley WG, Daroff RB, Fenichel GM, et
_____________________________ al, editors. Neurology in clinical practice.
Question(s) 242: Pathology 3rd ed. Boston: Butterworth-Heinemann,
2000
Discussion:
_____________________________
In patients with meningeal carcinomatosis,
the CSF protein is usually elevated and the Question(s) 246: Pathology
glucose is occasionally reduced. Discussion:
Paraneoplastic cerebellar degneration is
Reference: most commonly associated with ovarian
carcinoma and some breast tumors. It can
Nelson JS, Parisi JE, Schochet SS.
also be seen with Hodgkin's lymphoma.
Principles and practice of neuropathology.
St. Louis: Mosby, 1993.
_____________________________ Reference:
Question(s) 243: Clinical Pediatrics Graham DI, Lantos PL. Greenfield's
neuropathology. 6th ed. London: Arnold,
Discussion: 1997.
Zellweger syndrome presents in infancy,
_____________________________
and affected individuals have characteristic
facial features with high forehead, Question(s) 247: Physiology
hypoplastic supraorbital ridges and midface Discussion:
hypoplasia. Severe weakness, hypotonia Intermittent generalized slow wave bursts
and seizures are common. Diagnosis is can be seen as a normal feature of the EEG
confirmed by elevated levels of saturated in children during drowsiness and
and unsaturated very long-chain fatty acids hyperventilation.
in body fluids.

Reference:
Reference: Daly DD, Pedley TA. Current practice of
Menkes JH, Sarnat HB, editors. Child clinical electroencephalography. 2nd ed.
neurology. 6th ed. Philadelphia: Lippincott New York: Raven Press, 1990.
Williams & Wilkins, 2000. _____________________________
_____________________________

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 2002 AAN RITE Discussion & Reference Manual

Question(s) 248: Behavioral interacts with actin and is critically involved

in the early phases of migration.
Discussion:
Olanzapine is an atypical antipsychotic that
frequently causes significant weight gain. Reference:
Quetiapine, risperidone, haloperidol, and Gleeson JG, Walsh CA. Neuronal migration
molindone are less likely to do so. disorders: from genetic disease to
developmental mechanisms. Trends
Neurosci 2000;22:402-410.
Reference:
_____________________________
Puzantian T, Stimmel G. Review of
psychotropic drugs. New York: McMahon, Question(s) 252: Clinical Adult
2001. Discussion:
_____________________________ The classic syndrome of vitamin A toxicity is
Question(s) 249: Physiology pseudotumor cerebri, with headache,
papilledema, and occasionally abducens
Discussion: palsy.
Renshaw cells are interneurons that have a
direct input to alpha motoneurons.
Reference:
Bradley WG, Daroff RB, Fenichel GM, et al.
Reference: Neurology in clinical practice. 2nd ed.
Kandel ER, Schwartz JH, Jessel TM. Boston: Butterworth-Heinemann, 1996.
Principles of neural science. 4th ed. New
_____________________________
York: McGraw-Hill, 2000.
_____________________________ Question(s) 253: Physiology
Question(s) 250: Pathology Discussion:
The most common EEG finding in patients
Discussion: who have narcolepsy is sleep onset REM
Meningiomas are associated with sleep.
monosomy 22, leading to loss of a tumor
suppressor gene (merlin). Even when the
genetic loss is not as gross as loss of an Reference:
entire chromosome, there is a loss of Kryger MH, Roth T, Dement WC. Principles
heterozygosity for this locus. Meningiomas, and practice of sleep medicine. 2nd ed.
especially spinal, are much more common in Philadelphia: WB Saunders Co., 1994.
women. They constitute one fourth to one _____________________________
third of all primary intraspinal tumors. There Question(s) 254: Anatomy
is a marked gender asymmetry in
meningiomas of the spinal cord with a 10- Discussion:
20:1 female:male ratio. The superior olivary nuclei are part of the
brain stem auditory pathways and are
involved in the function of the 8th cranial
Reference: nerve.
Graham DI, Lantos PL. Greenfield's
neuropathology. 6th ed. London: Arnold,
1997. Reference:
_____________________________ Benarroch EE, Westmoreland BF, Daube
JR, et al. Medical neurosciences - an
Question(s) 251: approach to anatomy, pathology and
Pharmacology/Chemistry physiology by systems and levels. 4th ed.
Discussion: New York: Lippincott, Williams & Wilkins,
Mutations of the FIL-1 gene in chromosome 1999.
Xq28, encoding for filamin-1, produce _____________________________
periventricular heterotopy. This reflects a
complete failure of neuronal migration that
may cause seizures. It is dominant in
females and lethal in males. Filamin-1

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Question(s) 255: Question(s) 258: Clinical Adult

Pharmacology/Chemistry Discussion:
Discussion: Autoantibodies to GAD (glutamic acid
Homocystinuria is inherited as an autosomal decarboxylase) may occur with stiff man
recessive disorder due to deficiency of (stiff person) syndrome.
cystathionine synthase. It is associated with
long limbs, arachnodactyly, and downward Reference:
dislocation of the lens (ectopia lentis).
Barker RA, Revesz T, Thom M, et al.
Approximately 50% of the patients have
Review of 23 patients affected by the stiff
mental retardation. Most neurologic features
man syndrome: clinical subdivision into stiff
result from cerebral thromboembolic
trunk (man) syndrome, stiff limb syndrome,
disease. Approximately 40% of the patients
and progressive encephalomyelitis with
respond to pyridoxine.
rigidity. J Neurol Neurosurg Psychiatry
1998;65.
Reference: _____________________________
Bradley WG, Daroff RB, Fenichel GM, et al, Question(s) 259: Physiology
editors. Neurology in clinical practice. 3rd
Discussion:
ed. Boston: Butterworth-Heinemann, 2000.
Hyperkalemic periodic paralysis is inherited
_____________________________ in an autosomal dominant pattern. A distinct
Question(s) 256: familial form has prominent clinical and
Pharmacology/Chemistry electromyographic evidence of myotonia as
Discussion: an associated feature.
Cerebral autosomal dominant arteriopathy
with cerebral infarcts and Reference:
leukoencephalopathy (CADASIL) is due to a
Brown WF, Bolton CF, editors. Clinical
mutation of the Notch-3 gene, which
electromyography. 2nd ed. Boston:
encodes for a protein that is critically
Butterworth-Heinemann, 1993.
involved in neural determination and
_____________________________
neuritogenesis. Notch is a transmembrane
receptor that is internalized after cleavage of Question(s) 260: Clinical Adult
its cytoplasmic domain by presenilin. Notch Discussion:
is then translocated to the nucleus where it In order to standardize terminology around
controls expression of a variety of the world, the World Health Organization
transcription factors. The pathogenic defines impairment as the clinical signs and
relationship between Notch-3 mutation and symptoms produce by damage to the
development of arteriopathy is still nervous system. Disability is the personal
undetermined. limitation imposed on the activities of daily
living by neurological impairment, and
Reference: handicap is the social or environmental
effects of the disability or impairment.
Kalaria RN. Advances in molecular genetics
and pathology of cerebrovascular disorders.
Trends Neurosci 2001;24:392-400. Reference:
_____________________________ Burks JS, Johnson KP. Multiple sclerosis:
Question(s) 257: Clinical Adult Medical management and rehabilitation.
New York: Demos, 2000;223.
Discussion:
_____________________________
Selective impairment of vertical eye
movement is most characteristic of a lesion
in the midbrain.

Reference:
Joynt RJ. Clinical neurology. Philadelphia:
JB Lippincott Co., 1989.

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Question(s) 261: Anatomy Reference:

Parent A. Carpenter's human
Discussion:
neuroanatomy. 9th ed. Baltimore: Williams &
The nucleus tractus solitarius is the site of
Wilkins, 1996.
termination of afferents from the
baroreceptors. Involvement of this structure _____________________________
results in paroxysmal hypertension Question(s) 265: Physiology
resembling a pheochromocytoma. Discussion:
The sodium-potassium-ATP pump maintains
Reference: the resting membrane potential despite
Biaggioni I, Whetsell WO, Jobe J, et al. passive diffusion of sodium into and
Baroreflex failure in a patient with central potassium out of the cell. Inhibition of this
nervous system lesions involving the pump would result in an increase in
nucleus tractus solitarii. Hypertension 1994; intracellular sodium concentration, a
23:491-95. decrease in intracellular potassium and a
resulting depolarization of the cell
_____________________________
membrane.
Question(s) 262: Pathology
Discussion: Reference:
The most helpful immunostain for confirming
Kandel ER, Schwartz JH, Jessel TM.
a diagnosis of meningioma is epithelial
Principles of neural science. 4th ed. New
membrane antigen (EMA). S-100 protein is
York: McGraw-Hill, 2000.
positive in gliomas and schwannomas.
Transthyretin is positive in choroid plexus _____________________________
papillomas. Question(s) 266: Clinical Adult
Discussion:
Reference: Locked-in syndrome is a state in which
1. Kleihues P, Cavenee WK, editors. quadriplegia prevents the usual means of
Pathology and genetics of tumors of the gestural or verbal communication. Usually
nervous system. 2nd ed. New York: Oxford the patient can communicate by way of
University Press, 2000. vertical eye movements or blinking and can
demonstrate full comprehension of his
2. Fuller GN, Goodman JC. Practical review
plight. A sleep wake cycle is often
of neuropathology. Philadelphia: Lippincott
preserved. The most common vascular
Williams & Wilkins, 2001.
lesion underlying the locked-in syndrome is
_____________________________ basilar artery occlusion with extensive
Question(s) 263: Physiology destruction of the pontine base.
Discussion:
Pain is mediated by free nerve endings. Reference:
Barnett HJM, Mohr JP, Stein BM, et al,
Reference: editors. Stroke, pathology, diagnosis and
Kandel ER, Schwartz JH, Jessel TM. management. New York: Churchill
Principles of neural science. 4th ed. New Livingston, 1993.
York: McGraw-Hill, 2000. _____________________________
_____________________________
Question(s) 264: Anatomy
Discussion:
The basal nucleus of Meynert is in the
substantia innominata and contains clusters
of large cholinergic neurons that have
widespread projections to the cortex and
amygdala.

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Question(s) 267: Reference:

Pharmacology/Chemistry Indo Y, Tsuruta M, Hayashida Y, et al.
Mutation in the TRKA/NGF receptor gene in
Discussion:
patients with congenital insensitivity to pain
Mutations in the alpha-synuclein gene have
and anhidrosis. Nat Genet 1996;13:485-488.
been found in some families with autosomal
dominant Parkinson's disease. The _____________________________
abnormal alpha-synuclein is present in the Question(s) 270: Behavioral
ubiquitin-positive Lewy bodies. Synuclein is Discussion:
a normal protein in the nucleus and synaptic Anosognosia (unawareness of deficit or
terminals of neurons, but its role is still illness) is usually seen associated with non-
incompletely defined. dominant parietal lobe lesions.
Achromatopsia is found after lesions of the
Reference: inferior lip of the occipital lobe. Limb kinetic
Polymeropolulos MH, et al. Mutation in the apraxia is seen after lesions of the anterior
alpha-synuclein gene identified in families corpus callosum. Expressive aprosodia is
with autosomal dominant Parkinson's seen after right frontal lesions. Semantic
disease. Science 1997;276:2045-2047. aphasia is seen after dominant hemisphere
lesions.
_____________________________
Question(s) 268:
Pharmacology/Chemistry Reference:
Feinberg TE, Farah MJ. Behavioral
Discussion:
neurology and neuropsychology. New York:
Periodic movements of sleep (nocturnal
McGraw-Hill, 1997.
myoclonus) consists of the occurrence
during sleep of periodic episodes of highly _____________________________
stereotypic leg muscle jerks. If severe Question(s) 271:
enough to warrant therapy, pergolide, Pharmacology/Chemistry
pramipexole, or controlled release Discussion:
levodopa/carbidopa at bedtime is often Midodrine raises blood pressure by
effective. stimulating alpha-adrenergic receptors. It is
a pro-drug with a short half-life and does not
Reference: cause fluid retention like the
Commella CL, Tanner CM. Four unusual mineralocorticoids.
movement disorders: painful legs and
moving toes syndrome, periodic movements Reference:
of sleep, restless legs syndrome and Wright RA, Kaufmann HC, Perera R, et al. A
akathisia. In: Klawans HL, Goetz CG, double-blind, dose-response study of
Tanner CM, editors. Textbook of clinical midodrine in neurogenic orthostatic
neuropharmacology and therapeutics. 2nd hypotension. Neurology 1998;51(1):120-4.
ed. New York: Raven Press, 1992;220-222. _____________________________
_____________________________
Question(s) 272: Physiology
Question(s) 269:
Pharmacology/Chemistry Discussion:
Multifocal motor neuropathy with conduction
Discussion: block presents with slowly progressive,
Nerve growth factor, acting via trk A asymmetric weakness, atrophy and reflex
receptors, is critical for survival of loss with normal or minimally abnormal
nociceptive and sympathetic neurons. sensation. Nerve conduction studies
Mutations of the Trk A gene are associated involving clinically weak muscles
with the phenotype of congenital insensitivity demonstrate evidence of partial conduction
to pain and anhidrosis, as seen in hereditary block, but sensory nerve conduction studies
sensory and autonomic neuropathies types are typically normal.
IV and V.

