Residency In-service

Training Exam
Discussion and
Reference Manual
2003 Test Dates • February 28, March 1, 2

AMERICAN ACADEMY OF NEUROLOGY

Executive Office
1080 Montreal Avenue
St. Paul, MN 55116
Direct RITE questions to:
Phone: 651-695-2706
Fax: 651-361-4806
E-mail: lwiener@[Link]
 2003 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

American Psychiatric Association. Diagnostic and statistical manual of mental disorders.
4th rev. ed. Washington: American Psychiatric Association, 2000.

Arana GW, Rosenbaum JF. Handbook of psychiatric drug therapy. 4th ed. Philadelphia:
Lippincott, Williams & Wilkins, 2000.

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, 1999.

Brazis PW, Masdeu JC, Biller J. Localization in clinical neurology. 4th ed. Philadelphia:
Lippincott, Williams & Wilkins, 2001.

Burger PC, Scheithauer BW, Vogel FS. Surgical pathology of the nervous system and its
coverings. 4th ed. New York: Churchill Livingston, 2002.

Fuller GN, Goodman JC. Practical review of neuropathology. Philadelphia: Lippincott,

Williams & Wilkins, 2001.

Graham DI, Lantos PL. Greenfield's neuropathology. 7th ed. New York: Arnold Press,
2002.

Greenberg JO. Neuroimaging: a companion to Adams and Victor's principles of

neurology. New York: McGraw-Hill, Co., 1999.

Haines DE. Fundamental neuroscience. 2nd ed. New York: W.B. Saunders, 2002.

Kandel ER, Schwartz JH, Jessel TM. Principles of neural science. 4th ed. New York:
McGraw-Hill, 2000.
 2003 AAN RITE Discussion & Reference Manual

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

Kryger MH, Roth T, Dement WC. Principles and practice of sleep medicine. 3rd ed.
Philadelphia: WB Saunders Company, 2000.

Leigh RJ, Zee DS. The neurology of eye movements. 3rd ed. New York: Oxford
University Press, 1999.

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

Mesulam MM. Principles of behavioral and cognitive neurology. 2nd ed. New York:
Oxford University Press, 2000.

Niedermeyer E, Lopes da Silva F, editors. Electroencephalography: basic principles,

clinical applications, and related fields. 4th ed. Philadelphia: Lippincott, Williams &
Wilkins, 1998.

Osborn AG. Diagnostic neuroradiology. St. Louis: CV Mosby, Inc., 1994.

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

Scriver CR, Beaudet AL, Sly WS, et al, editors. The molecular and metabolic bases of
inherited disease. 8th ed. New York: McGraw-Hill, 2001.

Swaiman KF, Ashwal S, editors. Pediatric neurology. 3rd ed. St. Louis: Mosby, 1999.
 2003 AAN RITE Discussion & Reference Manual

Question(s) 1: Behavioral Question(s) 3: Clinical Adult

Discussion: Discussion:
Mild memory impairment or subtle changes When the eye is rotated outward about 23
in other cognitive functions that do not degrees, the superior rectus is a pure
interfere with daily activities and for which no elevator.
other underlying cause can be found
constitute Mild Cognitve Impairment (MCI).
Reference:
Brazis PW, Masdeu JC, Biller J. Localization
Reference: in clinical neurology. 4th ed. Philadelphia:
Bennett DA, Wilson RS, Schneider JA, et al. Lippincott, Williams & Wilkins, 2001.
Natural history of mild cognitive impairment _____________________________
in older persons. Neurology 2002;59:198- Question(s) 4: Physiology
205.
_____________________________ Discussion:
In acquired demyelinating polyneuropathy,
Question(s) 2: Clinical Pediatrics multifocal demyelination results in abnormal
Discussion: temporal dispersion of the compound
Ataxia telangiectasia commonly presents muscle action potential.
with recurrent infections before the onset of
conjunctival and malar telangiectasia, which
develop before nystagmus and ataxia. Reference:
Patients with ataxia telangiectasia, unlike Kimura J. Electrodiagnosis in disease of
those with other immunodeficiencies, nerve and muscle. 3rd ed. New York: Oxford
develop B-cell neoplasia such as lymphoma University Press, 2001.
and Hodgkin's disease. Bassen-Kornzweig _____________________________
syndrome is characterized by Question(s) 5: Physiology
malabsorption, peripheral neuropathy and Discussion:
pigmentory retinopathy that responds to The 3 Hz spike-and-wave pattern is
therapy with vitamin E. Friedreich's ataxia is facilitated by hyperventilation, alkalosis,
a progressive, autosomal recessive disorder hypoglycemia and drowsiness, but is
presenting between age 10 and 12. diminished during REM sleep.
Spinocerebellar ataxia type III is a
trinucleotide repeat disorder that presents in
adulthood. Refsum disease (phytanic acid Reference:
oxidase deficiency) presents with ataxia, Niedermeyer E, Lopes da Silva F, editors.
retinitis pigmentosa and icthyosis. Electroencephalography: basic principles,
clinical applications, and related fields. 4th
ed. Philadelphia: Lippincott, Williams &
Reference:
Wilkins, 1998
1. Oski FA, DeAngelis CD, Feigin RD, et al.
_____________________________
Principles and practice of pediatrics.
Philadelphia: JB Lipincott, 1990.
2. Menkes JH, Sarnat HB, editors. Child
neurology. Philadelphia: Lippincott, Williams
& Wilkins, 2000.
_____________________________

1
 2003 AAN RITE Discussion & Reference Manual

Question(s) 6: Clinical Adult Question(s) 9: Pharmacology/Chemistry

Discussion: Discussion:
Dementia is now recognized as an important The prominent manifestation of congenital
feature of Parkinson's disease, eventually deficit of dopamine-beta-hydroxylase is
occurring in about a third of patients (though orthostatic hypotension. Elevated levels of
reported incidence varies widely between plasma dopamine and undetectable levels of
studies). Dementia is also a major symptom plasma norepinephrine are diagnostic
of progressive supranuclear palsy (PSP), features of this disorder. DL threo-
but the manifestations may be overlooked or dihydroxyphenylserine (DL-threo-DOPS) is
attributed to the dysarthria and eye an unantural amino acid that is converted to
movement abnormalities. Mental norepinephrine through a single
deterioration occurs in about 85% of patients decarboxylation step by the dopa
with juvenile Huntington's disease. Higher decarboxylase, bypassing the dopamine-
cortical functions are often altered in beta-hydroxylase step, and is very effective
Wilson's disease. Machado-Joseph disease for management of orthostatic hypotension
is a hereditary disorder with variable in this disorder.
manifestations that can include dystonia,
athetosis, rigidity, spasticity, lower motor Reference:
neuron signs, and ataxia, but intelligence
Biaggioni I, Roberstson D. Endogenous
remains intact.
restoration of noradrenaline by precursor
therapy in dopamine-beta-hydroxylase
Reference: deficiency. A novel orthostatic syndrome.
Bradley WG, Daroff RB, Fenichel GM, et al, Lancet 1987;1(8526):183-188.
editors. Neurology in clinical practice. 3rd _____________________________
ed. New York: Butterworth-Heinemann, Question(s) 10: Clinical Adult
1999.
Discussion:
_____________________________ Leukemics have a very high incidence of
Question(s) 7: Clinical Adult leptomeningeal involvement (up to 70% of
Discussion: patients) although prophylactic CNS
The pronator quadratus is responsible for treatment has markedly reduced the
pronation of the forearm when the elbow is incidence of symptomatic disease.
flexed. It is supplied by the anterior
interosseous nerve, a branch of the median Reference:
nerve.
Bradley WG, Daroff RB, Fenichel GM, et al,
editors. Neurology in clinical practice. 3rd
Reference: ed. New York: Butterworth-Heinemann,
Brown WF, Bolton CF, editors. Clinical 1999.
electromyography. 2nd ed. Boston: _____________________________
Butterworth-Heinemann, 1993. Question(s) 11: Physiology
_____________________________ Discussion:
Question(s) 8: Physiology Injury to the deep peroneal nerve results in
Discussion: weakness of foot and toe dorsiflexion with
Bilateral absence of cortical responses decreased recruitment in the anterior tibialis
(N20) on median somatosensory evoked muscle and sensory loss in the web space
potentials is associated with a poor outcome between the first two toes.
in comatose patients.
Reference:
Reference: Kimura J. Electrodiagnosis in disease of
Chiappa KH. Evoked potentials in clinical nerve and muscle. 3rd ed. New York: Oxford
medicine. 3rd ed. New York: Lippincott- University Press, 2001.
Raven Press, 1997. _____________________________
_____________________________

2
 2003 AAN RITE Discussion & Reference Manual

Question(s) 12: Anatomy Question(s) 15: Anatomy

Discussion: Discussion:
Light first encounters the innermost layer of The armpit's dermatome is usually T2.
the retina, the ganglion cell layer.
Reference:
Reference: Parent A. Carpenter's human
Haines DE. Fundamental neuroscience. 2nd neuroanatomy. 9th ed. Baltimore: Williams &
ed. New York: W.B. Saunders, 2002. Wilkins, 1996.
_____________________________ _____________________________
Question(s) 13: Clinical Adult Question(s) 16: Physiology
Discussion: Discussion:
CADASIL (cerebral autosomal dominant Mu activity is attenuated by movement of an
arteriopathy with subcortical infarcts and extremity.
leukoencephalopathy) is a hereditary
disorder that presents with ischemic strokes,
dementia, migraine with aura, and Reference:
emotional-intellectual disturbances. Specific Fisch B. Spehlmann's EEG primer. 2nd ed.
diagnosis can be made by skin-muscle Amsterdam: Elsevier Science Publications,
biopsy which demonstrates thickening of 1991.
smooth arteriopathic muscle cells that _____________________________
eventually degenerate. Electron microscopy Question(s) 17: Behavioral
can diagnose the disease by identifying Discussion:
granular, osmophilic materials in arterial Motor and verbal tics are required for a
smooth muscle. clinical diagnosis of Tourette's syndrome.
Motor tics alone are not sufficient for the
Reference: diagnosis of Tourette's syndrome and
Bousser MG, Tournier-Lasserve EJ. coprolalia is not required (as it occurs in only
Cerebral autosomal dominant arteriopathy approximately 30% of cases).
with subcortical infarcts and
leukoencephalopathy: from stroke to vessel Reference:
wall phsiology. J Neurol Neurosurg Moore DP. Textbook of clinical
Psychiatry 2001;70:285-287. neuropsychiatry. New York: Oxford
_____________________________ University Press, 2001.
Question(s) 14: Physiology _____________________________
Discussion: Question(s) 18: Clinical Adult
Sensory responses would be expected to be Discussion:
affected in lesions distal to the dorsal root A drowsy patient with a right hemiplegia and
ganglion (ie., plexopathies); while they eyes deviated to the left that conjugately
would be spared in lesions proximal to the cross the midline with the doll's eye
dorsal root ganglion (i.e., radiculopathies). maneuver is more likely due to a frontal
Thus, they are quite useful in distinguishing lesion than a lateral medullary, occipital,
between root and plexus localizations. pontine, or thalamic lesion.