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 2002 AAN RITE Discussion & Reference Manual

Question(s) 276:
Reference: Pharmacology/Chemistry
Lewis RA, et al. Multifocal demyelinating Discussion:
neuropathy with persistent conduction block. Topiramate is a weak inhibitor of carbonic
Neurology 1982;32:958-963. anhydrase, and therefore may increase
_____________________________ urinary pH and decrease urinary citrate
excretion. Both actions may predispose to
Question(s) 273: Pathology
kidney stones.
Discussion:
Nerve specimens from patients with Krabbe
disease and metachromatic leukodystrophy Reference:
contain characteristic deposits of Marson AG, Kadir AZ, Chadwick DW. The
sphingolipids. Lead intoxication produces a new antiepileptic drugs: a systemic review of
demyelinating neuropathy. Alcoholic their efficacy and tolerability. Epilepsia
neuropathy is an axonal neuropathy with 1997;38:859-880.
secondary demyelination. Canavan's _____________________________
disease shows characteristic spongiform Question(s) 277: Behavioral
leukodystrophy only in the brain.
Discussion:
Good spontaneous and repetitive affective
Reference: prosody as well as spontaneous gesturing
Graham DI, Lantos PL. Greenfield's with poor affective comprehension of
neuropathology. 6th ed. London: Arnold, language and gesture is known as a
1997. transcortical sensory aprosodia.
_____________________________
Question(s) 274: Behavioral Reference:
Discussion: Ross E. Affective prosody and the
Of clozapine, quetiapine, phenelzine, aprosodies. In: Mesulam M. Principles of
olanzapine, and risperidone the one most behavioral and cognitive neurology. 2nd ed.
likely to produce extrapyramidal side effects New York: Oxford University Press,
is risperidone. 2000;316-331.
_____________________________
Reference: Question(s) 278: Behavioral
Puzantian T, Stimmel G. Review of Discussion:
psychotropic drugs. New York: McMahon, The memory disorder of early Alzheimer's
2001. disease involves impairment in word recall
_____________________________ (recent memory) with normal digit span
(immediate memory), and relatively spared
Question(s) 275: Clinical Adult
remote memory.
Discussion:
The diaphragm is innervated by C3-C5, not
C6. The brachioradialis muscle is innervated Reference:
primarily by C5-C6 roots. The subscapular Cummings JL, Benson DF. Dementia: a
nerve innervates the teres major muscle and clinical approach. Boston: Butterworth-
the radial nerve innervates the supinator Heinemann, 1992.
muscle. The first dorsal interosseus is _____________________________
innervated by the ulnar nerve.

Reference:
Brazis PW, Masdeu JC, Biller J. Localization
in clinical neurology. 3rd ed. Boston: Little,
Brown, 1996.
_____________________________

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Question(s) 279: Behavioral Question(s) 282: Physiology

Discussion: Discussion:
Obsessive-compulsive disorder is often REM or active sleep accounts for 50% of
associated with Tourette's syndrome. sleep in neonates.

Reference: Reference:
Coffey BJ, Park KS. Behavioral and Kryger MH, Roth T, Dement WC. Principles
emotional aspects of Tourette's syndrome. and practice of sleep medicine. 2nd ed.
In: Jankovic J, editor. Neurologic clinics - Philadelphia: WB Saunders Co., 1994.
Tourette's syndrome. Philadelphia: WB _____________________________
Saunders, 1997. Question(s) 283: Anatomy
_____________________________
Discussion:
Question(s) 280: Behavioral The dorsomedial (or mediodorsal) nucleus
Discussion: of the thalamus provides innervation to the
The supplementary motor area (SMA) is whole prefrontal cortex, which consists of a
thought to act as a pacemaker for verbal paralimbic orbitofrontal region involved in
output. Damage to the SMA or to its control of affect and behavior, and an
connections with Broca's area produces the associative dorsolateral region involved in
clinical picture of transcortical motor executive functions.
aphasia.
Reference:
Reference: Parent A. Carpenter's human
Freedman M, Alexander MP, Naeser MA. neuroanatomy. 9th ed. Baltimore: Williams &
The anatomical basis of transcortical motor Wilkins, 1996.
aphasias. Neurology 1984;34:409-417. _____________________________
_____________________________ Question(s) 284: Clinical Adult
Question(s) 281: Discussion:
Pharmacology/Chemistry Myeloma, especially of the osteosclerotic
Discussion: type, has a frequent association with a
Lamotrigine is efficacious for treatment of demyelinating polyneuropathy.
partial and generalized seizures, including
the Lennox-Gastaut syndrome. It is
metabolized in the liver. Valproate inhibits Reference:
metabolism and increases considerably the 1. Kelly JJ, et al. Osteosclerotic myeloma
half life of lamotrigine. Lamotrigine is started and peripheral neuropathy. Neurology
at a dose of 50 mg/d for two weeks in 1983;33:202-210.
patients not receiving valproate, and 25 mg 2. Kelly JJ, et al. The spectrum of peripheral
every other day for two weeks in patients neuropathy in myeloma. Neurology
receiving the drug. The incidence of serious 1981;31:24-31.
rash is 0.3% in adults and 1% in children. _____________________________
Co-administration with valproate and rapid Question(s) 285: Physiology
escalation of the dose increase the risk of
Discussion:
this complication.
Cerebral blood flow is increased most
effectively by administration of 5% carbon
Reference: dioxide.
Holland KD. Efficacy, pharmacology, and
adverse effects of antiepileptic drugs. Neurol Reference:
Clin 2001;19:313-345.
Kandel ER, Schwartz JH, Jessel TM.
_____________________________ Principles of neural science. 4th ed. New
York: McGraw-Hill, 2000.
_____________________________

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Question(s) 286: Pathology Question(s) 289:

Discussion: Pharmacology/Chemistry
Shadow plaques in multiple sclerosis were Discussion:
formerly thought to represent areas of Niemann-Pick disease, type C (NPC) is an
partial, arrested, or incomplete autosomal recessive lipidosis, in which
demyelination. They are now recognized to impaired intracellular trafficking of
be areas of partial remyelination. There is cholesterol and glycolipids lead to
little agreement on any characteristic intracellular sequestration of unesterfied
precursor or nascent lesion in multiple cholesterol, and increased concentrations of
sclerosis; ultrastructural studies fail to detect glycolipids in the brain. Secondary
changes in myelin sheaths or responses to exogenous LDL-cholesterol
oligodendrocytes that clearly precede the loading, including down regulation of LDL
sudden appearance of macrophages in the receptors and HMG CoA reductase and
plaque lesions. upregulation of ACAT are impaired. There is
no primary deficiency in these enzymes. At
Reference: Least 95% of cases are associated with
mutations in NPC1; the remainder are
Graham DI, Lantos PL. Greenfield's
associated with mutations in NPC2 (HE1).
neuropathology. 6th ed. London: Arnold,
1997.
_____________________________ Reference:
Question(s) 287: Anatomy 1. Patterson MC, Vanier MT, Suzuki K, et al.
Niemann-Pick disease, type C: a lipid
Discussion:
trafficking disorder. In: Scriver CR, Beaudet
The pulvinar receives fibers from the
AL, Sly WS, et al, editors. The metabolic
superior colliculus and projects to areas 17,
and molecular bases of inherited disease.
18 and 19. Both the inferior and lateral
4th ed. New York: McGraw-Hill, 2001:3611-
pulvinar have reciprocal connections with
3633.
the occipital cortex.
2. Naureckiene S, Sleat DE, Lackland H, et
al. Identification of HE1 as the second gene
Reference: of Niemann-Pick C disease. Science
Parent A. Carpenter's human 2000;290:2298-2301.
neuroanatomy. 9th ed. Baltimore: Williams & _____________________________
Wilkins, 1996. Question(s) 290: Pathology
_____________________________
Discussion:
Question(s) 288: It is important to remember that although
Pharmacology/Chemistry retinal hemorrhages are well described in
Discussion: non-accidental trauma (most notably in
Tardive dyskinesia results from chronic "shaken baby syndrome") there are many
treatment with dopaminergic D2 receptor other causes of retinal hemorrhages,
blockers. These include the classical high- including following accidental trauma, in
potency neuroleptics, but also antiemetic coagulopathies, and in newborns after
agents such as prochlorperazine vaginal delivery. Retinal hemorrhage after
(Compazine) or metoclopramide (Reglan). resuscitation is rare, but has been
described.
Reference:
Tucker GJ. Psychiatry for the neurologist, Reference:
part A. Continuum 1997 Nov;3(6). DiMaio V, DiMaio D, editors. Forensic
_____________________________ Pathology. 2nd ed. Boca Raton: CRC Press,
2001.
_____________________________

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 2002 AAN RITE Discussion & Reference Manual

Question(s) 291: Behavioral Question(s) 294: Behavioral

Discussion: Discussion:
Decreased hemoglobin and elevated mean Chronic alcoholic hallucinosis is
corpuscular volume are compatible with a characterized by auditory hallucinations with
macrocytic anemia that may be secondary a clear sensorium.
to vitamin B12 deficiency. Vitamin B12
deficiency, in turn may cause combined Reference:
system degeneration (affecting posterior
Hales RE, Yudofsky SC. Textbook of
columns and lateral corticospinal tracts).
neuropsychiatry. 3rd ed. Washington, DC:
Dementia and peripheral neuropathy may
American Psychiatric Press, 1997.
also be seen.
_____________________________
Question(s) 295: Clinical Adult
Reference:
Discussion:
Samuels MA, Feske S. Office practice of
A history of abrupt loss of consciousness
neurology. New York: Churchill Livingston,
and examination findings of pinpoint pupils,
1996.
ocular bobbing, and absent oculovestibular
_____________________________ responses would suggest an acute, severe
Question(s) 292: Clinical Adult pontine lesion, such as pontine hematoma.
Discussion:
The serotonin syndrome displays Reference:
myoclonus, fever, confusion, ataxia,
Plum F, Posner JB. The diagnosis of stupor
movement problems, sweating, and
and coma. Philadelphia: FA Davis Co.,
shivering. Prominent myoclonus helps
1982.
differentiate it from the neuroleptic malignant
_____________________________
syndrome.
Question(s) 296: Behavioral
Reference: Discussion:
The pathophysiology of schizophrenia as
1. Kampoliti K. Drug-induced and iatrogenic
associated with pathological changes in the
neurological disorders. In: Goetz CG,
dorsomedial thalamus and the dorsolateral
Pappert EJ, editors. Textbook of clinical
prefrontal cortex thereby affecting the
neurology. Philadelphia: WB Saunders,
pathway between the two sites. Any role of
1999.
pedunculopontine, nigrostriatal,
2. Frucht S, Fahn S. Movement disorder hippocampal-fornical-mamillary, and
emergencies. Proceedings of the Annual amygdala-orbitofrontal pathways is not
Meeting of the American Academy of clearly established in schizophrenia if in fact
Neurology; 2001: Philadelphia (PA): 6 there is a role in these pathways.
BS.001-12.
_____________________________
Question(s) 293: Behavioral Reference:
Lewis DA. Is there a neuropathology of
Discussion: schizophrenia? Recent findings converge on
Patients with obsessive-compulsive disorder atered thalamic-prefrontal cortical
have been shown to respond to treatment connectivity. The Neuroscientist 2000
with certain selective serotonin reuptake June;6(3):208-218.
inhibitors (SSRIs), including fluoxetine and
_____________________________
fluvoxamine.

Reference:
Pigott TA. OCD: where the serotonin
selectivity story begins. Clinical Psychiatry
1996;57:11-20.
_____________________________

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Question(s) 297: Behavioral Reference:

Rowland LP, editor. Merritt's textbook of
Discussion:
neurology. Philadelphia: Lea & Febiger,
Bupropion has essentially no anticholinergic,
1989.
sedating, sexual dysfunction, or orthostatic
hypotension side effects. Fluoxetine, _____________________________
citalopram, and venlafaxine have a high to Question(s) 301:
very high risk of sexual dysfunction. Pharmacology/Chemistry
Mirtazapine is sedating. None of the above Discussion:
medications have anticholinergic or Fetal vitamin K deficiency with hemorrhagic
significant orthostatic hypotensive side complications occurs in 10% of neonates
effects. born from mothers receiving antiepileptic
drugs that induce liver metabolism of vitamin
Reference: K, including phenobarbital and phenytoin.
Puzantian T, Stimmel G. Review of Women taking enzyme-inducing antiepileptic
psychotropic drugs. New York: McMahon, drugs should be treated with vitamin K1, 10-
2001. 20 mg daily during the last month of
pregnancy. Infants should receive 1 mg
_____________________________
intramuscularly at birth and, if needed, fresh
Question(s) 298: Anatomy frozen plasma.
Discussion:
The chemoreceptor trigger zone is located in
Reference:
the area postrema.
Foldvary N. Treatment issues for women
with epilepsy. Neurol Clin 2001;19:409-425.
Reference: _____________________________
Parent A. Carpenter's human Question(s) 302: Pathology
neuroanatomy. 9th ed. Baltimore: Williams &
Wilkins, 1996. Discussion:
Mycotic aneurysms are most commonly
_____________________________
found in the distal branches of the middle
Question(s) 299: Behavioral cerebral artery.
Discussion:
Callosal apraxia results from a lesion in the Reference:
genu of the corpus callosum. This results in
Graham DI, Lantos PL. Greenfield's
a left limb kinetic apraxia. Tactile and
neuropathology. 6th ed. London: Arnold,
auditory input cross the corpus callosum
1997.
posteriorly and are therefore unaffected by a
genu lesion. Alexia without agraphia results _____________________________
from a left occipital splenium of the corpus Question(s) 303: Clinical Adult
callosum lesion. Discussion:
Numerous studies of the cognitive
Reference: disturbance in multiple sclerosis
Heilman K, Valenstein E. Clinical demonstrate the absence of classic cortical
neuropsychology. New York: Oxford (e.g., language, cognition, visuospatial,
University Press, 1985. memory) impairments but clearly show a
slowing of information processing.
_____________________________
Question(s) 300: Behavioral
Reference:
Discussion:
Beatty WW, Goodkin DE, Monson N, et al.
This history depicts a case of herpes
Cognitive disturbances in patients with
simplex virus encephalitis. The treatment of
relapsing remitting multiple sclerosis. Arch
choice is acyclovir.
Neurol 1989;46:1113-1119.
_____________________________