Reference: Reference:
Brown WF, Bolton CF, editors. Clinical Brazis PW, Masdeu JC, Biller J. Localization
electromyography. 2nd ed. Boston: in clinical neurology. 4th ed. Philadelphia:
Butterworth-Heinemann, 1993. Lippincott, Williams & Wilkins, 2001.
_____________________________ _____________________________

3
 2003 AAN RITE Discussion & Reference Manual

Question(s) 19: Behavioral Question(s) 22: Clinical Adult

Discussion: Discussion:
Mirtazapine is sedating even at lower doses. Tardive dyskinesia and parkinsonian
It would not be safe to use it in a cross- symptoms are hazards of metoclopramide
country truck driver. Fluoxetine, bupropion, administration, especially in the elderly.
desipramine, and sertraline are far less
sedating than mirtazapine.
Reference:
Bradley WG, Daroff RB, Fenichel GM, et al,
Reference: editors. Neurology in clinical practice. 3rd
Arana GW, Rosenbaum JF. Handbook of ed. New York: Butterworth-Heinemann,
psychiatric drug therapy. 4th ed. 1999.
Philadelphia: Lippincott, Williams & Wilkins, _____________________________
2000. Question(s) 23: Clinical Adult
_____________________________
Discussion:
Question(s) 20: Physiology The most useful laboratory study for
Discussion: diagnosing amyotrophic lateral sclerosis
In a lesion of the peroneal nerve above the (ALS) is the EMG, which shows widespread
knee, the short head of the biceps femoris changes of denervation. Spinal fluid is
muscle will show evidence of denervation in usually normal, but mild protein elevation
addition to the tibialis anterior and peroneus (less than 100 mg/dl) may occur. While
longus muscles. serum creatine kinase levels are typically
normal, they may be increased to two to
three times normal in almost half the
Reference: patients with ALS. Sedimentation rate is
Kimura J. Electrodiagnosis in disease of usually normal.
nerve and muscle. 3rd ed. New York: Oxford
University Press, 2001.
_____________________________ Reference:
Question(s) 21: Physiology Brooke MH. A clinician's view of
neuromuscular diseases. 2nd ed. Baltimore:
Discussion: Williams & Wilkins, 1986.
The femoral nerve innervates muscles _____________________________
involved in hip flexion and knee extension.
Its sensory territory includes the territory of Question(s) 24: Physiology
the saphenous nerve below the knee. Discussion:
In an acute lumbar radiculopathy, the only
abnormal findings on a needle EMG
Reference: examination performed within a few days of
Kimura J. Electrodiagnosis in disease of onset of symptoms may be reduced
nerve and muscle. 3rd ed. New York: Oxford recruitment of motor unit potentials.
University Press, 2001.
_____________________________
Reference:
Kimura J. Electrodiagnosis in disease of
nerve and muscle. 3rd ed. New York: Oxford
University Press, 2001.
_____________________________

4
 2003 AAN RITE Discussion & Reference Manual

Question(s) 25: Anatomy Reference:

Discussion: Aminoff MJ. Neurology and general
medicine. 3rd ed. New York: Churchill
Microglial cells are of mesodermal origin and
Livingston, 2001.
have a phagocytic function when central
nervous system tissue is injured. Schwann _____________________________
cells, ependymal cells, tanycytes and Question(s) 29: Clinical Pediatrics
oligodendrocytes are of ectodermal origin. Discussion:
Myotonic dystrophy is an autosomal
Reference: dominant disorder that is associated with
Parent A. Carpenter's human CTG repeats at the 19q13.3 locus. Maternal
neuroanatomy. 9th ed. Baltimore: Williams & inheritance can lead to a severe form of
Wilkins, 1996. neonatal myotonic dystrophy. Testicular
atrophy is usually not associated with
_____________________________
sterility.
Question(s) 26: Physiology
Discussion: Reference:
The peripheral neuropathy associated with
Swaiman KF, Ashwal S, editors. Pediatric
monoclonal gammopathy is demyelinating in
neurology. 3rd ed. St. Louis: Mosby, 1999.
character. Nerve conduction velocities in the
teens or twenties would be characteristic of _____________________________
this entity. Question(s) 30: Pharmacology/Chemistry
Discussion:
Reference: The main site of disposal of manganese in
Dyck PJ, Thomas PK, Griffin JW, et al, the body is biliary excretion. Patients with
editors. Peripheral neuropathy. 3rd ed. biliary atresia, chronic liver disease, or
Philadelphia: WB Saunders, 1993. exposure to high dose of manganese during
prolonged parenteral nutrition, are prone to
_____________________________
develop manganese intoxication. Clinically,
Question(s) 27: Behavioral it is characterized by parkinsonism and
Discussion: dystonia, which do not respond to levodopa.
Borderline personality disorder is manifested T1-weighted MRI shows hyperintensity in
by a wide range of psychiatric and the globus pallidus, striatum, and midbrain.
behavioral features, but it has a single The primary site of damage is the globus
consistent characteristic - instability of pallidus.
mood.
Reference:
Reference: Pal PK, Samili A, Calne DB. Manganese
American Psychiatric Association. neurotoxicology: a review of clinical
Diagnostic and statistical manual of mental features, imaging, and pathology.
disorders. 4th rev. ed. Washington: Neurotoxicology 1999;20:227-238.
American Psychiatric Association, 2000. _____________________________
_____________________________
Question(s) 28: Clinical Adult
Discussion:
Hypothyroid myopathy is commonly
associated with painful cramps, normal
reflexes, and impressive elevations in serum
creatine kinase (CK) levels. Percussion of
muscle commonly causes a slow,
prolonged, electrically silent local mounding
called myoedema.

5
 2003 AAN RITE Discussion & Reference Manual

Question(s) 31: Pharmacology/Chemistry approach to anatomy, pathology and

physiology by systems and levels. 4th ed.
Discussion: New York: Lippincott, Williams & Wilkins,
Rotenone is commonly used as an herbicide 1999.
and is a potent inhibitor of mitochondrial
_____________________________
complex I. Chronic administration of
rotenone in rats reproduces several findings Question(s) 35: Physiology
of Parkinson's disease, including selective Discussion:
loss of susbtantia nigra compacta neurons Temporal lobe seizures are associated with
and accumulation of inclusions resembling confusion, contralateral posturing of the
Lewy bodies. hand, automatisms, and difficulty speaking.
This constellation of findings suggests an
origin in the left temporal lobe.
Reference:
Betarbet R, Sherer TB, MacKenzie G, et al.
Chronic systemic pesticide exposure Reference:
reproduces features of Parkinson’s disease. 1. Walker M, Shorvon S. Partial epilepsy
Nat Neurosci 2000;3:1301-1306. syndromes in adults. In: Porter RJ,
_____________________________ Chadwick D, editors. The epilepsies 2.
Question(s) 32: Physiology Boston: Butterworth-Heinemann, 1997.
2. Williamson PD, Engel J. Complex partial
Discussion: seizures. In: Engel J, Pedley TA, editors.
Fusimotor fibers transmit impulses to
Epilepsy: a comprehensive textbook. New
muscles spindles.
York: Lippincott-Raven, 1998.
_____________________________
Reference: Question(s) 36: Pathology
Kandel ER, Schwartz JH, Jessel TM.
Discussion:
Principles of neural science. 4th ed. New
Vitamin B12 deficiency produces combined
York: McGraw-Hill, 2000.
system degeneration, with damage to the
_____________________________ posterior columns and lateral columns.
Question(s) 33: Physiology Associated megaloblastic anemia may be
Discussion: present. Amyotrophic lateral sclerosis
The amplitude of the facial nerve CMAP is classically produces neuroaxonal loss of the
correlated with the number of functional upper and lower motor neurons with sparing
axons in the facial nerve. The lower the of the sensory columns; posterior column
CMAP amplitude the more axonal involvement is rarely seen in familial ALS.
degeneration and the poorer the prognosis. Anterior spinal artery thrombosis spares the
posterior columns supplied by the paired
posterior spinal arteries. Neurosyphilis
Reference: produces tabes dorsalis as its primary spinal
Kimura J. Electrodiagnosis in disease of cord manifestation, with demyelination and
nerve and muscle. 3rd ed. New York: Oxford axonal loss in the posterior columns but with
University Press, 2001. sparing of the anterior corticospinal tracts.
_____________________________ Vitamin E deficiency produces dystrophic
Question(s) 34: Physiology axons in the posterior columns.
Discussion:
Postcentral cortical cells that have small Reference:
receptive fields are likely to have their Fuller GN, Goodman JC. Practical review of
receptive fields on the distal parts of neuropathology. Philadelphia: Lippincott,
contralateral extremities. Williams & Wilkins, 2001.
_____________________________
Reference:
Benarroch EE, Westmoreland BF, Daube
JR, et al. Medical neurosciences - an

6
 2003 AAN RITE Discussion & Reference Manual

Question(s) 37: Behavioral Question(s) 39: Pathology

Discussion: Discussion:
Electroconvulsive therapy (ECT) may In males suspected of having Duchenne's or
improve both mood and motor symptoms in Becker's muscular dystrophy, dystrophin
patients with advanced Parkinson's disease analysis may reveal the presence of a
and major depression. ECT is safer than mutation in affected individuals. Carrier
tricyclic antidepressants in patients with analysis can also be performed. The precise
cardiac conduction disturbances. location and size of the dystrophin mutation
Nefazodone, amitriptyline, and imipramine determines whether the more severe
would be poor choices because of their Duchenne's phenotype or the milder
potential cardiac side effects and in the case Becker's phenotype will be seen.
of the later two drugs their anticholinergic
properties. Anticholinergics, such as
benztropine, are not effective in treating Reference:
major depression and may cause cardiac Graham DI, Lantos PL. Greenfield's
side effects, memory loss, and confusion in neuropathology. 7th ed. New York: Arnold
the elderly. Press, 2002.
_____________________________
Question(s) 40: Physiology
Reference:
Rasmussen K, Abrams R. Treatment of Discussion:
Parkinson's disease with electroconvulsive Averaging in evoked potential testing
therapy. (Review) Psychiatric Clinics of increases the signal to noise ratio.
North America. Philadelphia: WB Saunders,
Co., 1991. Reference:
_____________________________ Harper CM. Somatosensory evoked
Question(s) 38: Anatomy potentials. In: Daube JR, editor. Clinical
Discussion: neurophysiology. Philadelphia: FA Davis
Ballism is associated with discrete lesions in Company, 1996.
the subthalamic nucleus. The dyskinesia _____________________________
occurs contralateral to the lesion and is Question(s) 41: Physiology
associated with hypotonia. Discussion:
Paraspinal muscle denervation usually
Reference: indicates a lesion at the level of the ventral
Haines DE. Fundamental neuroscience. 2nd nerve root.
ed. New York: W.B. Saunders, 2002.
_____________________________ Reference:
Kimura J. Electrodiagnosis in disease of
nerve and muscle. 3rd ed. New York: Oxford
University Press, 2001.
_____________________________

7
 2003 AAN RITE Discussion & Reference Manual

Question(s) 42: Clinical Adult Question(s) 45: Physiology

Discussion: Discussion:
The pain that occurs with meralgia Diminished slow component peak velocity
paresthetica (damage to the lateral with cool and warm water caloric testing on
cutaneous nerve of the thigh) is often widely one side during ENG indicate an ipsilateral
distributed and can include the low back, peripheral vestibular abnormality.
buttock, anterolateral thigh, and lateral knee
regions. It is often aggravated by standing Reference:
and relieved by sitting. Paresthesias and
Brey RH. Vertigo and balance. In: Daube
hypesthesia usually involve a much more
JR, editor. Clinical neurophysiology.
limited area in the anterolateral thigh. The
Philadelphia: FA Davis Company, 1996.
syndrome is often associated with obesity or
_____________________________
pregnancy, and symptoms disappear with
weight loss or delivery of the baby. Question(s) 46: Clinical Adult
Discussion:
Reference: The blood supply to the mid-thoracic (T4-T6)
cord is relatively tenuous making this region
Stewart JD. Focal peripheral neuropathies.
of the spinal cord most vulnerable to
New York: Elsevier, 1987.
ischemia.
_____________________________
Question(s) 43: Anatomy
Reference:
Discussion:
Bradley WG, Daroff RB, Fenichel GM, et al,
This patient has a cervical central spinal
editors. Neurology in clinical practice. 3rd
cord syndrome manifested by lower motor
ed. New York: Butterworth-Heinemann,
findings in his hands, upper motor neuron
1999.
hyper-reflexia in his legs and a classical
_____________________________
sensory disturbance in which secondary
order neurons subserving pain and thermal Question(s) 47: Clinical Adult
sense are interrupted as they decussate in Discussion:
the central spinal cord. The primary order Cerebellum is a more frequent site of CNS
neuronal axons in the dorsal columns metastases than brain stem, leptomeninges,
subserving fine touch and proprioception are thalamus or spinal cord.
spared. The central spinal cord syndrome
most commonly results from syringomyelia
or intrinsic tumors. Reference:
Bradley WG, Daroff RB, Fenichel GM, et al,
editors. Neurology in clinical practice. 3rd
Reference: ed. New York: Butterworth-Heinemann,
Patten J. Neurological differential diagnosis. 1999.
2nd ed. New York: Springer, 1996. _____________________________
_____________________________
Question(s) 48: Pathology
Question(s) 44: Physiology
Discussion:
Discussion: Devic's disease is also known as
Damage to autonomic fibers in the leg can neuromyelitis optica; the spinal cord and
result in tachycardia, vasodilation, optic nerves are predominantly involved
hypotension, loss of sweating, and loss of although other regions of the CNS may
nerve terminals that produce acetylcholine. show lesions indistinguishable from classic
multiple sclerosis.
Reference:
Low PA. Clinical autonomic disorders: Reference:
evaluation and management. 2nd ed. New Graham DI, Lantos PL. Greenfield's
York: Lippincott-Raven, 1997. neuropathology. 7th ed. New York: Arnold
_____________________________ Press, 2002.

8
 2003 AAN RITE Discussion & Reference Manual

Question(s) 49: Clinical Adult rudimentary. Knowledge of this variability is

important in order to avoid attributing
Discussion: asymmetry of the occipital horns to disease.
Alpha coma refers to the rare occurrence of
clinical coma associated with EEG activity in
the alpha frequency range (8-13 Hz) that is Reference:
unresponsive to external stimuli. It has been Haines DE. Fundamental neuroscience. 2nd
reported with hypoxia, drug overdose, and ed. New York: W.B. Saunders, 2002.
lesions in the pontomesencephalic region. _____________________________
Prognosis depends on etiology.
Question(s) 53: Behavioral
Discussion:
Reference: The hallmark of the acute confusional state
Niedermeyer E, Lopes da Silva F, editors. is impaired attention.
Electroencephalography: basic principles,
clinical applications, and related fields. 4th
ed. Philadelphia: Lippincott, Williams & Reference:
Wilkins, 1998. Taylor MA. The fundamentals of clinical
_____________________________ neuropsychiatry. New York: Oxford
University Press, 1999.
Question(s) 50: Clinical Adult _____________________________
Discussion: Question(s) 54: Physiology
The suprascapular nerve may be damaged
by trauma or by compression within the Discussion:
suprascapular notch (e.g., by a ganglion or Wave V of the brainstem auditory evoked
by the suprascapular ligament). The affected potential is thought to be generated at the
muscles are the supraspinatus and level of the inferior colliculus and an
infraspinatus, responsible for initial absence of wave V would suggest a lesion
abduction and external rotation of the of the lower midbrain at the level of the
shoulder, respectively. inferior colliculus.

Reference: Reference:
Stewart JD. Focal peripheral neuropathies. Chiappa KH. Evoked potentials in clinical
New York: Elsevier, 1987. medicine. 3rd ed. New York: Lippincott-
_____________________________ Raven Press, 1997.
_____________________________
Question(s) 51: Pharmacology/Chemistry
Question(s) 55: Physiology
Discussion:
Suboptimal doses of carbidopa is a frequent Discussion:
cause of nausea/vomiting at the initiation of In a patient with myxedema, a single
levodopa therapy. It takes 100-150 mg of stimulus to a nerve results in slow
carbidopa per day to saturate the peripheral contraction and relaxation of the muscle.
aromatic aminoacid decarboxylase enzyme
to prevent peripheral side effects of Reference:
levodopa.
Kimura J. Electrodiagnosis in disease of
nerve and muscle. 3rd ed. New York: Oxford
Reference: University Press, 2001.
Roger E, Kurlan JB. Treatment of movement _____________________________
disorders. Philadelphia: Lippincott Co.,
1995.
_____________________________
Question(s) 52: Anatomy
Discussion:
The occipital horn exhibits a high degree of
variability and asymmetry and is often

9
 2003 AAN RITE Discussion & Reference Manual

Question(s) 56: Anatomy Reference:

Discussion: Niedermeyer E, Lopes da Silva F, editors.
Electroencephalography: basic principles,
The area postrema, a circumventricular
clinical applications, and related fields. 4th
organ, lacks a blood-brain barrier. It acts as
ed. Philadelphia: Lippincott, Williams &
a chemoemetic center.
Wilkins, 1998.
_____________________________
Reference: Question(s) 60: Clinical Adult
Fuller GN, Goodman JC. Practical review of
neuropathology. Philadelphia: Lippincott, Discussion:
Williams & Wilkins, 2001. While not always the earliest finding, the
diagnosis of progressive supranuclear palsy
_____________________________
is confirmed by the appearance of vertical
Question(s) 57: Anatomy gaze paresis.
Discussion:
Although they are part of the sympathetic
Reference:
nervous system, the postganglionic fibers to
Watts RL, Koller WC. Movement disorders:
the sweat glands (sudomotor fibers) are
neurologic principles and practice. New
cholinergic.
York: McGraw-Hill, Co., 1997.
_____________________________
Reference: Question(s) 61: Anatomy
Haines DE. Fundamental neuroscience. 2nd
ed. New York: W.B. Saunders, 2002. Discussion:
Compression of the optic apparatus at the
_____________________________
junction of the optic nerve and the optic
Question(s) 58: Clinical Adult chiasm will damage fibers streaming to the
Discussion: chiasm from the ipsilateral eye as well as
Patients with severe hypermagnesemia may fibers from the contralateral eye’s nasal
also have low calcium and cardiac retina subserving temporal visual space.
conduction abnormalities. Treatment The patient has a junctional scotoma
involves administration of intravenous consisting of ipsilateral visual loss and a
calcium gluconate. Patients should be contralateral temporal visual field defect. For
monitored closely for impending respiratory those inclined toward eponyms, this is
failure, and if it occurs, they should be known as the anterior chiasmal syndrome of
immediately intubated and mechanically Traquair, and the looping forward fibers from
ventilated (preferably with simultaneous the contralateral eye are known as Von
calcium administration). Saline and calcium Wilbrand’s knee.
are used together if the patient is not in renal
failure. Hypermagnesemia associated with
Reference:
renal failure requires hemodialysis for
Patten J. Neurological differential diagnosis.
treatment.
2nd ed. New York: Springer, 1996.
_____________________________
Reference: Question(s) 62: Physiology
Riggs JE, Neurologic manifestations of fluid
and electrolyte disturbances. Neurol Clin Discussion:
1989;7:509. Phenytoin reduces post-tetanic potentiation.
_____________________________
Question(s) 59: Physiology Reference:
Discussion: Levy RH, Mattson RH, Meldrum BS, editors.
Antiepileptic drugs. 4th ed. New York:
A myoclonic seizure is the most common
Raven Press, 1995.
type of seizure induced by photic stimulation
in patients with light sensitivity. _____________________________

10
 2003 AAN RITE Discussion & Reference Manual

Question(s) 63: Clinical Adult Question(s) 66: Anatomy

Discussion: Discussion:
Cluster headache is characterized by severe The terminal branches of the posterior cord
unilateral headache associated with of the brachial plexus are the radial and
autonomic symptoms. Headache attacks axillary nerves.
typically last 15 minutes to two to three
hours. A Horner's syndrome may occur on Reference:
the side of the headache. The entity is much
Brazis PW, Masdeu JC, Biller J. Localization
more common in men than women and
in clinical neurology. 4th ed. Philadelphia:
usually occurs in young individuals (mean
Lippincott, Williams & Wilkins, 2001.
onset 28 years).
_____________________________
Question(s) 67: Pharmacology/Chemistry
Reference:
Discussion:
Raskin NH. Headache. 2nd ed. New York:
Anandamide is an endogenous ligand for
Churchill-Livingstone, 1998.
the cannabinoid receptor, which has been
_____________________________ implicated in several functions including pain
Question(s) 64: Physiology control. In addition, anandamide is an
Discussion: endogenous ligand of the vanilloid 1
The cerebellum exerts its effect on synergy receptor (VR1), which is activated by heat
of movement by way of the and low pH and mediates the effects of
dentatorubrothalamic pathway. capsaicin on primary nociceptive afferents.