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Question(s) 304: Reference:

Pharmacology/Chemistry Cummings JL, Benson DF. Dementia: a
clinical approach. Boston: Butterworth-
Discussion:
Heinemann, 1992.
Streptococcus pneumoniae is a common
cause of bacterial meningitis. As several _____________________________
causes of ceftriaxone resistant Question(s) 308: Clinical Adult
streptococcus pneumoniae have been Discussion:
reported, the initial treatment in suspect The characteristic feature of conduction
cases should include vancomycin, until the aphasia is a striking deficit of repetition, with
results of drug sensitivities are available. relative sparing of speech comprehension
and output, though literal paraphasic errors
Reference: may be present.
Quagliarello VJ, Scheld WM. Treatment of
bacterial meningitis. NEJM 1997;336:607. Reference:
_____________________________ Bradley WG, Daroff RB, Fenichel GM, et al.
Question(s) 305: Anatomy Neurology in clinical practice. 2nd ed.
Boston: Butterworth-Heinemann, 1996.
Discussion:
The sweat glands are innervated by _____________________________
postganglionic sympathetic cholinergic Question(s) 309: Anatomy
fibers. Cholinergic inputs stimulate sweat Discussion:
production via M3 type muscarinic Alexia without agraphia follows combined
receptors. damage to the dominant medial occipital
region and the inferior fibers of the splenium
Reference: of the corpus callosum. This is in the
Low PA, editor. Clinical autonomic distribution of the posterior cerebral artery.
disorders. 2nd ed. New York: Lippincott-
Raven, 1997. Reference:
_____________________________ Brazis PW, Masdeu JC, Biller J. Localization
Question(s) 306: Pathology in clinical neurology. 3rd ed. Boston: Little,
Brown, 1996.
Discussion:
Spontaneous or traumatic dissections occur _____________________________
most frequently in the carotid artery at the Question(s) 310: Clinical Pediatrics
neck. Vertebral artery dissections may Discussion:
occur after chiropractic manipulations of the MELAS - mitochondrial encephalomyopathy,
neck. lactic acidosis and stroke-like episodes is
one of several mitochondrial
Reference: encephalopathies. In cases where a clear
Graham DI, Lantos PL. Greenfield's maternal inheritance has been established,
neuropathology. 6th ed. London: Arnold, there is a mutation in the mitochondrial
1997. tRNA leu. Cranial CT and MR scans in
patients with MELAs reveal infarct-like areas
_____________________________
bilaterally in the posterior cerebrum and
Question(s) 307: Behavioral almost always hypodensities or calcifications
Discussion: in the basal ganglia region.
The loss of remote memory including
autobiographical memory in the face of Reference:
intact new learning ability is consistent with
Di Mauro S, et al. The mitochondrial
psychogenic amnesia.
disorders. In: Berg BO, editor. Principles of
child neurology. New York: McGraw-Hill,
1996.
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Question(s) 311: Behavioral Question(s) 315: Clinical Pediatrics

Discussion: Discussion:
Witzelsucht (inappropriate jocularity) is seen In both Duchenne's and Becker's muscular
in patients with orbitofrontal cortex lesions. dystrophy the dystrophin gene is affected
Lesions in the orbitofrontal cortex also but the molecular mechanisms differ in the
include disinhibited and antisocial behavior. two disorders. Absence of dystrophin in the
muscle confirms the diagnosis of
Duchenne's muscular dystrophy. Whereas
Reference: altered or reduced dystrophin in the muscle
Feinburg TE, Farah MJ. Behavioral is consistent with Becker's muscular
neurology and neuropsychology. New York: dystrophy.
McGraw Hill, 1997.
_____________________________
Reference:
Question(s) 312: Anatomy
Dubowitz V. Muscle disorders in childhood.
Discussion: Philadelphia: WB Saunders, Co., 1995.
Sensation to the umbilicus is carried by T10. _____________________________
Question(s) 316: Clinical Pediatrics
Reference: Discussion:
Parent A. Carpenter's human The cerebrospinal fluid in preterm newborn
neuroanatomy. 9th ed. Baltimore: Williams & infants without bacterial meningitis and
Wilkins, 1996. without other disease can have a protein of
_____________________________ 65-150 mg/dl and the white blood cell count
Question(s) 313: Physiology can have a range of 0-29 cells/cu mm with a
Discussion: mean of nine.
Ictal EEG is the most important factor in
determining the site of seizure onset on Reference:
chronic, intracranial EEG monitoring. Volpe JJ. Neurology of the newborn. 4th ed.
Philadelphia: WB Saunders, 2001.
Reference: _____________________________
Cascino GD. Electroencephalographic Question(s) 317: Pathology
recording for epilepsy surgery. In: Daube Discussion:
JR, editor. Clinical neurophysiology. The brain is very sensitive to the effects of
Philadelphia: FA Davis Company, 1996;132- carbon monoxide intoxication. A cherry-red
140. color may be seen in many tissues
_____________________________ (including the brain) due to the presence of
Question(s) 314: Anatomy carboxyhemoglobin. With survival of 24-48
Discussion: hours, petechial hemorrhages may be seen
The optic nerve provides inputs to the in the white matter along with more
superior colliculus (for reflex saccades), significant hemorrhage in the globus
lateral geniculate nucleus (relay of the visual pallidus. Cavitation is seen in longer-term
pathway), pretectal nucleus (relay of the survivors.
light reflex) and the suprachiasmatic nucleus
(the circadian pacemaker). The medial Reference:
preoptic nuclei would be spared by Ellison D, Love S, Chimelli L, et al, editors.
enucleation of the eye. Neuropathology. London: Mosby, 1998.
_____________________________
Reference:
Parent A. Carpenter's human
neuroanatomy. 9th ed. Baltimore: Williams &
Wilkins, 1996.
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Question(s) 318: Physiology

Discussion: Reference:
Acetylcholine is the neurotransmitter of Volpe JJ. Neurology of the newborn. 4th ed.
preganglionic sympathetic and vagal nerve Philadelphia: WB Saunders, 2001.
fibers. _____________________________
Question(s) 322: Pathology
Reference: Discussion:
Benarroch EE, Westmoreland BF, Daube Approximately 90-95% of epidural
JR, et al. Medical neurosciences. 3rd ed. hematomas are associated with a skull
Boston: Little, Brown and Co., 1994. fracture. They are usually unilateral, disc-
_____________________________ shaped, and are most often due to fractures
Question(s) 319: Clinical Pediatrics of the squamous-temporal bone with
laceration of the middle meningeal artery.
Discussion:
The incidence of lamotigine-associated
severe rash is estimated to be one in 1,000 Reference:
adults and one in 200 children. The risk in DiMaio V, DiMaio D, editors. Forensic
children is increased with a rapid increase in Pathology. 2nd ed. Boca Raton: CRC Press,
dose and when given with valproate. 2001.
_____________________________
Reference: Question(s) 323 - 325: Clinical Pediatrics
Pellock JM, Dodson WE, Bourgeois BFD, Discussion:
editors. Pediatric epilepsy: diagnosis and The majority of normal children will pick up a
therapy. 2nd ed. New York: Demos, 2001. small object with a thumb and forefinger
_____________________________ (pincer) grasp by one year of age, can walk
Question(s) 320: Clinical Pediatrics unsupported by 15 months and when asked,
will point to several body parts correctly by
Discussion: two years of age.
The movements of Sydenham chorea
cannot be suppressed by the patient,
whereas tics can be suppressed voluntarily Reference:
for at least a short period of time. Tics are Berg BO, editor. Principles of child
stereotyped with a limited variety of neurology. New York: McGraw-Hill, 1996.
movements at any one time. In contrast _____________________________
chorea is very variable.

Reference:
Menkes JH, Sarnat HB, editors. Child
neurology. 6th ed. Philadelphia: Lippincott
Williams & Wilkins, 2000.
_____________________________
Question(s) 321: Clinical Pediatrics
Discussion:
Most cases of newborn infants with
myelomeningocele have a Chiari type II
malformation. If hydrocephalus develops,
overt clinical signs most commonly appear
two to three weeks after birth.

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Question(s) 326 - 330: Anatomy Question(s) 335 - 337: Behavioral

Discussion: Discussion:
The inferior cerebellar peduncle contains The amygdala is associated with emotional
primary and secondary vestibulocerebellar memory; the hippocampus with declarative
fibers and certain cerebellovestibular memory and the pulvinar with visual
(juxtarestiform) fibers from the nodulus, attention.
uvula, and fastigial nuclei. The middle
cerebellar peduncle carries all
pontocerebellar fibers. The superior Reference:
cerebellar peduncle carries the Feinberg TE, Farah MJ. Behavioral
dentatorubral, dentatothalamic and anterior neurology and neuropsychology. New York:
(ventral) and spinothalamic tracts. McGraw-Hill, 1997.
_____________________________
Question(s) 338 - 340: Physiology
Reference:
Benarroch EE, Westmoreland BF, Daube Discussion:
JR, et al. Medical neurosciences - an AMPA, kainate and NMDA receptors are
approach to anatomy, pathology and ionotropic glutamate receptors. Domoic acid
physiology by systems and levels. 4th ed. is an agonist at the kainate receptor. NMDA
New York: Lippincott, Williams & Wilkins, receptor responses are blocked by
1999. magnesium ions. Metabotropic glutamate
_____________________________ receptors are coupled to second
messengers such as phospholipase A,
Question(s) 331 - 334: Clinical Pediatrics adenylate cyclase and G-proteins.
Discussion:
The skin lesions of tuberous sclerosis
include hypopigmented macules, which may Reference:
require a Wood's lamp to visualize, Clark S, Wilson W. Mechanisms of
adenoma sebaceum, shagreen patch, epileptogenesis and the expression of
subungal and periungual fibromas. Rocky epileptiform activity. In: Wyllie E, editor. The
mountain spotted fever classically has a treatment of epilepsy: principles and
generalized petechial rash and Lyme practice. 2nd ed. Baltimore: Williams and
disease frequently has erythema migrans as Wilkins, 1997;53-81.
the typical rash. Rheumatic disease has _____________________________
erythema marginatum, subcutaneous Question(s) 341 - 342: Clinical Adult
nodules, carditis, polyarthritis, and Discussion:
Sydenham chorea as major manifestations. 14-3-3 protein has been reported in the CSF
of 90% of patients with classical Creutzfeldt-
Reference: Jakob disease. pcr of CSF for Epstein Barr
Behrman RE, Kliegman RM, Jenson HB. Virus DNA is frequently positive in primary
Nelson textbook of pediatrics. Philadelphia: central nervous system lymphoma,
WB Saunders, Co., 2000. particularly in patients with AIDS.
_____________________________
Reference:
1. Brown P. Transmissible spongiform
encephalopathy. In: Goetz CG, Pappert EJ,
editors. Textbook of clinical neurology.
Philadelphia: WB Saunders, 1999.
2. Belman AL, Preston T, Milazzo M. Human
immunodeficiency virus and acquired
immunodeficiency syndrome. In: Goetz CG,
Pappert EJ, editors. Textbook of clinical
neurology. Philadelphia: WB Saunders,
1999.
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Question(s) 343 - 345: Clinical Pediatrics Reference:

Discussion: Benarroch EE, Westmoreland BF, Daube
Symptoms in opsoclonus-myoclonus JR, et al. Medical neurosciences - an
syndrome typically precede detection of approach to anatomy, pathology and
occult neuroblastoma. Medulloblastoma, physiology by systems and levels. 4th ed.
some ependymomas and New York: Lippincott, Williams & Wilkins,
ependymoblastomas have the capability to 1999.
seed along the CSF pathways. Children with _____________________________
neurofibromatosis have an increased Question(s) 353 - 356: Anatomy
incidence of optic pathway gliomas.
Discussion:
Hemisection of the spinal cord (Brown-
Reference: Sequard syndrome) results in ipsilateral
Menkes JH, Sarnat HB, editors. Child paralysis and loss of position sense, and
neurology. 6th ed. Philadelphia: Lippincott contralateral loss of pain sensation below
Williams & Wilkins, 2000. the lesion. The bladder is only temporarily
_____________________________ affected.
Question(s) 346 - 348: Clinical Pediatrics
Discussion: Reference:
The Walker-Warburg syndrome is a type II Parent A. Carpenter's human
lissencephaly with a congenital muscular neuroanatomy. 9th ed. Baltimore: Williams &
dystrophy and has autosomal recessive Wilkins, 1996.
inheritance. Rett syndrome is characterized _____________________________
by progressive microcephaly and Question(s) 357 - 360: Anatomy
developmental regression in the first two
Discussion:
years of life. The disorder occurs almost
The gluteus medius is innervated by the
exclusively in females. Individuals with
superior gluteal nerve. The gluteus maximus
Angelman syndrome, also known as the
is innervated by the inferior gluteal nerve.
"happy puppet" syndrome, have mental
The short head of the biceps femoris is
retardation, jerky movements, and seizures.
innervated by the peroneal division of the
The diagnosis can be confirmed by
sciatic nerve. The tibialis posterior is
demonstrating a deletion on chromosome
innervated by the tibial nerve.
15.