Reference: Reference:
Kandel ER, Schwartz JH, Jessel TM. Di Marzo V, Bisogno T, De Petrocellis L.
Principles of neural science. 4th ed. New Anandamide: some like it hot. Trends
York: McGraw-Hill, 2000. Pharmacol Sci 2001;22:346-349.
_____________________________ _____________________________
Question(s) 65: Anatomy Question(s) 68: Clinical Adult
Discussion: Discussion:
Secondary auditory fibers from the cochlear Symptoms of perilymph fistula are variable,
nuclei form the dorsal, intermediate, and but may include recurrent vestibulopathy.
ventral acoustic striae. The dorsal and Characteristic precipitating factors include
intermediate striae cross the midline and cough, sneeze, straining, and exercise.
enter the lateral lemniscus. The fibers of the
ventral stria terminate in the superior olivary Reference:
nuclei and the nucleus of the trapezoid
Baloh RW, Honrubia V. Clinical
body. These nuclei give rise to tertiary fibers
neurophysiology of the vestibular system.
that enter the lateral leminsci. The lateral
Philadelphia: FA Davis Co., 1990.
lemniscus ascends to the midbrain where
_____________________________
most of the fibers terminate in the inferior
colliculi.

Reference:
1. 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.
2. Haines DE. Fundamental neuroscience.
2nd ed. New York: W.B. Saunders, 2002.
_____________________________

11
 2003 AAN RITE Discussion & Reference Manual

Question(s) 69: Behavioral Question(s) 72: Behavioral

Discussion: Discussion:
The rapid course of the dementia with Alexia without agraphia (also known as pure
myoclonus, visual and cerebellar impairment word blindness or acquired pure alexia) is
(wide-based gait) is suggestive of most often produced by left posterior
Creutzfeldt-Jakob disease. EEG often cerebral artery occlusion. A frequent
reveals pseudoperiodic high voltage sharp associated finding is a right homonymous
and slow waves superimposed on an hemianopsia.
increasingly slow and low voltage
background. These sharp waves are usually Reference:
synchronous with myoclonus, but may
Damasio A, Tranel D, Rizzo M. Disorders of
persist in the absence of myoclonus. CSF
complex visual processing. In: Mesulam
immunoassay of peptide fragments of brain
MM, editor. Principles of behavioral and
proteins known as 14-3-3, (although not
cognitive neurology. 2nd ed. New York:
pathognomonic for a specific disease), in
Oxford University Press, 2000.
this clinical setting, is consistent with
_____________________________
Creutzfeldt-Jakob disease.
Question(s) 73: Pharmacology/Chemistry
Reference: Discussion:
Reduced concentrations of coenzyme Q-10
Victor M, Ropper A. Principles of neurology,
(CoQ-10) have been found in patients with
7th ed. New York: McGraw-Hill, 2001.
myopathy complicating the use of statins.
_____________________________ Primary CoQ-10 deficiency produces either
Question(s) 70: Clinical Adult a myopathy or progressive cerebellar
Discussion: degeneration, reflecting the key role of CoQ-
Saddle anesthesia, sphincter loss and loss 10 as an electron shuttle between
of ankle reflexes after a fall signify midline complexes I or II and complex III in the
disk herniation with compression of the respiratory chain.
caudal equina.
Reference:
Reference: 1. Musumeci O, Naini A, Slonim AE.
Bradley WG, Daroff RB, Fenichel GM, et al, Familiar cerebellar ataxia with muscle
editors. Neurology in clinical practice. 3rd coenzyme Q-10 deficiency. Neurology
ed. New York: Butterworth-Heinemann, 2001;56: 849-855.
1999. 2. Farmer JA. Learning from the cerivastatin
_____________________________ experience. Lancet 2001;358:1383-1384.
Question(s) 71: Behavioral _____________________________

Discussion: Question(s) 74: Clinical Pediatrics

Anticholinergic side effects are of Discussion:
considerable significance in the Between 60% and 75% of children with
pharmacologic treatment of depression. epilepsy who have been seizure-free for
Among those that are most likely to cause more than two years on medications will
these side effects are the tricyclic remain seizure-free for 2 years when anti-
antidepressants, especially the tertiary epileptic drugs are withdrawn.
amines such as amitriptyline.
Reference:
Reference: Pellock JM, Dodson WE, Bourgeois BFD,
Arana GW, Rosenbaum JF. Handbook of editors. Pediatric epilepsy, diagnosis and
psychiatric drug therapy. 4th ed. treatment. 2nd ed. New York: Demos, 2001.
Philadelphia: Lippincott, Williams & Wilkins, _____________________________
2000.
_____________________________

12
 2003 AAN RITE Discussion & Reference Manual

Question(s) 75: Anatomy material into the subarachnoid space

occurs.
Discussion:
Mastication is spared with a facial nerve
lesion. The facial nerve conveys special Reference:
visceral efferent fibers (to the buccinator), Youmans JR, editor. Neurological surgery: a
general visceral efferent fibers (to comprehensive reference guide to the
parasympathetic ganglia), special visceral diagnosis and management of neurosurgical
afferent fibers (from taste buds), and general problems. Philadelphia: WB Saunders
somatic afferent fibers (from the skin of the Company, 1996.
external auditory canal). _____________________________
Question(s) 79: Pharmacology/Chemistry
Reference: Discussion:
Haines DE. Fundamental neuroscience. 2nd Recurrent stupor is a rare disorder
ed. New York: W.B. Saunders, 2002. associated with excessive accumulation of
_____________________________ endozepine-4, an endogenous ligand of the
Question(s) 76: Clinical Adult benzodiazepine receptor. These patients
show elevated endozepine levels in blood
Discussion: and cerebrospional fluid. The
A lesion of the posterior interosseous nerve electroenecphalogram during the episodes
typically results in a "finger drop" rather than resemble that found in patients taking
wrist drop, because the branches to the benzodiazepines. Flumazenil induces
extensor carpi radialis brevis and longus transient (10-15 min) of wakefulness in
muscles usually arise from the main trunk of these patients.
the radial nerve proximal to the origin of the
posterior interosseous nerve. The triceps
and brachioradialis muscles are spared for Reference:
similar reasons. In contrast, the supinator Lugaresi E, Montagna P, Tinuper P, et al.
muscle is innervated by the posterior Endozepine stupor: recuring stupor linked to
interosseous nerve (as it pierces the endozepine-4 accumulation. Brain
muscle). 1998;121:127-133.
_____________________________
Reference: Question(s) 80: Physiology
Stewart JD. Focal peripheral neuropathies. Discussion:
New York: Elsevier, 1987. The EEG in Huntington's chorea shows a
_____________________________ low voltage background.
Question(s) 77: Physiology
Discussion: Reference:
Periodic sharp waves and/or spikes are Niedermeyer E, Lopes da Silva F, editors.
most likely to be seen with a post-anoxic Electroencephalography: basic principles,
state. clinical applications, and related fields. 4th
ed. Philadelphia: Lippincott, Williams &
Wilkins, 1998.
Reference:
_____________________________
Niedermeyer E, Lopes da Silva F, editors.
Electroencephalography: basic principles,
clinical applications, and related fields. 4th
ed. Philadelphia: Lippincott, Williams &
Wilkins, 1998.
_____________________________
Question(s) 78: Clinical Adult
Discussion:
Chemical meningitis is a rare complication of
craniopharyngioma in which seeding of cyst

13
 2003 AAN RITE Discussion & Reference Manual

Question(s) 81: Clinical Adult Reference:

Discussion: Benson DF. Aphasia, alexia, and agraphia.
New York: Churchill Livingston, 1979.
Limb, cranial nerve and autonomic
dysfunction commonly accompany Guillain- _____________________________
Barré syndrome, but sensory loss is rare. In Question(s) 84: Anatomy
those who may not have received Discussion:
vaccination, the diagnosis of diphtheria The nucleus ambiguus contains motor
should be considered. This is especially true neurons that supply striated muscles of the
when symptoms are preceded by palate, pharynx and larynx; therefore,
pharyngeal exudation. Diphtheria typically disruption of this nucleus will impair
begins with a pharyngeal infection and phonation.
exudate, sometimes followed by local palatal
neuropathy. This may be followed by
paralysis of pupillary accommodation. About Reference:
10% of patients develop a diffuse motor and Haines DE. Fundamental neuroscience. 2nd
sensory polyneuropathy about 8-12 weeks ed. New York: W.B. Saunders, 2002.
after the pharyngeal infection. Cerebrospinal _____________________________
fluid protein is usually elevated. Question(s) 85: Behavioral
Discussion:
Reference: In most instances, the duration of transient
Bradley WG, Daroff RB, Fenichel GM, et al, global amnesia is less than 12 hours,
editors. Neurology in clinical practice. 3rd produces no other focal neurologic
ed. New York: Butterworth-Heinemann, abnormalities and usually disappears
1999. without residua. The amnesia affects recall
_____________________________ of recent events and impairs new learning,
Question(s) 82: Behavioral but spares digit span (immediate recall).
Discussion:
Similarities between Alzheimer's disease Reference:
and Down's syndrome include the presence Hodges JR, Warlow CP. Syndromes of
of typical neuropathological changes transient amnesia: towards a classification.
(plaques and neurofibrillary tangles) and A study of 153 cases. Neurol, Neurosurg,
abnormalities of chromosome 21. Down's and Psychiatry 1990;53:834-843.
syndrome individuals routinely develop the _____________________________
pathology of Alzheimer's disease by their Question(s) 86: Clinical Adult
late 30s/early 40s. By the time Down's
patients are age 50 years, over 50% will Discussion:
experience seizures. In those with dementia Metoclopramide has been implicated as a
the figure approaches 80%. cause of the neuroleptic malignant
syndrome in some cases.

Reference:
Moore DP. Textbook of clinical
Reference:
neuropsychiatry. New York: Oxford Aminoff MJ. Neurology and general
University Press, 2001. medicine. 3rd ed. New York: Churchill
_____________________________ Livingston, 2001.
_____________________________
Question(s) 83: Behavioral
Discussion:
Transcortical motor and sensory aphasia
patients retain the ability to repeat. Broca's,
Wernicke's, and conduction aphasia as well
as pure word deafness all have impairments
of repetition.

14
 2003 AAN RITE Discussion & Reference Manual

Question(s) 87: Clinical Adult Reference:

Discussion: McEvoy KM, Windebank AJ, Daube JR, et
al. 3,4-diaminopyridine in the treatment of
Following ischemic stroke, motor recovery
Lambert-Eaton myasthenic syndrome.
tends to plateau more quickly than functional
NEJM 1989;321:1567-1571.
recovery with only small changes seen after
8-12 weeks. Arm movement generally _____________________________
recovers less than leg movement. Question(s) 90: Anatomy
Hemiparesis recovers significantly over the Discussion:
course of a year following stroke with severe The arcuate fasciculus connects the frontal
hemiplegia persisting in only 9% of patients. gyri with parts of the temporal lobe.
More than 50% of patients are able to sit
unsupported in the first week after stroke.
Reference:
Haines DE. Fundamental neuroscience. 2nd
Reference: ed. New York: W.B. Saunders, 2002.
Dombovy M. Rehabilitation and the course _____________________________
of recovery after stroke. In: Whisnant JP,
editor. Stroke: populations, cohorts, and Question(s) 91: Anatomy
clinical trials. Boston: Butterworth- Discussion:
Heinemann, 1993. The middle cerebellar peduncle consists of
_____________________________ crossed afferent fibers from the pontine
Question(s) 88: Behavioral nucleus, the pontocerebellar tract.
Discussion:
Any Alzheimer's patient who acutely Reference:
develops symptoms of an acute confusional Brazis PW, Masdeu JC, Biller J. Localization
state and behavioral changes first warrants in clinical neurology. 4th ed. Philadelphia:
a workup to look for the underlying cause. Lippincott, Williams & Wilkins, 2001.
Even mild changes in metabolic status, _____________________________
medications, or an infection such as a Question(s) 92: Clinical Adult
urinary tract infection may precipitate
confusion and behavioral changes. Discussion:
Patients with McArdle's disease and
phosphofructokinase deficiency do not have
Reference: a normal rise in serum lactate with the
Cummings JL, Benson DF. Dementia: a ischemic exercise test.
clinical approach. Boston: Butterworth-
Heinemann, 1992.
Reference:
_____________________________
Engel AG, Franzini-Armstrong C. Myology.
Question(s) 89: Pharmacology/Chemistry New York: McGraw Hill, 1994.
Discussion: _____________________________
3,4-diaminopyrimidine (3,4-DAP) is effective
in improving both neuromuscular and
autonomic manifestations of the Lambert-
Eaton myasthenic syndrome. This disorder
is due to an immune-mediated blockade of
prejunctional P/Q type voltage-gated
calcium channels responsible for
acetylcholine release. 3,4-DAP blocks the
voltage-gated potassium channels
responsible for repolarization of the action
potential, and thus increases action potential
duration and opening of the P/Q channels.

15
 2003 AAN RITE Discussion & Reference Manual

Question(s) 93: Pathology Reference:

Discussion: 1. Brazis PW, Masdeu JC, Biller J.
Localization in clinical neurology. 4th ed.
Lewy bodies, both cortical and brainstem
Philadelphia: Lippincott, Williams & Wilkins,
type, stain with antibodies directed against
2001.
alpha synuclein. The gene for synuclein,
located on chromosome 4, is mutated in rare 2. Victor M, Ropper A. Adams and Victor's
familial forms of Parkinson's disease. principles of neurology. 7th ed. New York:
Various types of inclusions in sporadic forms McGraw-Hill, Co., 2001.
of Parkinson's disease, diffuse Lewy body _____________________________
disease, and multiple system atrophy can be Question(s) 96: Behavioral
identified by alpha-synuclein staining; these Discussion:
three disorders are considered Major depression is frequent in Parkinson's
synucleinopathies. In contrast, "tauopathies" disease, occurring in 40 to 60% of patients
include Alzheimer's disease, Pick's disease, during the course of their illness. It is less
frontotemporal dementia with parkinsonism common in dementia of the Alzheimer type
(FTDP-17), progressive supranuclear palsy, or Pick's disease. In addition, depression is
and corticobasal ganglionic degeneration. not associated with Anton's syndrome or
Kluver-Bucy syndrome.
Reference:
1. Dickson, DW. Tau and synuclein and their Reference:
role in neuropathology. Brain Pathol Cummings JL, Benson DF. Dementia: a
1999;9:657-661. clinical approach. Boston: Butterworth-
2. Fuller GN, Goodman JC. Practical review Heinemann, 1992.
of neuropathology. Philadelphia: Lippincott, _____________________________
Williams & Wilkins, 2001.
_____________________________ Question(s) 97: Behavioral
Question(s) 94: Anatomy Discussion:
Nortriptyline is an antidepressant with a well-
Discussion: defined therapeutic window. It is less
The common peroneal nerve divides into effective at both very low and very high
two branches, the deep and superficial doses.
peroneal nerves. The deep peroneal nerve
innervates the tibialis anterior and extensor
digitorum brevis. The peroneus longus and Reference:
brevis are supplied by the superficial Arana GW, Rosenbaum JF. Handbook of
peroneal nerve. The soleus is innervated by psychiatric drug therapy. 4th ed.
the tibial nerve. Philadelphia: Lippincott, Williams & Wilkins,
2000.
_____________________________
Reference:
Brazis PW, Masdeu JC, Biller J. Localization Question(s) 98: Physiology
in clinical neurology. 4th ed. Philadelphia: Discussion:
Lippincott, Williams & Wilkins, 2001. The parasympathetic nerves exiting at the
_____________________________ S2-S4 dorsal roots mediate the urge to
Question(s) 95: Clinical Adult urinate.
Discussion:
The initial ocular motor deficit in patients Reference:
with progressive supranuclear palsy consists Benarroch EE, Westmoreland BF, Daube
of impairment of vertical saccades, with JR, et al. Medical neurosciences - an
downward saccades usually affected first. approach to anatomy, pathology and
Tremor is rare. Axial rigidity is greater than physiology by systems and levels. 4th ed.
extremity rigidity, postural instability with New York: Lippincott, Williams & Wilkins,
falls is common, and dementia is often mild. 1999.
_____________________________

16
 2003 AAN RITE Discussion & Reference Manual

Question(s) 99: Clinical Adult Reference:

Discussion: Lupski JR, Chance PF, Garcia CA. Inherited
Clinical features with Horner's syndrome primary peripheral neuropathies. Molecular
include miosis and mild ptosis. The light and genetics and clinical implications of CMT 1A
near responses of the pupil are normal. The and HNPP. JAMA 1993;270:2326-2330.
anisocoria is worse in the dark and less _____________________________
evident in bright light. A solution of 10% Question(s) 102: Anatomy
cocaine will dilate a normal pupil but will not
Discussion:
dilate the pupil in a patient with Horner's
The posterior tuberomammillary nucleus of
syndrome.
the hypothalamus provides diffuse
histaminergic innervation to the cerebral
Reference: cortex.
Brazis PW, Masdeu JC, Biller J. Localization
in clinical neurology. 4th ed. Philadelphia: Reference:
Lippincott, Williams & Wilkins, 2001.
Benarroch EE, Westmoreland BF, Daube
_____________________________ JR, et al. Medical neurosciences - an
Question(s) 100: approach to anatomy, pathology and
Pharmacology/Chemistry physiology by systems and levels. 4th ed.
Discussion: New York: Lippincott, Williams & Wilkins,
Zidovudine (AZT), used for treatment of HIV 1999.
infection, is a thymidine analog that inhibits _____________________________
reverse transcriptase and mitochondrila Question(s) 103: Physiology
DNS polymerase, leading to depletion of
Discussion:
mitochondrial DNA. Myalgia, weakness and
Tetrodotoxin blocks sodium channels.
elevated CK levels occur after 6-11 months
of exposure with doses of 800-1200 mg/day.
The diagnosis requires muscle biopsy, Reference:
which shows ragged red fibers. There is Kandel ER, Schwartz JH, Jessel TM.
recovery over several months after Principles of neural science. 4th ed. New
discontinuation of AZT. York: McGraw-Hill, 2000.
_____________________________
Reference: Question(s) 104: Pathology
Wald JJ. The effects of toxins on muscle. Discussion:
Neurology Clin 2000;18:695-717. Methanol intoxication causes necrosis of the
_____________________________ optic nerves and necrosis of the putamina
Question(s) 101: Clinical Adult bilaterally.
Discussion:
The primarily demyelinating form of Reference:
hereditary motor and sensory neuropathy is 1. Graham DI, Lantos PL. Greenfield's
designated as HMSN I, or Charcot-Marie- neuropathology. 7th ed. New York: Arnold
Tooth disease type 1A (CMT 1). In the Press, 2002.
majority of families with autosomal dominant 2. Fuller GN, Goodman JC. Practical review
CMT 1, the disease is associated with of neuropathology. Philadelphia: Lippincott,
duplication of a 1.5 megabase pair region on Williams & Wilkins, 2001.
chromosome 17; these families are _____________________________
classified as CMT 1A. Deletion of the same
chromosomal region has been
demonstrated in patients with hereditary
neuropathy with liability to pressure palsies
(HNPP), also called tomaculous neuropathy.
This is also inherited in an autosomal
dominant pattern.

17
 2003 AAN RITE Discussion & Reference Manual

Question(s) 105: Anatomy necrotizing myopathy is attributed to the

effects of the drug in inhibiting synthesis of
Discussion: mevalonic acid, a precursor of several
The medial temporal cortex projects to the essential metabolites, including coenzyme
mammillary bodies via the fornix. Q10 (ubiquinone). This toxic action is
potentiated by clofibrate, gemfibrozil,
Reference: nicotinic acid, and cyclosporine.
Brazis PW, Masdeu JC, Biller J. Localization
in clinical neurology. 4th ed. Philadelphia: Reference:
Lippincott, Williams & Wilkins, 2001. 1. Victor M, Sieb JP. Myopathies due to
_____________________________ drugs, toxins, and nutritional deficiencies. In:
Question(s) 106: Clinical Adult Engel AW, Franzini-Armstrong C, editors.
Discussion: Myology. 2nd ed. New York: McGraw-Hill,
Functional recovery from the effects of an 1994.
ischemic stroke is most significant during the 2. Karpati G, Hilton-Jones D, Griggs RC.
first few weeks following stroke. There is Disorders of voluntary muscle. 7th ed. New
some continued improvement between six York: Churchill Livingstone, 2001.
months and one year. After one year, the _____________________________
proportion of patients with deterioration in Question(s) 109: Anatomy
function increases and by three to five years
Discussion:
after stroke, more survivors experience
The group of long association fibers
increased disability than improvement,
interconnecting the superior and middle
possibly due to the effects of co-morbidity
frontal gyri with the posterior superior
and increased age.
temporal gyrus is the arcuate fasciculus. In
the dominant hemisphere, interruption of this
Reference: fasciculus results in conduction aphasia.
Dombovy M. Rehabilitation and the course
of recovery after stroke. In: Whisnant JP, Reference:
editor. Stroke: populations, cohorts, and
Parent A. Carpenter's human
clinical trials. Boston: Butterworth-
neuroanatomy. 9th ed. Baltimore: Williams &
Heinemann, 1993.
Wilkins, 1996.
_____________________________
_____________________________
Question(s) 107: Behavioral Question(s) 110: Clinical Adult
Discussion: Discussion:
Major depression occurs in over 40% of
Symptoms of Parkinson's disease usually
Huntington's disease patients and suicide
begin asymmetrically. Myoclonus is not a
occurs in close to 10% of patients. Suicide is
feature. A variety of autonomic symptoms
far less common in Alzheimer's disease,
may occur, but they are rarely disabling.
olivo-pontocerebellar degeneration, and
Vomiting is uncommon. Oculomotor
amyotrophic lateral sclerosis and is rarely
abnormalities may also occur, but usually
associated with right middle cerebral artery
not of the magnitude seen in progressive
infarction.
suranuclear palsy. Hallucinations are
uncommon except in association with
Reference: medications.
Moore DP. Textbook of clinical
neuropsychiatry. New York: Oxford Reference:
University Press, 2001.
Bradley WG, Daroff RB, Fenichel GM, et al,
_____________________________ editors. Neurology in clinical practice. 3rd
Question(s) 108: ed. New York: Butterworth-Heinemann,
Pharmacology/Chemistry 1999.
Discussion: _____________________________
Lovastatin (and other statins) induced