Reference:
Reference:
Patten J. Neurological differential diagnosis.
Menkes JH, Sarnat HB, editors. Child
2nd ed. New York: Springer, 1996.
neurology. 6th ed. Philadelphia: Lippincott
_____________________________
Williams & Wilkins, 2000.
_____________________________
Question(s) 349 - 352: Anatomy
Discussion:
The motor nucleus of the trigeminal nerve
sends fibers to muscles of mastication. The
spinal trigeminal nucleus subserves pain
and temperature functions. The
mesencephalic nucleus contains primary
sensory neurons involved with pressure and
kinesthetic sense of teeth, periodontium,
hard palate, and joint capsules. The
principal (main) sensory nucleus is
concerned with tactile and pressure sense of
the face.

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Question(s) 361 - 364: Clinical Pediatrics Question(s) 374: Clinical Adult

Discussion: Discussion:
Leber's optic atrophy is transmitted via In an HIV positive patient, an enhancing
maternal mitochondria. Becker's muscular brain mass, which does not respond to anti-
dystrophy is inherited in a sex linked toxoplasmosis therapy, is most likely a B-cell
recessive fashion. Spinal muscular atrophy lymphoma. Toxoplasmosis is the most
is an autosomal recessive disorder. common cause of focal intracranial mass
Tuberous sclerosis can be inherited in an lesions in patients with AIDS. Lymphoma is
autosomal dominant mode but the second most common cause.
approximately one-half of the cases are due Lesscommon causes include pyogenic
to new mutations. abscess, syphilitic gumma, Candida or
Nocardia abscess, cryptococcoses,
cryptococcal pseudocyst, other fungal and
Reference: parasitic infections, and infarction. About
Menkes JH, Sarnat HB, editors. Child 80% of patients with established CNS
neurology. 6th ed. Philadelphia: Lippincott toxoplasmosis have positive toxoplasma
Williams & Wilkins, 2000. titers in serum,and about 85% of patients
_____________________________ with CNS toxoplasmosis have multiple
Question(s) 365 - 369: Physiology lesions on brain MRI. Thus, patients with
Discussion: negative titers and only a single lesion on
Drug-induced muscle disorders include MRI should have a stereotactic biopsy, but
myalgia, myotonia, type 2 atrophy (e.g., patients with either positive titers or multiple
prednisone), and focal (e.g., oxycodone), MRI lesions should be treated empirically
necrotizing, inflammatory (e.g., L- withantitoxoplasmosis therapy for 2 to 3
tryptophan), mitochondrial (e.g., zidovudine) weeks and monitored clinically and
or autophagic (e.g., amiodarone) radiologically.
myopathies.
Reference:
Reference: 1. Dina TS. Primary central nervous system
Mastaglia FL, Laing NG. Investigation of lymphoma versus toxoplasmosis in AIDS.
muscle disorders. J Neurol Neurosur Psych Radiology 1991;179:823-828.
1996;60:256-274. 2. Aminoff MJ. Neurology and general
_____________________________ medicine. New York: Churchill Livingstone,
1995.
Question(s) 370 - 373: Physiology _____________________________
Discussion:
Serotonin is associated with the dorsal
raphe nuclei; norepinephrine is associated
with the locus ceruleus; acetylcholine with
the basal forebrain and parasympathetic
neurons of cranial nerves, and histamine
with the hypothalamus.

Reference:
Kandel ER, Schwartz JH, Jessel TM.
Principles of neural science. 4th ed. New
York: McGraw-Hill, 2000.
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Question(s) 375: Clinical Adult Question(s) 377:

Pharmacology/Chemistry
Discussion:
Confusion, multiple cerebral lesions, multiple Discussion:
sensory and perceptual deficits indicate a The most common form of disulfiram
poorer prognosis than hemiparesis alone. neurotoxicity is peripheral polyneuropathy
Preventive measures for decubitus ulcers, which may on occasion affect proximal
pulmonary embolism, contractures, muscles first, leading to potential
dysphagia and aspiration are regularly misdiagnosis as myopathy. The neuropathy
employed in most stroke facilities. Although typically appears 5-6 months after institution
it is important to treat depression when it is of disulfiram treatment. Disulfiram-induced
present, prophylactic medication is not CNS toxicity is less common. A progressive
indicated. neuropathy first appearing 6 months after
stopping alcohol consumption is unlikely to
be alcohol-induced. Although thiamine
Reference: deficiency can result in neuropathy, it should
Dobkin BH, Thompson AJ. Principles of not occur on thiamine replacement unless
neurological rehabilitation. In: Bradley WG, there is some block of intestinal absorption.
Daroff RB, Fenichel GM, et al, editors. Vitamin E deficiency can produce posterior
Neurology in clinical practice. 3rd ed. column, spinocerebellar, and peripheral
Boston: Butterworth-Heinemann, 2000;959- nerve dysfunction, but this also would not
1005. occur in the face of replacement therapy
unless a malabsorption process is present.
_____________________________ Abetalipoproteinemia (Bassen-Kornzweig
Question(s) 376: Clinical Adult disease) is a process where malabsorption
results in severe Vitamin E deficiency.
Discussion: Thiamine is an important coenzyme for a
The diagnosis of multiple sclerosis (MS) is number of cellular reactions, including those
considered to be clinically definite when responsible for ATP generation. The
there have been two attacks and there is combination of disulfiram and alcohol
clinical evidence of two separate lesions. produces the "Antabuse syndrome" or
This evidence must be an abnormality on "acetaldehyde syndrome", characterized by
physical examination or on paraclinical the unpleasant combination of vasodilatation
testing such as evoked potential or imaging of the face and body, headache,
techniques. Pattern shift visual evoked tachycardia, respiratory difficulty, vomiting,
responses reveal abnormalities in over 90% weakness, and hypotension. It is probably
of patients with a history of optic neuritis, produced by buildup of acetaldehyde due to
even when visual acuity has returned to inhibition of ethanol metabolism.
normal. In a well-designed clinical trial,
subcutaneous beta interferon was
demonstrated to reduce the relapse rate of Reference:
certain relapsing-remitting patients. No 1. O'Donoghue JL. Neurotoxicity of industrial
beneficial effect on ultimate disability was and commercial chemicals. Boca Raton:
demonstrated over the two to three year CRC Press, 1985.
follow-up period. An expert consensus 2. Heimburger DC. Nutrition's interface with
panel has concluded that beta interferon health and disease. In: Bennett JC, Plum F,
may be helpful for patients with clinically editors. Cecil textbook of medicine.
definite MS who have had at least two acute Philadelphia: WB Saunders, 1996.
exacerbations in the previous two years. _____________________________

Reference:
Bradley WG, Daroff RB, Fenichel GM, et al.
Neurology in clinical practice. 2nd ed.
Boston: Butterworth-Heinemann, 1996.

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Question(s) 378 - 380: Clinical Pediatrics Question(s) 385: Behavioral

Discussion: Discussion:
Typical childhood absence seizures do not Lewy Body disease is a progressive
have auras or post-ictal confusion. dementing illness manifested by combined
Automatisms can be seen. The usual EEG cortical and subcortical clinical signs and
shows 3-4 Hz generalized spike-wave symptoms, and fluctuating episodes of acute
discharges and imaging studies of the brain confusion. Psychosis with delusions and
are normal. The initial drug of choice is visual hallucinations are common.
ethosuximide or valproate. Parkinsonism and gait disturbance are
prominent. Deficits in memory, executive
function, visual spatial and language are
Reference: usual. Patients with this disorder have
Pearl PL, Holmes GL. Absence seizures. In: significant susceptibility to adverse effects of
Pellock JM, Dodson WE, Bourgeois BFD, even low doses of neuroleptics. Lewy body
editors. Pediatric epilepsy: diagnosis and disease patients with hallucinations
therapy. 2nd ed. New York: Demos, 2001. demonstrate a relatively greater reduction in
_____________________________ choline acetyltransferase activity compared
Question(s) 381 - 383: Clinical Pediatrics to those patients without hallucinations.
Discussion:
Congenital myotonic dystrophy is inherited Reference:
from the mother, who may be relatively Papka M, Rubio A, Schiffer RB. A review of
asymptomatic. EMG and muscle biopsy of Lewy body disease, an emerging concept of
the baby is not helpful. EMG of the mother cortical dementia. J Neuropsychiatry Clin
can support the diagnosis. The diagnosis of Neurosci 1998;10(3):267-279.
the baby is confirmed by finding an _____________________________
abnormal expansion of a trinucleotide repeat
on chromosome 19. Children with congenital Question(s) 386:
myotonic dystrophy have delayed motor Pharmacology/Chemistry
milestones. Mental retardation is common. Discussion:
Nitrous oxide abuse can produce
myeloneuropathy that is clinically virtually
Reference: indistinguishable from that produced by
Dubowitz V. Muscle disorders in childhood. vitamin B12 deficiency. Typical symptoms
Philadelphia: WB Saunders, Co., 1995. include paresthesias in the hands and feet,
_____________________________ gait ataxia, and leg weakness. A so-called
Question(s) 384: Clinical Pediatrics reverse Lhermitte's sign, in which neck
Discussion: flexion induces electrical shock-like
Children, who have simple febrile seizures, sensations traveling from the feet upwards,
have a 2-4% risk of later, unprovoked non- may [Link] the setting of nitrous oxide
febrile seizures (epilepsy). The risk is 7% abuse the serum B12 level and the Schilling
when these patients are followed to age 25 test are almost always normal. Because
years. nitrous oxide interferes with the vitamin B12-
dependent conversion of homocysteine to
methionine and not with the conversion of
Reference: methylmalonyl coA to succinyl coA, only the
Rosman NP. Febrile seizures. In: Pellock homocysteine level will be elevated, in
JM, Dodson WE, Bourgeois BFD, editors. contrast to vitamin B12 deficiency where
Pediatric epilepsy: diagnosis and therapy. both homocysteine and methylmalonic acid
New York: Demos, 2001. levels will be elevated.
_____________________________

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Question(s) 388:
Reference: Pharmacology/Chemistry
Lockwood AH. Toxic and metabolic Discussion:
encephalopathies. In: Bradley WG, Daroff Temporal arteritis typically affects the elderly
RB, Fenichel GM, et al. Neurology in clinical and is ushered in by headaches. Jaw
practice. 2nd ed. Boston: Butterworth- claudication is a characteristic symptom and
Heinemann, 1996. low grade fever is often present. The
_____________________________ erythrocyte sedimentation rate is usually
markedly elevated. Treatment of temporal
Question(s) 387:
arteritis consists of high dose prednisone
Pharmacology/Chemistry
therapy. Low dose therapy will not protect
Discussion: from temporal arteritis-induced blindness.
Paralytic shellfish poisoning is caused by
consumption of bivalve mollusks (mussels,
clams, scallops, oysters) contaminated with Reference:
the dinoflagellate-produced toxin called Goodwin JA. Neuro-ophthalmologic
saxitoxin, which blocks sodium channels emergencies. In: Weiner WJ, editor.
and, thus, interferes with impulse conduction Emergent and urgent neurology.
in peripheral nerves and muscles. Philadelphia: JB Lippincott, 1992.
Respiratory failure can result. A number of _____________________________
other marine toxins can produce very similar Question(s) 389 - 390:
symptoms. Ciguatera toxin is another Pharmacology/Chemistry
dinoflagellate-derived toxin that
concentrates in certain large fish. Discussion:
Neurotoxic shellfish poisoning is due to Fabry disease is an X-linked recessive
consumption of oysters and clams disorder caused by deficiency of alpha-
contaminated with brevotoxin B, yet another galactosidase A. This enzyme deficiency
dinoflagellate toxin. Domoic acid poisoning causes accumulation of
occurs following consumption of mussels globotriaosylceramide (GB3) in multiple
contaminated with the marine diatom tissues. The most debilitating symptom is
Nitzchia pungens. Neurologic features pain, that may occur as attacks of burning
include confusion and altered states of pain in the palms and soles, or as a chronic
arousal. Seizures may also occur. Domoic nagging pain in the digits. This is a
acid is a glutamate agonist. Tetrodotoxin, manifestation of small fiber neuropathy, that
unlike the previously mentioned toxins, is may also produce autonomic dysfunction
produced by the puffer fish itself. Saxitoxin manifest as nausea, diarrhea, vomiting and
poisoning occurs in the Northeast and vascular instability of the extemities. GB3 is
Northwest USA, ciguatoxin poisoning in also deposited in the skin and its vessels,
Florida and Hawaii, brevotoxin B poisoning leading to the formation of angiokeratomas
in the Gulf of Mexico and Caribbean Sea, that may be sparse and restricted to the
domoic acid poisoning in eastern Canada scrotum and umbilicus. The eyes, kidneys,
and tetrodotoxin poisoning wherever puffer heart and cerebral circulation are also
fish are eaten, primarily in Japan. involved to a variable extent. Phenytoin
and/or carbamazepine in combination are
effective in treating the pain of Fabry
Reference: disease, and the other systemic
Ayyar R. Marine toxins. In: Bradley WG, manifestations may also be treated
Daroff RB, Fenichel GM, et al. Neurology in symptomatically. Enzyme replacement
clinical practice. 2nd ed. Boston: therapy has been shown to be effective for
Butterworth-Heinemann, 1996. relief of pain and other manifestations.
_____________________________