18
 2003 AAN RITE Discussion & Reference Manual

Question(s) 111: Pathology Reference:

Discussion: Engel AG, Franzini-Armstrong C. Myology.
New York: McGraw Hill, 1994.
The principal entities in the differential
diagnosis of a solitary tumor of the lumbar _____________________________
cistern are schwannoma, meningioma, Question(s) 114:
myxopapillary ependymoma, and Pharmacology/Chemistry
paraganglioma of the filum terminale. Discussion:
Solitary metastasis may also occur. The hypocretin/orexin neurons of the
Schwannomas arise from the nerve roots of posterior lateral hypothalamus play a critical
the cauda equina or from small peripheral role in preventing abrupt transitions between
nerve fascicles in the filum terminale. Two wakefulness and sleep. These neurons send
unique neoplasms arise from the filum excitatory projections to the cholinergic and
terminale: myxopapillary ependymoma and monoaminergic brainstem neurons involved
paraganglioma of the filum terminale. Both in arousal, and projections that inhibit the
tumors form well-circumscribed fusiform ventrolateral preoptic neurons involved in
masses. Myxopapillary ependymomas are sleep onset. Impaired activity of the
immunopositive for glial fibrillary acidic hypocretin/orexin neurons is responsible for
protein and S-100 protein while narcolepsy.
paragangliomas, as in this case, are strongly
positive for the neuronal markers
synaptophysin and chromogranin. Of the Reference:
other two choices listed, central Sutcliffe JG, de Lecea L. The hypocretins:
neurocytomas arise in the lateral and third setting the arousal threshold. Nat Rev
ventricles and dysembryoplastic Neurosci 2002;3:339-349.
neuroepithelial tumors are found in the _____________________________
cerebral cortex. Question(s) 115: Anatomy
Discussion:
Reference: The anterior choroidal artery supplies the
Fuller GN, Goodman JC. Practical review of anteromedial part of the head of the caudate
neuropathology. Philadelphia: Lippincott, nucleus. The internal carotid artery sends
Williams & Wilkins, 2001. branches directly to the genu of the internal
_____________________________ capsule. The anterior and medial parts of
Question(s) 112: Physiology the thalamus are supplied by posteromedial
(thalamoperforating) arteries. The
Discussion: hippocampal formation receives its blood
REM sleep is characterized by fluctuations supply from the anterior choroidal artery.
in blood pressure, muscle twitches,
dreaming, a low voltage EEG pattern and a
decrease in muscle tone. Susceptability to Reference:
apnea is increased during REM. Haines DE. Fundamental neuroscience. 2nd
ed. New York: W.B. Saunders, 2002.
_____________________________
Reference:
Kryger MH, Roth T, Dement WC. Principles
and practice of sleep medicine. 3rd ed.
Philadelphia: WB Saunders Company, 2000.
_____________________________
Question(s) 113: Clinical Adult
Discussion:
Polymyositis often presents as an acquired
progressive proximal weakness with
elevated creatine kinase levels and EMG
demonstrating fibrillations and small
polyphasic potentials.

19
 2003 AAN RITE Discussion & Reference Manual

Question(s) 116: Reference:

Pharmacology/Chemistry Harper CM, Thomas JE, Cascino TL, et al.
Discussion: Distinction between neoplastic and radiation
induced brachial plexopathy, with emphasis
HIV-1 infection is initiated by the interaction
on the role of EMG. Neurology 1989;39:502-
of virion envelope glycoproteins, such as
506.
gp120, with a least two cellular receptors,
the CD4 molecule and a chemokine _____________________________
receptor. Macrophage-tropic strains of HIV-1 Question(s) 119: Clinical Adult
replicate in macrophages and CD4+ T cells Discussion:
and use the CC chemokine receptor CCR5, In the presence of a positive HIV test the
whereas T cell-tropic HIV-1 strains most probable brain mass lesion would be
replicated primarily in CD4+ T cells and use either toxoplasmosis or B-cell non-Hodgkin's
the CXC chemokine receptor CXCR4. HIV-1 lymphoma. Kaposi's sarcoma rarely
enters the brain in association with infected metastasizes to brain.
macrophages soon after infection. HIV does
not replicate in neurons and infects
astrocytes and oligodendrocytes Reference:
nonproductively, but replicate actively in the MacArthur J. NeuroAIDS: diagnosis and
microglia, as well as infected macrophages management. Hosp Pract 1997;32:73-74.
that migrated to the brain. _____________________________
Question(s) 120: Clinical Adult
Reference: Discussion:
Power C, Gill MJ, Johnson RT. The Patients with Duchenne muscular dystrophy
neuropathogenesis of HIV infection: host- may develop tight heel cord muscles due to
virus interaction and the impact of therapy. paresis of dorsiflexors of the foot. These
Can J Neurol Sci 2002;29:19-32. children should be referred to physical
_____________________________ therapy for a daily muscle-stretching
Question(s) 117: Anatomy program and should attempt to maintain
their activity level.
Discussion:
To reach its area of distribution, the
posterior cerebral artery crosses the border Reference:
of the tentorium cerebelli from the Karpati G, Hilton-Jones D, and Griggs RC.
infratentorial compartment into the Disorders of voluntary muscle. 7th ed. New
supratentorial compartment. Increased York: Churchill Livingston, 2001.
intracranial pressure in the supratentorial _____________________________
compartment may compress the posterior Question(s) 121: Anatomy
cerebral artery against the tentorium
cerebelli leading to compromised blood flow Discussion:
to the visual cortex. Unilateral deafness could result from lesions
in the cochlea, cochlear branch of the eighth
nerve, or both the dorsal and ventral
Reference: cochlear nuclei.
Haines DE. Fundamental neuroscience. 2nd
ed. New York: W.B. Saunders, 2002.
Reference:
_____________________________
Parent A. Carpenter's human
Question(s) 118: Clinical Adult neuroanatomy. 9th ed. Baltimore: Williams &
Discussion: Wilkins, 1996.
Myokymia on EMG is very suggestive of _____________________________
radiation-induced plexopathy. Presence of
Horner's syndrome, a discrete mass on CT
scanning, lower trunk involvement, and the
presence of pain all favor neoplastic over
radiation-induced brachial plexopathy.

20
 2003 AAN RITE Discussion & Reference Manual

Question(s) 122: Behavioral Febiger, 2001.

_____________________________
Discussion:
Among the defining characteristics of post Question(s) 126: Anatomy
traumatic stress disorder are duration more Discussion:
than a month, the occurrence of flashbacks, The superior cervical ganglion provides the
reminiscences of the episode of severe noradrenergic innervation to the pineal
stress that the patient suffered, and gland. These inputs are important to entrain
persistent symptoms of increased arousal, melatonin secretion with the light-dark cycle.
including an exaggerated startle response.
Reference:
Reference: Parent A. Carpenter's human
American Psychiatric Association. neuroanatomy. 9th ed. Baltimore: Williams &
Diagnostic and statistical manual of mental Wilkins, 1996.
disorders (DSM-IV-R). 4th ed. Washington: _____________________________
American Psychiatric Association, 2000.
Question(s) 127: Pathology
_____________________________
Discussion:
Question(s) 123: Anatomy Intracranial epidural hematomas usually
Discussion: arise from a laceration of the meningeal
The sural nerve is a pure sensory nerve; artery secondary to a skull fracture. The
therefore, all of its axons arise from cell patient may be fully lucid for an extended
bodies located in the dorsal root ganglion. period of time following the injury while
The dorsal root ganglion that contributes the blood accumulates in the epidural space.
majority of axons to the sural nerve is S1.
Reference:
Reference: Graham DI, Lantos PL. Greenfield's
Midroni G, Bilbao JM. Biopsy diagnosis of neuropathology. 7th ed. New York: Arnold
peripheral neuropathy. Boston: Butterworth- Press, 2002.
Heinemann, 1995. _____________________________
_____________________________ Question(s) 128: Physiology
Question(s) 124: Anatomy Discussion:
Discussion: Focal polymorphic delta slowing can be
Dorsal midbrain tegmental lesions injure the seen with focal cerebral lesions such as a
red nucleus, the third nerve nucleus, and the supratentorial tumor, a cerebral infarction or
brachium conjunctivum, resulting in a cerebral abscess. Pseudotumor cerebri is
Claude syndrome. usually associated with mild generalized
slowing or a normal EEG. Lacunar
infarctions are not generally associated with
Reference:
prominent EEG findings.
Brazis PW, Masdeu JC, Biller J. Localization
in clinical neurology. 4th ed. Philadelphia:
Lippincott, Williams & Wilkins, 2001. Reference:
_____________________________ Niedermeyer E, Lopes da Silva F, editors.
Question(s) 125: Physiology Electroencephalography: basic principles,
clinical applications, and related fields. 4th
Discussion: ed. Philadelphia: Lippincott, Williams &
The region of the brain with the lowest Wilkins, 1998.
threshold to electrical stimulation is the
_____________________________
hippocampus.

Reference:
Wyllie E. Treatment of epilepsy: principles
and practice. 3rd ed. Philadelphia: Lea &

21
 2003 AAN RITE Discussion & Reference Manual

Question(s) 129: Question(s) 132: Physiology

Pharmacology/Chemistry Discussion:
Discussion: Vertex waves are first seen at 2-3 months of
The patient is suffering from restless legs age. The full term neonate has a
syndrome. Oxycodone, carbidopa/levodopa discontinuous rhythm in quiet sleep (trace
and pergolide are all effective treatments, alternant). Delta brushes are a pattern of
but dopamine agonists, such as pergolide, prematurity but may also be seen in a full-
do not produce the daytime rebound term infant. Rare temporal sharp waves may
restlessness that can occur with also been seen in the premature infant.
carbidopa/levodopa and do not have the Rhythmic anterior slow waves can be seen
addictive potential of oxycodone. during sleep in full-term infants.
Benzodiazepines can be effective, but
daytime drowsiness may be a problem for
elderly patients placed on diazepam. Reference:
Pergolide would be the drug of choice. Niedermeyer E, Lopes da Silva F, editors.
Electroencephalography: basic principles,
clinical applications, and related fields. 4th
Reference: ed. Philadelphia: Lippincott, Williams &
Jankovic J, Tolosa E, Scott-Conner CE, Wilkins, 1998.
editors. Parkinson's disease and movement _____________________________
disorders. 4rd ed. Baltimore: Williams & Question(s) 133: Pathology
Wilkins, 2002.
_____________________________ Discussion:
Fatal familial insomnia is a human prion
Question(s) 130: Anatomy disease. The cause of ALS and corticobasal
Discussion: ganglionic degeneration remains obscure,
The paraventricular nucleus of the but transmission studies have failed to
hypothalamus provides a major excitatory support prion etiology. Familial
input to the preganglionic sympathetic dentatopallidoluyseal degeneration is a
neurons and plays a critical role in genetic disorder characterized by
integrated responses to stress. trinucleotide repeat amplification.
Striatonigral degeneration is a component of
multiple system atrophy.
Reference:
Parent A. Carpenter's human
neuroanatomy. 9th ed. Baltimore: Williams & Reference:
Wilkins, 1996. Graham DI, Lantos PL. Greenfield's
_____________________________ neuropathology. 7th ed. New York: Arnold
Question(s) 131: Clinical Adult Press, 2002.
_____________________________
Discussion:
The most important initial study to obtain in Question(s) 134: Clinical Adult
a patient over the age of 55 suspected of Discussion:
having temporal arthritis is an erythrocyte Organophosphate poisoning may occur in
sedimentation rate. patients exposed to pesticides.
Intramuscular atropine administration is
standard initial therapy.
Reference:
Bradley WG, Daroff RB, Fenichel GM, et al,
editors. Neurology in clinical practice. 3rd Reference:
ed. New York: Butterworth-Heinemann, Bradley WG, Daroff RB, Fenichel GM, et al,
1999. editors. Neurology in clinical practice. 3rd
_____________________________ ed. New York: Butterworth-Heinemann,
1999.
_____________________________