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Reference: Reference:
1. Desnick RJ, Ioannou Y, Eng CM. Alpha- Nierenberg DW, Nordgren RE, Chang MB,
galactosidase A deficiency: Fabry disease. et al. Delayed cerebellar disease and death
In: Scriver CR, Beaudet AL, Sly WS, et al. after accidental exposure to
The metabolic and molecular bases of dimethylmercury. N Engl J Med 1998;
inherited disease. 8th ed. New York: 338:1672-1676.
McGraw-Hill, 2001:3733-3774. _____________________________
2. Schiffmann R, Kopp JB, Austin HA, et al. Question(s) 394: Pathology
Enzyme replacement therapy in Fabry
disease. JAMA 2001;285:2743-2749. Discussion:
Multiple periventricular areas of
_____________________________
demyelination are characteristically seen in
Question(s) 391 - 393: multiple sclerosis. These plaques have a
Pharmacology/Chemistry grey hue because of the loss of myelin that
Discussion: normally confers a glistening white
Dimethylmercury is extremely toxic, and appearance to the white matter.
may be absorbed transdermally, or by
inhalation. Latex gloves are not protective.
Reference:
Dimethylmercury is rapidly converted to
methylmercury, which has a half-life of about Graham DI, Lantos PL. Greenfield's
78 days in humans. Long latent periods can neuropathology. 6th ed. London: Arnold,
occur between exposure and the onset of 1997.
symptoms in humans and monkeys. _____________________________
Pathologically, the injury involves the Question(s) 395: Pathology
cerebral cortex, and cerebellum. Neuronal Discussion:
necrosis and gliosis is seen. Chelation The histologic figure shows a distinctive
therapy with succimer will markedly increase form of keratin composed of clusters of
elimination of mercury from the body, but plump keratinocytes that is referred to as
may not be clinically beneficial unless it is "wet" keratin. Among tumors of the central
begun soon after exposure. Arsenic nervous system, this type of keratin is
exposure produces severe gastrointestinal unique to the adamantinomatous
symptoms with or without encephalopathy craniopharyngioma. Wet keratin nodules
acutely, and a progressive polyneuropathy frequently undergo dystrophic calcification.
with cutanous changes, anemia and In contrast, the keratin seen in epidermoid
jaundice with chronic exposure. and dermoid cysts consists of layers of very
Cisplatiunum may produce an acute thin, flat, flaky anucleate squamous cells.
encephalopthy, and a more chronic Papillary craniopharyngioma arises primarily
cerebellar syndrome with peripheral in adults rather than children and is
neuropathy and renal impairment. Lead composed of squamous epithelium that, in
poisoning in adults occurs with industrial contrast to that of the adamantinomatous
exposures to lead salts, and in conjunction variant, does not form significant amounts of
with inhalation of gasoline fumes (tetraethyl keratin. The lining of Rathke cleft cysts
and tetranethyl lead). A delirium may consists of pseudostratified ciliated
develop. Organic lead poisoning is usually columnar epithelium with goblet cells;
reversible. Thallium exposure is now although focal squamous metaplasia can
commonly associated with ingestion of occur, the distinctive nodules of wet keratin
rodenticides. It may produce a lethal characteristic of adamantinomatous
encepahlopathy acutely, or a painful craniopharyngioma are not seen.
progressive sensorimotor polyneuropathy
with optic atrophy and ophthalmoplegia.
Diffuse alopecia occurs two to four weeks
after ingestion.

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Question(s) 397: Neuroradiology

Reference: Discussion:
1. Kleihues P, Cavenee WK, editors. Contrast-enhanced CT shows a densely
Pathology and genetics of tumors of the enhancing mass projecting into the upper
nervous system. 2nd ed. New York: Oxford margin of the sella turcica. This could be
University Press, 2000. either an intra- or suprasellar lesion based
2. Fuller GN, Goodman JC. Practical review on the CT findings. As such it could be a
of neuropathology. Philadelphia: Lippincott pituitary adenoma, meningioma, aneurysm,
Williams & Wilkins, 2001. or possibly another lesion. The sagittal T1
_____________________________ weighted MR image shows a hypointense
flow void, such as is found in the lumen of
Question(s) 396: Neuroradiology an aneurysm. The only other thing that could
Discussion: mimic this on the MR scan would be a
Axial T2 weighted image shows a diffuse collection of air or cortical bone. The
high signal intensity throughout all of the combination of the CT and the MR indicate
white matter that is shown within the semi- an aneurysm. The pituitary gland can be
centrum ovale. The sulci are enlarged for seen below the aneurysm within the sella.
the patient's age. Thus the patient has a
diffuse process involving the white matter
that does not produce mass effect. This is Reference:
not normal, not typical of demyelinating Zimmerman RA, Bilaniuk LT, Hackney DB,
disease in the sense of multiple sclerosis, et al. MRI of cerebral aneurysm. Acta
making the subacute form of AIDS the most Radiologica 1987;369:107-109.
likely diagnosis. Pick's disease would _____________________________
involve more of the frontal and temporal Question(s) 398: Neuroradiology
lobes, while a glioblastoma would be a focal
mass lesion.
Discussion:
The lesion could be either radiation injury or
recurrent brain tumor. Hemorrhage is not
Reference: present. The lesion crosses anterior cerebral
Zimmerman RA, Bilaniuk LT, Sze G. and middle cerebral artery vascular
Intracranial infection. In: Brant-Zawadski M, boundaries, and spares the cortex, thus is
Norman D, editors. Magnetic resonance not a stroke. Moderate mass effect is
imaging of the brain. New York: Raven present. T2 prolongation (hyperintensity) is
Press, 1986. associated with the lesion, but marked T2
_____________________________ shortening (hypointensity-blackness) of the
lesion is not present. If clinically indicated,
the next logical imaging study would include
a functional nuclear neurology study such as
FDG-PET or Thallium-SPECT to
differentiate tumor from radiotherapy injury.

Reference:
Bakshi R, Lindsay BD, Kinkel PR. Brain
magnetic resonance imaging in clinical
neurology. In: Joynt RJ, Griggs RC, editors.
Clinical Neurology. Philadelphia: Lippincott,
Williams & Wilkins, 1998;1(4A):1-203.
_____________________________

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Question(s) 399: Pathology Question(s) 401: Neuroradiology

Discussion: Discussion:
The illustration depicts myofibrils with an The lesion lies in the motor cortex,
increased number of subsarcolemmal immediately anterior to the Rolandic
mitochondria, imparting a red coloration to (central) sulcus. Therefore, the correct
the muscle fiber: the "ragged red fiber." answer is frontal lobe.
Although ragged red fibers can occasionally
be seen in other conditions, they are most
commonly observed in the mitochondrial Reference:
myopathies. Electron microscopic Carpenter MB. Core text of neuroanatomy.
examination in such cases confirms the Baltimore: William & Wilkins, 1991
presence of abnormal or too-numerous _____________________________
mitochondria. Of the choices offered in the Question(s) 402: Neuroradiology
question, only Kearns-Sayre myopathy is a Discussion:
mitochondrial disease. Werdnig-Hoffman is The axial T1 weighted image shows an
a motor neuron disease; Duchenne's is an extra-axial hyperintense lesion that lies
X-linked-inherited myopathy involving between the hypointense inner table of the
abnormalities of dystrophin; central core skull and the isointense brain parenchyma.
myopathy is a genetic condition where The sulci are displaced and the cortex
muscle biopsy demonstrates the presence appears intact. There is a corresponding
of core-structures in the myofibrils; and displacement of the ventricular system. The
dermatomyositis is an autoimmune high signal intensity (T1 shortening) is
inflammatory myopathy. consistent with methemoglobin-containing
subacute clot in the subdural space.
Reference: Epidural hematoma is excluded because the
Graham DI, Lantos PL. Greenfield's blood collection crosses suture lines.
neuropathology. 6th ed. London: Arnold, Subdural hygroma and empyema are
1997. excluded because the signal is not
_____________________________ consistent with CSF or pus. En-plaque
meningioma is usually iso to hypointense on
Question(s) 400: Neuroradiology noncontrast T1-weighted images, although it
Discussion: could present a similar appearance on
The lesion in the right paracentral lobule is postcontrast images.
likely to be demyelinating. It affects the white
matter, but the ring of enhancement opens
when it touches the cortex of the medial Reference:
aspect of the hemisphere. The open ring Fujioka M, et al. Bilateral organized chronic
appearance of the ring of enhancement subdural haematomas: high field magnetic
("open-ring sign") is characteristic of resonance images and histological
demyelinating lesions. Histology was considerations. Acta Neurochir
confirmed by biopsy. Glioblastoma, 1994;131:265-269.
lymphoma and abscess tend to have a _____________________________
closed ring of enhancement. Astrocytoma
generally does not enhance.

Reference:
Masdeu JC, Quinto C, Olivera C, et al.
Open-ring imaging sign: highly specific for
atypical brain demyelination. Neurology
2000;54:1427-1433.
_____________________________

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Question(s) 403: Neuroradiology Question(s) 406: Neuroradiology

Discussion: Discussion:
The AP and lateral contrast myelogram The patient was treated for an obstructive
demonstrates narrowing of the dural sac at hydrocephalus by means of a ventriculo-
the disc space of multiple levels. The peritoneal shunt. The shunt track can be
narrowing of the dural sac is limited to the seen in the right peri-atrial white matter and
disc space consistent with spinal stenosis the shunt can be seen entering the atrium of
which is the combination of disc the lateral ventricle.
herniation/bulge and degenerative changes
of the posterior elements. Arachnoiditis Reference:
would not be limited to the disc space. The
Greenberg JO. Neuroimaging: a companion
roots are well seen without clustering. AV
to Adams and Victor's principles of
malformation is usually associated with
neurology. New York: McGraw-Hill, Co.,
tortuous, tangle of vessels. The spinal
1999.
ependymoma in this region usually occurs at
_____________________________
the end of conus. In this particular patient,
there is no evidence for neoplastic lesions. Question(s) 407: Clinical Adult
Discussion:
Reference: When an optic nerve lesion extends
posteriorly to involve the anterior chasm, the
Ullrich, et al. Quantitative assessment of the
earliest indication is the occurrence of a
lumbar spinal canal by computed
temporal field defect in the contralateral eye
tomography. Radiology 1980;134:137-141.
(usually, but not always in the upper
_____________________________ temporal field).
Question(s) 404: Neuroradiology
Discussion: Reference:
Axial T2-weighted image shows a symmetric
Miller NR. Walsh and Hoyt's clinical neuro-
brain with normal size ventricles. The frontal
ophthalmology. Baltimore: Williams &
lobe cortex is flat, and the white matter that
Wilkins, 1982.
is hypointense does not show the normal
_____________________________
interdigitation into the gray matter in this
region. Thus the cortex is thick, and the Question(s) 408: Neuroradiology
patient has pachygyria. Such an abnormality Discussion:
develops during the period of neuronal The small and medium sized vessels are
migration, occurring during the second ragged and demonstrate the segmented
trimester. pattern of vasculitis. While vasospasm is
possible, this typically affects larger vessels
Reference: than those affected in this case. This
appearance is known as "beads on a string".
Zimmerman RA, Bilaniuk LT. Pediatric
central nervous system. In: Stark DD,
Bradley WG, editors. Magnetic resonance Reference:
imaging. St. Louis: CV Mosby, 1988. Vanderzan TC, Bromberg M, MacGuire A, et
_____________________________ al. Isolated small-vessel angitis of the
Question(s) 405: Neuroradiology central nervous system. Arch Neurol
1988;45:683-687.
Discussion:
_____________________________
The middle cerebral artery and its branches
are absent. The anterior cerebral artery and
the posterior communicating and posterior
cerebral artery are present.

Reference:
Kirkwood JR. Essentials of neuroimaging.
New York: Churchill Livingston, 1992.

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Question(s) 409: Pathology Question(s) 411: Neuroradiology

Discussion: Discussion:
The characteristic inclusion body of rabies The most likely cause is a chiasmal glioma.
(Negri body) is classically seen in large The lesion clearly does not arise from the
pyramidal neurons and is located in the sella. It contains no cystic region or area of
cytoplasm. In contrast, Herpes simplex and signal void that would be consistent with a
cytomegalovirus produce Cowdry A craniopharyngioma.
intranuclear inclusions. Poliovirus produces
Cowdry B intranuclear inclusions during the
acute phase (not seen in post-polio Reference:
syndrome). Viral inclusions are not present Daniels DL, Haughton VM. Magnetic
in eastern equine encephalitis and many resonance imaging of the sella and temporal
other viral encephalitides in which only the bone. In: Kressel HY, editor. Magnetic
non-specific findings of perivascular resonance annual. New York: Raven Press,
lymphocytic infiltrates, microglial nodules l987.
and granular ependymitis are seen. _____________________________
Question(s) 412: Neuroradiology
Reference: Discussion:
1. Graham DI, Lantos PL. Greenfield's Both of the MR images are post-injection of
neuropathology. 6th ed. London: Arnold, gadolinium DTPA. Both images show
1997. separate and discrete ring-enhancing
2. Fuller GN, Goodman JC. Practical review lesions, one in the right occipital pole, and
of neuropathology. Philadelphia: Lippincott one in the left frontoparietal region. Mass
Williams & Wilkins, 2001. effect is clearly demonstrated on the left side
with compression and displacement of the
_____________________________
ventricular system. The right occipital lesion
Question(s) 410: Anatomy has a thin wall, while that on the left is
Discussion: somewhat thicker and more irregular. Both
The photomicrograph showed numerous lesions arise at the corticomedullary
subpial corpora amylacea. Corpora junctures. Given the age of the patient,
amylacea are polyglucosan bodies that clinical presentation, and the appearance of
accumulate with age in astrocyte the two lesions, multiple brain abscesses
cytoplasmic processes. They are most would be the most likely diagnosis. Infarcts
numerous in the subpial and perivascular tend to be more cortical, demyelinating
regions. Among the other answer choices, diseases, more periventricular and more
Cowdry A inclusions are brightly eosinophilic homogeneous in enhancement, occult
intranuclear inclusions seen in several vascular malformations may enhance, but it
different viral infections, including herpes is the core of the lesion, and not marginal
simplex, varcella zoster, cytomegalovirus enhancement, while resolving hematomas
and subacute sclerosing panencephalitis; would have central high signal intensity on a
Hirano bodies are oval-to-oblong T1-weighted image.
eosinophilic inclusions seen in the
hippocampus in Alzheimer's disease (and to
Reference:
a lesser extent in some normal aged
individuals); and Negri bodies are Bakshi R, Lindsay BD, Kinkel PR. Brain
eosinophilic cytoplasmic inclusions seen in magnetic resonance imaging in clinical
rabies. neurology. In: Joynt RJ, Griggs RC, editors.
Clinical Neurology. Philadelphia: Lippincott,
Williams & Wilkins, 1998;1(4A):1-203.
Reference: _____________________________
Fuller GN, Goodman JC. Practical review of
neuropathology. Philadelphia: Lippincott,
Williams & Wilkins, 2001.
_____________________________

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Question(s) 413: Clinical Adult Reference:

Stark DD, Bradley WG. Magnetic resonance
Discussion:
imaging. 2nd ed. St. Louis: Mosby Year
Hereditary motor and sensory neuropathy,
Book, Inc., 1992.
type I (HMSN - 1) is characterized by
marked slowing of nerve conduction _____________________________
velocities. It is most often inherited in an Question(s) 416: Neuroradiology
autosomal dominant pattern, with variable Discussion:
(but nearly complete) expressivity. The age The findings are consistent with
of onset is variable; family members who glioblastoma. Glioblastomas have
appear to be unaffected may simply be pre- characteristic imaging features on
symptomatic, and may have slow nerve postcontrast studies, which show intense,
conduction velocities. inhomogeneous, nodular, ring-like
enhancement, which encloses a central
Reference: isointense necrotic core, and delineates the
Dyck PJ, et al. Peripheral neuropathy. gross tumor margin. Anaplastic
Philadelphia: WB Saunder Co., 1993. astrocytoma, fibrillary astrocytoma,
meningioma, and oligodendroglioma tumors
_____________________________
typically do not show this bizarre ring-like
Question(s) 414: Anatomy enhancement.
Discussion:
The figure shows neurohypophyseal tissue
Reference:
obtained by transphenoidal biopsy of the
Bakshi R, Lindsay BD, Kinkel PR. Brain
pituitary. The arrows point to Herring
magnetic resonance imaging in clinical
bodies. Herring bodies are axonal storage
neurology. In: Joynt RJ, Griggs RC, editors.
sites for the hormones oxytocin and
Clinical neurology. Philadelphia: Lippincott-
vasopressin.
Raven, 1997.
_____________________________
Reference: Question(s) 417: Anatomy
1. Haines DA. Fundamental neuroscience.
New York: Churchill Livingston, 1997. Discussion:
The filum terminale is the terminal
2. Fuller GN, Goodman JC. Practical review
continuation of the spinal cord beginning at
of neuropathology. Philadelphia: Lippincott,
the lower end of the conus medullaris. It is
Williams & Wilkins, 2001.
composed principally of collagen from the
_____________________________ pia mater and blood vessels. In addition,
Question(s) 415: Neuroradiology small nerve fascicles are present (whose
Discussion: investing Schwann cells occasionally give
Chiari type II malformation is associated with rise to schwannomas) and, most
significant beaking or bending of the importantly, a remnant of the spinal cord
brainstem, not evident in the current case. central canal is also present, usually in the
The Dandy-Walker malformation occurs in form of an eccentrically-placed cluster of
the midline and typically includes ependymal cell nests and rosettes. The
maldevelopment of the cerebellar vermis significance of this central canal remnant
with separation of the cerebellar lies in the fact that these are the cells of
hemispheres. There are three types of origin of myxopapillary ependymomas,
hemangioblastomas, two of which may which arise almost exclusively from the
contain cysts. This cyst is contiguous with conus and filum.
the fourth ventricle, and resulted from
surgical excision of a cerebellar atrocytoma. Reference:
Arachnoid cysts occur extrinsic to the brain,
Fuller GN, Goodman JC. Practical review of
rather than within the ventricular system.
neuropathology. Philadelphia: Lippincott,
Williams & Wilkins, 2001.
_____________________________

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Question(s) 418: Physiology structures. Bisnwanger's disease occurs in

Discussion: the adult population due to chronic
Focal conduction block of compound muscle cerebrovascular disease.
action potentials from demyelination can be
seen in chronic inflammatory demyelinating Reference:
polyneuropathy, causing decreased Osborne A. Diagnostic neuroradiology. St.
amplitudes without axonal involvement Louis: CV Mosby, Inc., 1994.
always occuring. _____________________________
Question(s) 422: Neuroradiology
Reference: Discussion:
Kimura J. Electrodiagnosis in disease of The dilated third ventricle projects superiorly
nerve and muscle. 3rd ed. New York: Oxford between bodies of the lateral ventricles. A
University Press, 2001. corpus callosum is not seen, therefore this is
_____________________________ agenesis of the corpus callosum.
Question(s) 419: Neuroradiology
Discussion: Reference:
High density mass within parietal lobe with Atlas SW, Zimmerman RA, Bilaniuk LT, et
marked associated edema consistent with al. Corpus callosum and limbic system:
intraparenchymal hematoma. neuroanatomic evaluation of developmental
anomalies. Radiology 1086; 160:355-362.
Reference: _____________________________
Drurym I, Whisnant JP, Garraway WM. Question(s) 423: Neuroradiology
Primary intracerebral hemorrhage: impact of Discussion:
CT on incidence. Radiology 1985;154:563. The most likely diagnosis is autosomal
_____________________________ dominant spinocerebellar ataxia type 6
Question(s) 420: Physiology (proved by genetic testing). These patients
Discussion: have pure cerebellar atrophy, affecting
Myokymic discharges can be seen as predominantly the superior vermis. There is
myokymia on the skin surface, are rhythmic no pontine atrophy, a feature of both olivo-
and may be seen as singlets or multiplets. ponto-cerebellar atrophy and Machado
They are sometimes associated with Joseph disease. There is no downward
potassium channel antibodies. displacement of the cerebellar tonsils, and
the posterior fossa is not smaller than
normal, ruling out Chiari type I malformation.
Reference: Finally, the cerebellar atrophy results in a
Kimura J. Electrodiagnosis in disease of widened supracerebellar cistern, but without
nerve and muscle. 3rd ed. New York: Oxford any evidence of cerebellar compression,
University Press, 2001. which would be present with a subarachnoid
_____________________________ cyst.
Question(s) 421: Neuroradiology
Discussion: Reference:
Metachromatic leukodystrophy (MLD) is the Satoh JI, Tokumoto H, Yukitake M, et al.
correct response. MLD is characterized by Spinocerebellar ataxia type 6: MRI of three
extensive white matter demyelination that Japanese patients. Neuroradiology
spares the subcortical U-fibers. Confluent 1998;40:222-227.
periventricular demyelination is present. The _____________________________
anterior white matter is more severely
affected. Ceroid lipofuscinosis spares white
matter relative to grey matter. Leigh's
disease primarily affects basal ganglia

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Question(s) 424: Neuroradiology vacuo hydrocephalus refers to ventricular

enlargement due to brain atrophy, with
Discussion: concurrent sulcal/fissure expansion, not
The location and dense contrast seen in this case.
enhancement strongly suggest an anterior
communicating artery aneurysm.
Dolichectasia usually results in fusiform Reference:
aneurysm formation, most commonly in the Runge VM, Brack MA, Garneau RA, et al.
posterior circulation. Magnetic resonance imaging of the brain.
Philadelphia: JB Lippincott Co., 1994.
_____________________________
Reference:
Yock DH. Imaging of CNS disease: a CT Question(s) 427: Neuroradiology
and MR teaching file. Baltimore: Mosby, Discussion:
1991. Saggital T1 and axial proton density images
_____________________________ show abnormal hyperintensity filling the vein
Question(s) 425: Neuroradiology of Galen and straight sinus, instead of the
normal flow void expected. This is
Discussion: secondary to thrombus in these structures,
T2 weighted axial and sagittal images are and have resulted in hemorrhagic venous
shown. There is abnormally increased infarction in the thalami. Mass effect from
signal intensity in the parasagittal parietal the infarctions has compressed the third
and occipital lobes, extending into the ventricle, resulting in obstructive
splenium of the corpus callosum, where it hydrocephalus of the lateral ventricles. The
crosses the midline. This is typical axial image shows prominent basal veins of
appearance of an infiltrating neoplasm, most Rosenthal (seen on either side of the vein of
commonly astrocytoma. Posterior cerebral Galen as flow void), likely providing
artery infarct can involve the hemisphere collateral venous drainage. The pineal
and splenium, but would not cross the gland is normal in appearance, but
midline into the vascular territory of the right displaced inferiorly by the thrombus and
PCA. Schilder's disease is a demyelinating mass effect.
process and would not involve gray matter
as is seen in this case. There are no
abnormal vessels present to suggest AVM. Reference:
An acute hematoma has the opposite signal Bakshi R, Lindsay BD, Bates VE, et al.
characteristics than in this case, with Cerebral venous infarctions presenting as
hypointensity seen on both T1 and T2 enhancing space-occupying lesions: MRI
weighted images. findings. Journal of Neuroimaging
1998;8:210-215.
_____________________________
Reference:
Greenberg JO. Neuroimaging: a companion Question(s) 428: Pathology
to Adams and Victor's principles of Discussion:
neurology. New York: McGraw-Hill, Co., The photograph shows remote bilateral
1999. frontal contusions with a slight orange tinge
_____________________________ secondary to the presence of residual
Question(s) 426: Neuroradiology hemosiderin-laden macrophages. The
olfactory nerves are disrupted, which
Discussion: undoubtedly would have led to anosmia.
Marked enlargement of the lateral and third
ventricles is noted with small sulci indicating
obstructive (non-communicating) Reference:
hydrocephalus which may be seen in Graham DI, Lantos PL. Greenfield's
aqueductal stenosis. Changes in the CSF neuropathology. 6th ed. London: Arnold,
spaces are not seen to indicate 1997.
colpocephaly or schizencephaly. Chiari type _____________________________
I is characterized by syringomyelia. Ex-

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Question(s) 429: Pathology parietal lobes, and other posterior regions.

These findings are seen in 85% of patients
Discussion: studied within 72 hours after onset of
Lacunar infarcts, as seen in the gross symptoms. The relative lack of sympathetic
photograph in the thalamus, result from innervation in vertebrobasilar vasculature
arteriolar hyalinization caused by long- may predispose this region to the
standing hypertension. development of the cerebral edema.

Reference: Reference:
Graham DI, Lantos PL. Greenfield's Bakshi R, et al. Occipital lobe seizures as
neuropathology. 6th ed. London: Arnold, the major clinical manifestation of reversible
1997. posterior leukoencephalopathy: MRI
_____________________________ findings. Epilepsia 1998;39:295-299.
Question(s) 430: Pathology _____________________________
Discussion: Question(s) 432: Neuroradiology
The biopsy of a right parietal lobe mass Discussion:
showed a tumor composed of cells with Axial image demonstrates small, constricted
monotonous round nuclei surrounded by spinal canal. This is mainly due to
prominent perinuclear halos ("fried egg" degenerative facet joint disease. No disc
apppearance). These features are herniation or synovial cyst is present. The
characteristic of oligodendroglioma. Of the nerve roots are coiled above the narrow
other choices listed, ependymomas and area, since they cannot slide freely though
astroblastomas characteristically show the narrow part. Distal nerve roots are
perivascular pseudorosettes, with stout non- stretched. The correct answer is spinal
tapering cytoplasmic processes in the latter. stenosis.
Fibrillary astrocytomas and gemistocytic
astrocytomas show eosinophilic cytoplasm,
rather than the clear perinuclear halos of Reference:
oligodendrogliomas, with multiple elongated Enzman DR, et al. Magnetic resonance of
cytoplasmic processes in fibrillary the spine. St. Louis: CV Mosby, 1990.
astrocytomas and large rounded globular _____________________________
cytoplasm in gemistocytic astrocytomas. Question(s) 433: Neuroradiology
Discussion:
Reference: This abnormality is clearly a herniated disc.
Kleihues P, Cavenee WK, editors. Pathology The herniated material is contiguous with
and genetics of tumors of the nervous disc material (non-sequestered).
system. 2nd ed. New York: Oxford
University Press, 2000.
_____________________________ Reference:
Czervionke LF. Cervical spine. In: Daniels
Question(s) 431: Neuroradiology DL, Haughton VM, Naidich TP, editors.
Discussion: Cranial and spinal magnetic resonance
Hypertensive encephalopathy, a cause of imaging: atlas and guide. New York: Raven
reversible posterior leukoencephalopathy Press, 1987.
syndrome (RPLS), is a rapidly evolving _____________________________
condition that develops in patients with
malignant hypertension. If not promptly
recognized and aggressively treated it leads
to progressive neurologic deterioration and
death. Renal failure and eclampsia are
contributory causes. Similar changes are
also seen in toxicity due to
immunosuppressive agents. MRI and CT
shows edema located in the occipital and