22
 2003 AAN RITE Discussion & Reference Manual

Question(s) 135: Anatomy Question(s) 138: Behavioral

Discussion: Discussion:
Normal median somatosensory evoked Lithium, haloperidol, valproate, and
potentials would be seen with a lesion of the electroconvulsive therapy are effective for
lateral cuneate nucleus. The fasciculus acute mania. Selegeline is not known to
cuneatus contains long ascending branches benefit acute mania and may exacerbate the
of the upper six thoracic and all cervical problem because of its monoamine oxidase
dorsal roots. Axons from the second order inhibitory effects.
neurons in the nucleus cuneatus cross the
midline as the internal arcuate fibers and Reference:
ascend in the medial lemniscus to the
Goldman HH. General psychiatry. Norwalk:
ventral posterolateral nucleus. From here,
Appleton and Lange, 1995.
third order neurons send axons through
_____________________________
thalami-cortical radiations to the sensory
cortex. Question(s) 139: Physiology
Discussion:
Reference: Calcium binding in the membrane facilitates
acetylcholine release following an action
1. Benarroch EE, Westmoreland BF, Daube
potential.
JR, et al. Medical neurosciences - an
approach to anatomy, pathology and
physiology by systems and levels. 4th ed. Reference:
New York: Lippincott, Williams & Wilkins, Kimura J. Electrodiagnosis in disease of
1999. nerve and muscle. 3rd ed. New York: Oxford
2. Haines DE. Fundamental neuroscience. University Press, 2001.
2nd ed. New York: W.B. Saunders, 2002. _____________________________
_____________________________ Question(s) 140:
Question(s) 136: Behavioral Pharmacology/Chemistry
Discussion: Discussion:
Bupropion has had a low incidence of The structural basis of the blood brain
erectile dysfunction associated with its use. barrier (BBB) is the presence of tight
All of the selective serotonin reuptake junctions (zonula occludens) between
inhibitors (SSRIs) have been reported to capillary endothelial cells. Tight junctions
have erectile dysfunction as a side effect. consists of macromolecular complexes,
Amitriptyline and venlafaxine also cause including the transmembrane proteins
erectile dysfunction. occludin and claudin, and the associated
zonula occludens (ZO) proteins and cingulin.
Reference:
Arana GW, Rosenbaum JF. Handbook of Reference:
psychiatric drug therapy. 4th ed. Huber JD, Egleton R, Davis TP. Molecular
Philadelphia: Lippincott, Williams & Wilkins, physiology of tight junctions in the blood-
2000. brain barrier. Trends Neurosci 2001;24:719-
_____________________________ 726.
Question(s) 137: Physiology _____________________________
Discussion:
The sense of vibration is dependent on
Pacinian corpuscles.

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

23
 2003 AAN RITE Discussion & Reference Manual

Question(s) 141: Question(s) 143: Anatomy

Pharmacology/Chemistry Discussion:
Discussion: The preganglionic sympathetic neurons are
Baroreflex failure may occur with peripheral located in the intermediolateral cell column
or central lesions that interrupt the in all thoracic and the upper two lumbar
baroreceptor reflex circuit, which in normal spinal segments. Thus, they are formed
conditions serves as a buffer against from the neural tube.
marked oscillations of blood pressure.
Causes of baroreflex failure include bilateral Reference:
carotid sinus denervation, as occurs
1. Benarroch EE, Westmoreland BF, Daube
following surgery or in disorders such as the
JR, et al. Medical neurosciences - an
Guillain-Barré syndrome, or with lesions that
approach to anatomy, pathology and
affect the nucleus tractus solitarius
physiology by systems and levels. 4th ed.
bilaterally, such as syringobulbia. The
New York: Lippincott, Williams & Wilkins,
syndrome is characterized by episodes of
1999.
fluctuating hypertension, resembling a
2. Haines DE. Fundamental neuroscience.
pheochromocytoma. Clonidine, a central
2nd ed. New York: W.B. Saunders, 2002.
alpha-2 receptor agonist that inhibits the
brainstem sympathoexcitatory drive, is _____________________________
effective in controlling these episodes. Question(s) 144: Physiology
Discussion:
Reference: Decerebrate rigidity is associated with tonic
Robertson D, Hollister AS, Biaggioni I, et al. neuronal activity in the vestibulospinal and
The diagnosis and treatment of baroreflex pontine reticulospinal nuclei.
failure. NEJM 1993;329:1449-1455.
_____________________________ Reference:
Question(s) 142: Kandel ER, Schwartz JH, Jessel TM.
Pharmacology/Chemistry Principles of neural science. 4th ed. New
York: McGraw-Hill, 2000.
Discussion:
Midodrine is a prodrug that is transformed in _____________________________
the liver to a potent agonist of alpha Question(s) 145: Physiology
receptors in arteries and veins. Given its Discussion:
predictable absorption and peak effect, it is Permanent diabetes insipidus is produced
the sympathomimetic of choice for treatment by destruction of the supraoptic and
of neurogenic orthostatic hypotension. One paraventricular nuclei, which release the
common side effect is scalp pruritus, a antidiuretic hormone vasopressin.
manifestation of alpha adrenergic mediated
piloerection. Its most potentially serious side
effect is supine hypertension. Midodrine Reference:
should not be administered late in the Kandel ER, Schwartz JH, Jessel TM.
evening and patients taking the drug should Principles of neural science. 4th ed. New
avoid lying flat. York: McGraw-Hill, 2000.
_____________________________
Reference:
1. Jankovik J, Gilden JL, Hiner BC, et al.
Neurogenic orthostatic hypotension: a
double-blind, placebo-controlled study with
midodrine. Am J Med 1993;95:38-48.
2. Bradley WG, Daroff RB, Fenichel GM, et
al, editors. Neurology in clinical practice. 3rd
ed. New York: Butterworth-Heinemann,
1999.
_____________________________

24
 2003 AAN RITE Discussion & Reference Manual

Question(s) 146: Pathology Reference:

Discussion: Benarroch EE, Westmoreland BF, Daube
JR, et al. Medical neurosciences - an
Carbon monoxide poisoning may produce
approach to anatomy, pathology and
bilateral lesions of the globus pallidus.
physiology by systems and levels. 4th ed.
Methanol toxicity may produce lesions of the
New York: Lippincott, Williams & Wilkins,
putamen. Bilateral petechial hemorrhages of
1999.
the mammillary bodies are seen in acute
Wernicke encephalopathy. _____________________________
Question(s) 150: Clinical Adult
Reference: Discussion:
Graham DI, Lantos PL. Greenfield's Lhermitte's sign (an abnormal sensation,
neuropathology. 7th ed. New York: Arnold usually described as "like an electric shock",
Press, 2002. precipitated by neck flexion and traveling
rapidly down the spine) has been described
_____________________________
in a variety of conditions, most of which are
Question(s) 147: Anatomy associated with demyelination in the
Discussion: posterior columns of the cervical spinal cord.
With a right unilateral lesion of the medial Common associated conditions include
longitudinal fasciculus, the patient can multiple sclerosis, radiation myelopathy and
abduct the left eye on attempted gaze to the subacute combined degeneration of the cord
left but the right eye cannot be adducted. (vitamin B12 deficiency).
Nystagmus occurs in the left (abducting)
eye.
Reference:
1. Kanchandani R, Howe JG. Lhermitte's
Reference: sign in multiple sclerosis: a clinical survey
1. Brazis PW, Masdeu JC, Biller J. and review of the literature. J Neurol
Localization in clinical neurology. 4th ed. Neurosurg Psych 1982;45:308-312.
Philadelphia: Lippincott, Williams & Wilkins, 2. Gautier-Smith PC. Lhermitte's sign in
2001. subacute combined degeneration of the
2. Haines DE. Fundamental neuroscience. cord. J Neurol Neurosurg Psych
2nd ed. New York: W.B. Saunders, 2002. 1973;36:861-863.
_____________________________ 3. Bradley WG, Daroff RB, Fenichel GM, et
Question(s) 148: Physiology al, editors. Neurology in clinical practice. 3rd
ed. New York: Butterworth-Heinemann,
Discussion: 1999.
Interneurons in the spinal cord are _____________________________
necessary for flexion, inverse myotatic and
crossed extension reflexes and for Question(s) 151: Anatomy
reciprocal innervation. No interneurons are Discussion:
required for the tendon stretch reflex which The neurohypophysis contains fenestrated
is monosynaptic. capillary endothelium with tight junctions. It
is a circumventricular organ that has no
blood-brain barrier.
Reference:
Kandel ER, Schwartz JH, Jessel TM.
Principles of neural science. 4th ed. New Reference:
York: McGraw-Hill, 2000. 1. Benarroch EE, Westmoreland BF, Daube
_____________________________ JR, et al. Medical neurosciences - an
Question(s) 149: Anatomy approach to anatomy, pathology and
physiology by systems and levels. 4th ed.
Discussion: New York: Lippincott, Williams & Wilkins,
The largest cerebellar efferent bundle, the 1999.
superior cerebellar peduncle, is formed by 2. Haines DE. Fundamental neuroscience.
fibers from the dentate, emboliform, and 2nd ed. New York: W.B. Saunders, 2002.
globose nuclei. _____________________________