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Question(s) 434: Pathology documented in the references. On the

coronal images note that the sulci at the
Discussion: high parietal convexity are compressed, as
The photomicrograph of the lumbar cistern compared to the markedly dilated posterior
mass shows large perivascular cuffs of extent of the Sylvian fissure.
mucin with adjacent ependymal cells.
These features are diagnostic of the
myxopapillary ependymoma, which arises Reference:
from ependymal remnants of the central 1. Holodny AI, George AE, de Leon MJ, et
canal in the filum terminale. al. Focal dilation and paradoxical collapse of
cortical fissures and sulci in patients with
normal-pressure hydrocephalus. J
Reference: Neurosurg 1998;89:742-747.
1. Graham DI, Lantos PL. Greenfield's
2. Kitagaki H, Mori E, Ishii K, et al. CSF
neuropathology. 6th ed. London: Arnold,
spaces in idiopathic normal pressure
1997.
hydrocephalus: morphology and volumetry.
2. Fuller GN, Goodman JC. Practical review AJNR Am J Neuroradiol 1998;19:1277-
of neuropathology. Philadelphia: Lippincott 1284.
Williams & Wilkins, 2001.
_____________________________
_____________________________
Question(s) 437: Neuroradiology
Question(s) 435: Neuroradiology
Discussion:
Discussion: This patient had documented E. faecalis
Contrast enhanced CT scan demonstrates a osteomyelitis affecting the L2 and L3
large, lobulated, densely enhancing vertebrae. There is a ring-enhancing lesion,
structure medial to the Sylvian fissure, with a affecting both of these vertebrae, rather than
large serpiginous structure leading from the simply the disc. Thus, there is more
lesion posteriorly towards the vein of Galen. pathology than can be explained simply by
This represents the nidus and a large discitis or expanding L3 neoplasm. There is
draining vein of an arteriovenous no evidence of an anterior or posterior spinal
malformation. Although AVM's can be approach to suggest surgery. The
easily diagnosed with enhanced CT and with expanding quality and ring enhancement are
MRI, angiography remains a necessity for not expected with hematoma and
complete evaluation of these lesions. compression fracture.
Selective injection of the intracerebral
vessels allows precise delineation of the
feeding arteries and draining veins, Reference:
something that cannot be determined on Osborne A. Diagnostic neuroradiology. St.
MRA or digital subtraction venous Louis: CV Mosby, Inc., 1994.
angiography - which are non-selective _____________________________
studies. Retrograde venography would not
Question(s) 438: Pathology
provide any information of the arterial supply
to the lesion. Discussion:
A coronal section of the brain at autopsy
shows mammillary bodies that are
Reference: congested and discolored, which is a
Osborne A. Diagnostic neuroradiology. St. characteristic finding in thiamine deficiency
Louis: CV Mosby, Inc., 1994. manifesting as Wernicke's encephalopathy.
_____________________________
Question(s) 436: Neuroradiology Reference:
Discussion: Graham DI, Lantos PL. Greenfield's
The correct diagnosis is "communicating neuropathology. 6th ed. London: Arnold,
hydrocephalus." Dilation of the Sylvian 1997.
fissure and other sulci may suggest atrophy. _____________________________
However, this feature is not uncommon in
shunt-responsive hydrocephalus, as

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Question(s) 439: Neuroradiology Question(s) 441: Pathology

Discussion: Discussion:
The most likely diagnosis is toxoplasmosis. The astrocyte illustrated in the
On MRI, toxoplasmosis lesions are most photomicrograph is a Creutzfeldt astrocyte.
commonly multiple and are located in the These reactive astrocytes are characterized
deep central grey nuclei, or lobar grey-white by multiple small nuclei (micronuclei) and
junction. Other common locations include abundant eosinophilic cytoplasm. Although
the posterior fossa, cerebral cortex, and not pathognomonic, they are typically seen
paraventricular white matter. in demyelinating diseases and serve as a
Toxoplasmosis lesions appear isointense to red flag to alert the pathologist to consider
hypointense on T1-weighted images and demyelinating disorders in the differential
mildly to markedly hyperintense on T2- diagnosis. There are a number of other
weighted images, exerting marked mass types of astrocytes with distinctive
effect. Copious edema is often noted which morphology. The Bergmann astrocytes of
is out-of-proportion to the lesion size. After the cerebellar cortex send long cytoplasmic
contrast administration, ring-like or nodular processes from the Purkinje cell layer to the
enhancement patterns are most commonly pial surface and show striking proliferation in
noted. Cytomegalovirus encephalitis and response to ischemic insult. Alzheimer type
progressive multifocal leukoencephalopathy II astrocytes can be seen in many types of
are usually not enhancing in this manner. liver failure that result in hyperammonemia.
Toxoplasmosis may be difficult to distinguish They differ from all other reactive astrocytes
from primary CNS lymphoma. Nocardia and in lacking discernible cytoplasm; rather, the
glioblastoma are much less likely in this nuclei are enlarged, pale, with only a thin
clinical setting. marginal rim of chromatin, and are often
irregular in shape. Superficial reactive
astrocytosis occurring in the normally very
Reference: hypocellular molecular layer is referred to as
Bakshi R, Lindsay BD, Kinkel PR. Brain Chaslin’s subpial gliosis. Finally, highly
magnetic resonance imaging in clinical atypical, bizarre reactive astrocytes can be
neurology. In: Joynt RJ, Griggs RC, editors. seen in progressive multifocal
Clinical neurology. Philadelphia: Lippincott- leukoencephalopathy (caused by infection
Raven, 1997. with the JC virus).
_____________________________
Question(s) 440: Physiology
Reference:
Discussion: Fuller GN, Burger PC. Central nervous
Incremental response of compound muscle system. In: Sternberg SS, editor. Histology
action potentials of >200% following for pathologists. 2nd ed. Philadelphia:
exercise, with or with or without decrement Lippincott-Raven, 1997.
on repetitive stimulation, is seen with _____________________________
Lambert-Eaton myasthenic syndrome. 50-
70% of cases of Lambert-Eaton myasthenic
syndrome are associated with cancer, most
commonly small cell carcinoma of the lung.

Reference:
Kimura J. Electrodiagnosis in disease of
nerve and muscle. 3rd ed. New York: Oxford
University Press, 2001.
_____________________________

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Question(s) 442: Pathology affected, appearing hypodense on CT and

hyperintense on long TR-short TE MRI.
Discussion: Fahr's disease is excluded by the absence
The photograph shows a biphasic cellular of calcification on the CT, Huntington's by
population consisting of small reactive the presence of normal caudate nuclei
lymphocytes and large neoplastic germ multiple sclerosis would be unusual due to
cells, which is characteristic of germinoma - gray matter location of lessions.
the most common pineal region tumor.
Pineocytomas, in contrast, are composed of
small mature pineocytes that form large Reference:
rosettes with fibrillary cores. Tom T, et al. J Neuroimaging 1996;6:161-
Pineoblastomas are densely cellular 166.
primitive neuroectodermal tumors that often _____________________________
form Homer Wright (neuroblastoma-type)
Question(s) 445: Neuroradiology
rosettes and occasionally fluerettes. Pineal
astrocytomas show prominent eosinophilic Discussion:
cytoplasmic processes. Regarding The hematoma has the typical concave
terminology, the designation "pinealoma" is appearance of subdural hematoma. The
an inaccurate, arcane and imprecise presence of abnormality immediately upon
historical relic that should not be used; it recovery from anaesthesia goes against the
dates to a time before a distinction was idea of infection and empyema is unlikely to
made between germinoma and be bright on T1 MRI. Meningioma is not
pineoblastoma. likely to be bright on T1 either and it is
typically isointense with gray matter and is
not likely to produce acute symptoms in
Reference: manner described. The subdural hematoma
Graham DI, Lantos PL. Greenfield's is hyperintense on both T1 and T2 images,
neuropathology. 6th ed. London: Arnold, and is a well known complication of spinal
1997. anesthesia and lumbar puncture.
_____________________________
Question(s) 443: Pathology Reference:
Discussion: Edelson, et al. Spinal subdural hematomas
Infarction with hemorrhage over an complicating lumbar puncture. Arch Neurol
extensive area of cortex and in the 1974.
underlying white matter of the cerebral _____________________________
hemisphere is consistent with sagittal sinus Question(s) 446: Anatomy
venous thrombosis. The lesion may be
either bilaterally symmetrical or prominently Discussion:
asymmetrical, as in the illustrated case. The photomicrographs show pigmented
Venous thrombosis is commonly associated cells in the leptomeninges that have
with hypercoagulable states and severe elongated cell processes. Of the various
dehydration. pigmented cells that might be encountered
in the central nervous system, the long
bipolar cytoplasmic processes and location
Reference: in the leptomeninges identify these cells as
Graham DI, Lantos PL. Greenfield's leptomeningeal melanocytes. These cells
neuropathology. 6th ed. London: Arnold, are normal constituents of the central
1997. nervous system and are found
_____________________________ predominantly in the leptomeninges of the
Question(s) 444: Neuroradiology upper cervical cord, medulla, and mesial
basal aspects of the brain stem and brain as
Discussion: far rostrally as the gyri recti.
The CT and proton density weighted MR
scans are classic for the findings of carbon
monoxide poisoning. The globus pallidi
structures of high metabolic rate are

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Question(s) 450: Anatomy

Reference: Discussion:
Fuller GN, Goodman JC. Practical review of The photomicrograph shows a
neuropathology. Philadelphia: Lippincott, meningothelial (arachnoid) cell cluster in the
Williams & Wilkins, 2001. fibrovascular stroma of the choroid plexus of
_____________________________ the lateral ventricular trigone (glomus
choroideum). Small nests of meningothelial
Question(s) 447: Pathology
cells like these are commonly found in the
Discussion: stroma throughout the choroid plexus and
The dysembryoplastic neuroepithelial tumor are a result of the developmental mode of
(DNT) is composed of neurons and formation of the choroid in which the
oligodendroglial-like cells clustered within vascular leptomeninges (including arachnoid
intracortical nodules. A prominent myxoid cells) invaginate into the ventricular cavity.
stroma is typically present within the tumor The clinical significance of these small
nodules. This indolent tumor is frequently arachnoid cell rests is that they occasionally
associated with cortical migration give rise to meningiomas (intraventricular
abnormalities. Surgery is curative. meningioma).

Reference: Reference:
Kleihues P, Cavenee WK, editors. Pathology Fuller GN, Goodman JC. Practical review of
and genetics of tumors of the nervous neuropathology. Philadelphia: Lippincott,
system. 2nd ed. New York: Oxford Williams & Wilkins, 2001.
University Press, 2000. _____________________________
_____________________________
Question(s) 451: Neuroradiology
Question(s) 448: Neuroradiology
Discussion:
Discussion: Pleomorphic xanthoastrocytoma typically
Of the available options, only the produce a large cyst with superficially
suppurative ventriculitis would cause positioned mural nodule. Most lesions arise
abnormal ependymal enhancement and the within the temporal or parietal lobes. This
CSF to appear hyperdense. Hemorrhage lesion is surrounded with minimal
into ventricle could have a similar hemosiderin rim, but no edema and this
appearance. The white matter shows excludes abscess and hematomas. The
marked edema. signal characteristics of cavernous
hemangioma are heterogeneous (popcorn).
Reference: The mass effect is too little for a hematoma
of this size. Porencephalic cysts do not exert
Yock DH. Imaging of CNS disease: a CT
mass effect.
and MR teaching file. Baltimore: Mosby,
1991.
_____________________________ Reference:
Question(s) 449: Neuroradiology Burger PC, et al. Surgical pathology of the
nervous system. 3rd ed. New York: Churchill
Discussion:
Livingston, 1991.
Multiple sclerosis plaques are shown. The
thoracic sensory level relates to plaques in _____________________________
the thoracic cord.

Reference:
Osborne A. Diagnostic neuroradiology. St.
Louis: CV Mosby, Inc., 1994.
_____________________________

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Question(s) 452: Neuroradiology Question(s) 454: Neuroradiology

Discussion: Discussion:
The uniform filling of this large vascular There is an intradural, extramedullary mass
anomaly and its position identify it as a vein in the thoracic spine causing significant cord
of Galen aneurysm which is a congenital compression. This represents either
anomaly. neurofibroma or meningioma, and requires
surgical removal. Neither tumor is
radiosensitive. Imaging with CT or
Reference: myelography is not likely to add any further
Bakshi R, Lindsay BD, Kinkel PR. Brain useful information.
magnetic resonance imaging in clinical
neurology. In: Joynt RJ, Griggs RC, editors.
Clinical neurology. Philadelphia: Lippincott, Reference:
Williams & Wilkins, 1998. Osborne A. Diagnostic neuroradiology. St.
_____________________________ Louis: CV Mosby, Inc., 1994.
Question(s) 453: Neuroradiology _____________________________
Discussion: Question(s) 455: Neuroradiology
Axial CTs show a large mass lesion filling Discussion:
the right orbit, compressing and deforming Focal, poorly marginated cortical lesion is
the posterior margin of the globe. The seen in the parasagittal left posterior frontal
inferior cut through the maxillary sinus lobe. The high signal on T1-weighted image
shows that the lateral wall of the maxilla is is due to T1 shortening and is consistent
destroyed and is associated with the soft with the presence of intracellular or
tissue mass. Metastatic disease commonly extracellular methemoglobin in a subacute
involves bone and adjacent soft tissue, as in hemorrhage. Of the lesions mentioned, only
this case. Facial trauma does not produce a hemorrhagic infarct and AVM would contain
disappearance of bone, but fractures it. subacute blood. The small size and cortical
Fibrous dysplasia expands bone but does location of the blood are more consistent
not destroy it. Hemangioma may involve with infarct than with vascular malformation.
bone and soft tissue, but is not a destructive The blood is in a vascular territory, most
process. In the orbit, capillary likely due to embolic occlusion of a
hemangiomas are commonly found in the paracentral branch of the anterior cerebral
cutaneous tissues and periorbita, but do not artery.
extend retroglobar and do not destroy bone.
Cavernous hemangiomas are discrete
masses that may be found intra- or Reference:
extracolonic, but do not usually cross these Bakshi R, Lindsay BD, Kinkel PR. Brain
margins. magnetic resonance imaging in clinical
neurology. In: Joynt RJ, Griggs RC, editors.
Clinical neurology. Philadelphia: Lippincott,
Reference: Williams & Wilkins, 1998.
Zimmerman RA, Bilaniuk LT. Computed _____________________________
tomography of the orbit. In: Lee S, Rao K,
editors. Cranial computerized tomography.
New York: McGraw-Hill, 1983.
_____________________________

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Question(s) 456: Anatomy Reference:

Osborne A. Diagnostic neuroradiology. St.
Discussion:
Louis: CV Mosby, Inc., 1994.
The photograph shows a cross section of
the brain stem at the superior pontine level. _____________________________
The locus ceruleus is identified by arrows Question(s) 459: Neuroradiology
bilaterally. The locus ceruleus is the major Discussion:
source of norepinephrine projections in the The scan shows sequelae of chronic herpes
central nervous system. simplex virus encephalitis.
Encephalomalacia is noted in both temporal
Reference: lobes, more prominent on the left with cystic
Haines DA. Fundamental neuroscience. change and tissue loss. The lesions are too
New York: Churchill Livingston, 1997. extensive for mesial temporal sclerosis. The
lesions cross vascular boundaries and are in
_____________________________
an usual location for strokes. Epidermoid
Question(s) 457: Pathology and arachnoid cysts are usually unilateral
Discussion: and not associated with surrounding
The lesion is a porencephalic cyst. The parenchymal hyperintensities.
lesion is partially covered by arachnoid and
extends from the subarachnoid space to the
Reference:
ventricular system. The lesion is typically
1. Bakshi R, Lindsay BD, Kinkel PR. Brain
smooth walled and surrounded by abnormal
magnetic resonance imaging in clinical
radially oriented gyri reflecting its
neurology. In: Clinical neurology. Joynt RJ,
development early in life.
Griggs RC, editors. Philadelphia: Lippincott-
Raven, 1997.
Reference: 2. Greenberg JO. Neuroimaging: a
1. Graham DI, Lantos PL. Greenfield's companion to Adams and Victor's principles
neuropathology. 6th ed. London: Arnold, of neurology. New York: McGraw-Hill, 1995.
1997. _____________________________
2. Duckett S. Pediatric neuropathology. Question(s) 460: Pathology
Baltimore: Williams & Wilkins, 1995.
Discussion:
_____________________________
The cell shown contains a neurofibrillary
Question(s) 458: Neuroradiology tangle - an intraneuronal intracytoplasmic
Discussion: inclusion composed of paired helical
This non-contrast CT reveals a hyperdense filaments. The section has been stained
tubular region in the proximal segment of the with a silver stain. Neurofibrillary tangles
left middle cerebral artery (MCA), consistent can be seen in a variety of conditions
with acute thrombosis (arrow). Also noted including Alzheimer disease, Pick disease,
are subtle left cerebral sulcal effacement, progressive supranuclear palsy, Down
hypodensity of the left basal ganglia, frontal, syndrome, Parkinson-dementia complex of
anterior parietal, and superior temporal Guam, and post-encephalitic Parkinsonism.
lobes. These findings are consistent with an
acute MCA infarction. This so-called dense
Reference:
MCA sign or hyperdense MCA sign has
Graham DI, Lantos PL. Greenfield's
been correlated angiographically with
neuropathology. 6th ed. London: Arnold,
embolic or atherothrombotic MCA occlusion.
1997.
The hyperdensity is most likely due to either
calcific or hemorrhagic components of the _____________________________
acute plaque. The hyperdense MCA sign is
nonspecific when present in isolation. False
positive hyperdense MCAs have been noted
in asymptomatic patients with high
hematocrit or calcific atherosclerotic
disease; these are usually bilateral.

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Question(s) 461: Neuroradiology Question(s) 463: Neuroradiology

Discussion: Discussion:
The non-enhanced CT scan demonstrates Sagittal T1-weighted and coronal proton
midline shift from right to left. On the right density weighted images show a high signal
side, there is noted extra-axial fluid intensity cortical-subcortical lesion in the
collection. The subdural hematoma is temporal lobe. This has the appearance of
crescent shaped. The extra-cerebral mass methemoglobin in that the lesion is bright on
typically locates over the frontal and parietal the T1 and the proton density image. Fat on
convexity. There are two different density the proton density image is becoming dark
regions: dependent portion is higher density as is seen in the scalp fat. In addition, there
than the brain tissue or the upper part of the is a rim of low signal intensity surrounding
hematoma. With unclotted blood the density the hematoma on the proton density image.
level may be seen from red blood cells from This is due to the presence of hemosiderin
settling into the dependent region. There is in macrophages surrounding the hematoma.
no need for further testing and craniotomy For this phenomenon to have occurred, the
should be performed. hematoma has to be approximately two
weeks or more in age.
Reference:
Yock DH. Imaging of CNS disease: a CT Reference:
and MR teaching file. Baltimore: Mosby, Gomori JM, Grossman RI, Goldberg HI, et
1991. al. Intracranial hematomas: imaging by high-
_____________________________ field MR. Radiology 1985;157:87-93.
Question(s) 462: Neuroradiology _____________________________
Discussion: Question(s) 464: Physiology
Elongated lesion demonstrating Discussion:
hyperintensity on T2-weighted images is The illustration shows typical centrotemporal
most consistent with multiple sclerosis (MS) spikes, often associated with benign rolandic
and represents focal demyelination. epilepsy of childhood, which is manifested
Degenerative myelomalacia may have these by clonic movements of the face and hand
signal characteristics and ill-defined that often progress to a more generalized
margins, but there is no adjacent spurring or seizure.
other degenerative bony disease to have
caused this problem. Ependymoma would
be seen as a more focal mass with cord Reference:
enlargement. Syrinx has well defined cavity Niedermeyer E, Lopes da Silva F.
margins, though would be of similar signal Electroencephalography. 4th ed. Baltimore:
intensity as the lesion pictured. The lesion Lippincott, Williams & Wilkins, 1998.
is too dorsal for motor neuron disease. MS _____________________________
cord lesions are usually one to two Question(s) 465: Neuroradiology
segments in length, or less. Discussion:
The angiogram shows the distal common
Reference: carotid artery and its branches. The proximal
1. Modic M, et al. Magnetic resonance internal carotid artery has a rounded stump
imaging of the spine. In: Stark DD, Bradley with no distal flow, consistent with occlusion
WG, editors. Magnetic resonance imaging. to atherosclerosis. A dissection usally has a
St. Louis: CV Mosby, 1988. tapered stump, not a rounded stump. Vessel
2. Bakshi R, et al. MRI findings in 22 cases occlusion in moya-moya is usually
of myelitis: comparison between patients intracraneal.
with and without multiple sclerosis. Eur J
Neurology 1998;5;35-48. Reference:
_____________________________ Watridge CB, Muhlbauer MS, Lowery RD.
Traumatic carotid artery dissection:
diagnosis and treatment. J Neurosurg 1989.

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 2002 AAN RITE Discussion & Reference Manual

Question(s) 466: Neuroradiology Reference:

Discussion: Greenberg JO. Neuroimaging: a companion
Right carotid arteriogram (AP and lateral to Adams and Victor's principles of
view, arterial phase). The arteriogram neurology. New York: McGraw-Hill, Co.,
demonstrates a "string of beads" in the 1999.
midportion of the internal carotid artery. The _____________________________
appearance is quite characteristic for Question(s) 469: Neuroradiology
fibromuscular dysplasia (FMD). FMD is a
Discussion:
nonatheromatous angiopathy of unknown
Both images demonstrate the missing
etiology. Characteristically FMD extend from
vermis thus the 4th ventricle connects with
C1-2 level to the entrance of the internal
the cisterna magna, the hallmark of Dandy-
carotid artery into petrous carotid canal.
Walker malformation. The posterior fossa is
FMD is bilateral in 65% of cases. Nearly one
enlarged.
third is of the cases are associated with
intracranial aneurysms.
Reference:
Bvarkovich AJ. Pediatric neuroimaging. St.
Reference:
Louis: CV Mosby, 1994.
Osborne A. Introduction to cerebral
_____________________________
angiography. Philadelphia: Harper & Row,
1980. Question(s) 470: Pathology
_____________________________ Discussion:
Question(s) 467: Neuroradiology The photomicrographs show classic
perivascular pseudorosettes characteristic of
Discussion: ependymoma.
Round soft tissue mass within the spinal
canal is located in the intradural,
extramedullary compartment. This is Reference:
evidenced by widening of the subarachnoid Kleihues P, Cavenee WK, editors. Pathology
space above and below the mass, and and genetics of tumors of the nervous
compression rather than enlargement of the system. 2nd ed. New York: Oxford
spinal cord. Of the choices mentioned, University Press, 2000.
meningioma is the only consistent intradural _____________________________
extramedullary mass; ependymoma,
astrocytoma and hemangioblastoma are
Question(s) 471: Physiology
intramedullary lesions. Lipomas are usually Discussion:
hyperintense on T1 weighted images. In a sensory nerve action potential
recording, the initial positivity corresponds to
the action potential passing beneath the
Reference: active recording electrode, and is used to
Osborne A. Diagnostic neuroradiology. St. measure the onset latency for terminal
Louis: CV Mosby, Inc., 1994. conduction velocity measures.
_____________________________
Question(s) 468: Neuroradiology Reference:
Discussion: Kimura J. Electrodiagnosis in disease of
The lesion at the tip of the left temporal lobe nerve and muscle. 3rd ed. New York: Oxford
spares the cortical ribbon and is clearly University Press, 2001.
extra-axial, ruling out old head trauma or _____________________________
herpes simplex encephalitis. The signal
characteristics are typical of CSF, ruling out
a metastasis. The lesion on the right
perimesencephalic cistern cannot be
incisural sclerosis, characterized by gliosis,
not by a CSF-like lesion like the one shown.
Both lesions are arachnoid cysts.

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 2002 AAN RITE Discussion & Reference Manual

Question(s) 472: Physiology images, with long TR and long TE, would
show hyperintense CSF. Fat saturation MR
Discussion: images would suppress the thin bright rim
Jitter on single fiber EMG can be seen with overlying the skull (subcutaneous fat). The
diseases of neuromuscular transmission structure pointed to by (A) shows the sylvian
such as myasthenia gravis as well as a (lateral) fissure/circular sulcus region. This
denervating neuropathy such as separates the frontal and temporal lobes.
amyotrophic lateral sclerosis. The structure pointed to by (B) shows the
trigone/atrium of lateral ventricle. The
Reference: structure marked by (C) shows the genu of
Kimura J. Electrodiagnosis in disease of corpus callosum. The structure marked by
nerve and muscle. 3rd ed. New York: Oxford (D) shows the frontal paranasal sinus. The
University Press, 2001. bright superficial rim around the skull is
_____________________________ subcutaneous fat (E), because it is bright on
T1-weighted image, due to T1 shortening
Question(s) 473 - 477: Neuroradiology effect. None of the other structures typically
Discussion: cause T1 shortening. Hyperintensity on T1-
The petrosal segment of the carotid artery weighted image results from shortening of
(C) has a vertical and horizontal segment, is T1 relaxation time. This is seen in a variety
just distal to the cervical portion of the of tissues on noncontrast images, most
carotid, and is followed by the cavernous commonly, blood, fat, calcium, and protein-
portion of the carotid artery. The ophthalmic rich tissue.
artery arises from the anterior surface of the
carotid artery and is a good marker for the
carotid artery piercing the dura. The Reference:
posterior communicating artery (A) connects 1. Westbrook K. MRI in practice. London:
the distal internal carotid artery (B) and Blackwell, 1993.
proximal posterior cerebral artery. Anterior 2. Bushong SC. Magnetic resonance
cerebral artery (D) and anterior imaging: physical and biological principles.
communicating artery (E) are also shown. St. Louis: CV Mosby, 1996.
3. Bakshi R, Ketonen L. Brain MRI in clinical
neurology. In: Joynt RJ, Griggs RC, editors.
Reference: Baker’s clinical neurology. Philadelphia:
Osborne A. Introduction to cerebral Lippincott, Williams & Wilkins, 2001.
angiography. Philadelphia: Harper & Row,
1987.
_____________________________
Question(s) 478 - 480: Neuroradiology
Discussion:
The images are MR T1-weighted images
(long TR/shortTE). This is apparent because
of the marked hypointensity of the CSF in
relation to brain parenchyma. T2-weighted

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