25
 2003 AAN RITE Discussion & Reference Manual

Question(s) 152: Physiology Question(s) 154: Anatomy

Discussion: Discussion:
Periodic lateralized epileptiform discharges The globus pallidus projects to the ventral
(PLEDs) are seen after an acute or anterior and ventral lateral (pars oralis)
subacute cerebral insult such as a stroke nuclei of the thalamus.
and are often associated with focal seizures.
Reference:
Reference: Parent A. Carpenter's human
1. Ebersole JS, Pedley TA. Current practice neuroanatomy. 9th ed. Baltimore: Williams &
of clinical electroencephalography. 3nd ed. Wilkins, 1996.
New York: Lippincott Williams Wilkins, 2003. _____________________________
2. Niedermeyer E, Lopes da Silva F. Question(s) 155: Anatomy
Electroencephalography. 4th ed. Baltimore:
Lippincott, Williams & Wilkins, 1998. Discussion:
The amygdalofugal fibers pass through the
_____________________________
inferior thalamic peduncle to the
Question(s) 153: magnocellular division of the mediodorsal
Pharmacology/Chemistry nucleus.
Discussion:
Horner's syndrome results from impaired
Reference:
sympathetic innervation of the eye. Lesions
may involve the central (first-order) neurons Benarroch EE, Westmoreland BF, Daube
of the hypothalamus, brainstem and cord, JR, et al. Medical neurosciences - an
the preganglionic (second-order) neurons of approach to anatomy, pathology and
the C8-T2 roots to the level of the superior physiology by systems and levels. 4th ed.
cervical ganglion or the postganglionic New York: Lippincott, Williams & Wilkins,
(third-order) neurons as they follow the 1999.
internal and external carotid arteries to _____________________________
innervate the orbit and vessels of the face Question(s) 156: Clinical Adult
respectively. All forms of Horner's syndrome Discussion:
show failure to dilate on exposure to Rest tremor is more common in idiopathic
cocaine, which is a norepinephrine reuptake Parkinson's disease (PD) than in diffuse
inhibitor. Preganglionic lesions show normal Lewy body disease (DLBD), but can occur in
or exaggerated response to either. Other parkinsonian features occur
hydroxyamphetamine, which releases with equal frequency in the two conditions.
norepinephrine from nerve terminals. The Most, but not all, patients with DLBD initially
response is absent or greatly attenuated in respond to dopaminergic agents, whereas
postganglionic lesions, where the nerve almost all patients with PD respond.
terminals are destroyed. Cognitive or psychiatric abnormalities are
less common in PD than in DLBD, which
Reference: typically presents with such symptoms.
Miller NR, Newman NJ. Walsh and Hoyt's
Clinical neuro-ophthalmology. The Reference:
essentials. 5th ed. Philadelphia: Lippincott, Louis ED, et al. Parkinsonian features of
Williams and Wilkins, 1999. eight pathologically diagnosed cases of
_____________________________ diffuse Lewy body disease. Movement
Disorders 1995.
_____________________________

26
 2003 AAN RITE Discussion & Reference Manual

Question(s) 157: Anatomy Question(s) 159: Clinical Adult

Discussion: Discussion:
The suprachiasmatic nucleus receives direct Extracranial vertebral artery dissections
bilateral projections from the retina and have most often been recognized in patients
functions as a biologic clock. who had either trauma or chiropractic
manipulation. Neurologic symptoms may be
delayed by hours, days or weeks. Transient
Reference: ischemic attacks (TIA's) most often include
1. Parent A. Carpenter's human dizziness, diplopia, veering, staggering, and
neuroanatomy. 9th ed. Baltimore: Williams & dysarthria. The diagnosis of arterial
Wilkins, 1996. dissection has traditionally been made using
2. Haines DE. Fundamental neuroscience. standard catheter angiography, although
2nd ed. New York: W.B. Saunders, 2002. MRI/MRA is very sensitive.
_____________________________
Question(s) 158: Clinical Adult Reference:
Discussion: 1. Caplan LR. Caplan's stroke, a clinical
Orthostatic tremor or "shaky leg" syndrome approach. 3rd ed. Boston: Butterworth-
is a disorder of middle-aged or elderly Heinemann, 2000.
people and is characterized by feelings of 2. Bradley WG, Daroff RB, Fenichel GM, et
unsteadiness in the legs and a fear of falling al, editors. Neurology in clinical practice. 3rd
when standing. Other associated symptoms ed. New York: Butterworth-Heinemann,
include difficulty in initiating walking and leg 1999.
discomfort when standing. The symptoms _____________________________
are attenuated by walking, abolished by
sitting, and due to a high frequency tremor in Question(s) 160: Anatomy
the weight-bearing muscles. Discussion:
The cephalad portion of the neural tube
initially forms three vesicles
Reference: (prosencephalon, mesencephalon, and
Brazis PW, Masdeu JC, Biller J. Localization rhombencephalon) that subsequently
in clinical neurology. 4th ed. Philadelphia: segment further to form the telencephalon,
Lippincott, Williams & Wilkins, 2001. diencephalon, mesencephalon,
_____________________________ metencephalon, and myelencephalon. The
pons arises from the metencephalon.

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.
_____________________________

27
 2003 AAN RITE Discussion & Reference Manual

Question(s) 161: Pathology cognitive neurology. 2nd ed. New York:

Oxford University Press, 2000.
Discussion: _____________________________
Prion diseases have long incubation times
and evoke no host immune response. Question(s) 164: Anatomy
Immunocompromised individuals are not Discussion:
preferentially affected by prion diseases. The anterior lobe of the cerebellum projects
The CSF does not show pleocytosis (as it to the nucleus interpositus (globose and
does in most other infections) and neither emboliform nuclei) and fastigial nucleus,
inflammation or microglial nodules are seen regulates ipsilateral extensor muscle tone,
in the brain. Spongiform change, intense receives some input form the pontine nuclei
gliosis, and neuronal loss unassociated with via pontocerebellar tracts, and has its output
mononuclear cell inflammation is the from the cerebellum via the superior
characteristic histologic pattern for cerebellar peduncle. It has direct (cortical)
Creutzfeldt-Jakob disease. connections and connections through deep
nuclei to the vestibular system.
Reference:
Graham DI, Lantos PL. Greenfield's Reference:
neuropathology. 7th ed. New York: Arnold 1. Benarroch EE, Westmoreland BF, Daube
Press, 2002. JR, et al. Medical neurosciences - an
_____________________________ approach to anatomy, pathology and
Question(s) 162: Anatomy physiology by systems and levels. 4th ed.
New York: Lippincott, Williams & Wilkins,
Discussion: 1999.
On the side of a spinal cord hemisection
2. Haines DE. Fundamental neuroscience.
there is an upper motor neuron syndrome,
2nd ed. New York: W.B. Saunders, 2002.
greatly impaired discriminatory tactile sense,
_____________________________
loss of kinesthetic sense, and reduced
muscle tone. Contralateral to the lesion Question(s) 165: Physiology
there is loss of pain and temperature due to Discussion:
interruption of the ascending spinothalamic Activity in the 8 Hz range can be seen in
tracts. comatose patients with pontine infarctions,
i.e., an alpha coma pattern.
Reference:
Brazis PW, Masdeu JC, Biller J. Localization Reference:
in clinical neurology. 4th ed. Philadelphia: Niedermeyer E, Lopes da Silva F, editors.
Lippincott, Williams & Wilkins, 2001. Electroencephalography: basic principles,
_____________________________ clinical applications, and related fields. 4th
Question(s) 163: Behavioral ed. Philadelphia: Lippincott, Williams &
Wilkins, 1998.
Discussion:
_____________________________
Misreaching under visual guidance (optic
ataxia) and failure to scan and integrate an
entire visual scene or
picture(simultanagnosia) is part of Balint's
syndrome. When these symptoms occur
together the occipito-parietal region is the
most common area affected. The third
clinical sign seen in Balint's syndrome is
ocular apraxia.

Reference:
Damasio A, Tranel D, Rizzo M. Disorders of
complex visual processing. In: Mesulam
MM, editor. Principles of behavioral and

28
 2003 AAN RITE Discussion & Reference Manual

Question(s) 166: Anatomy they are found. Hamartias have no growth

potential - lesions in this category include
Discussion: cortical tubers, retinal flat lesions and renal
The dorsal (posterior) spinocerebellar tract cysts; hamartomas have the potential to
is an uncrossed ascending tract that arises grow. Examples of the latter include facial
from the large neurons of Clarke's column angiofibromas, subependymal giant cell
(dorsal nucleus), which extends from L3 to astrocytomas and renal angiomyolipomas.
C8. Clarke's nucleus receives afferent fibers Mental retardation occurs as a result of
from all parts of the body except the head poorly controlled seizures, and intellect is
and neck but is functionally related primarily usually normal in the absence of seizures.
to the hind limb and caudal parts of the Hypomelanotic macules are a frequent skin
body. finding, but are not pathognomonic of the
disease. Some patients, particularly women,
Reference: develop pulmonary involvement that may be
Haines DE. Fundamental neuroscience. 2nd manifest as recurrent pneumothoraces.
ed. New York: W.B. Saunders, 2002.
_____________________________ Reference:
Question(s) 167: Physiology Gomez MR, editor. Tuberous sclerosis
Discussion: complex. 3rd ed. New York: Oxford
Development of a steady force is University Press, 1999.
characteristic of a type I muscle fiber. _____________________________
Question(s) 170: Anatomy
Reference: Discussion:
Kimura J. Electrodiagnosis in disease of In looking downward to the left, when the
nerve and muscle. 3rd ed. New York: Oxford eye is turned outward, the depressor is the
University Press, 2001. left inferior rectus. When eye is turned
_____________________________ inward, the depressor is the right superior
oblique.
Question(s) 168: Anatomy
Discussion:
Posterior cord lesions would affect muscles Reference:
innervated by subscapular, thoracodorsal, 1. Brazis PW, Masdeu JC, Biller J.
radial and axillary nerves. The flexor carpi Localization in clinical neurology. 4th ed.
ulnaris is innervated by the ulnar nerve of Philadelphia: Lippincott, Williams & Wilkins,
the medial cord. 2001.
2. Haines DE. Fundamental neuroscience.
2nd ed. New York: W.B. Saunders, 2002.
Reference: _____________________________
Brazis PW, Masdeu JC, Biller J. Localization
in clinical neurology. 4th ed. Philadelphia:
Lippincott, Williams & Wilkins, 2001.
_____________________________
Question(s) 169: Clinical Pediatrics
Discussion:
Tuberous sclerosis complex is an autosomal
dominant syndrome resulting from mutations
in one of two tumor suppressor genes, TSC
1 or TSC 2. Characteristic lesions include
both hamartias and hamartomas. Both of
these are circumscribed groups of cells that
are misaligned or otherwise disrupted in
their architectural relationships, and often
have dysplastic features. The cell types are,
however, appropriate for the tissue in which

29
 2003 AAN RITE Discussion & Reference Manual

Question(s) 171: Pathology Reference:

Discussion: Olanow CW, Watts RL, Koller WC. An
algorithm (decision tree) for the
The two most common adult tumors in the
management of Parkinson's disease (2001).
cerebellopontine angle region are vestibular
Neurology 2001;56(Suppl 5):S1-S88.
schwannoma and meningioma. In this case,
a meningioma was present. Most _____________________________
meningiomas are strongly positive for Question(s) 173: Anatomy
epithelial membrane antigen (EMA). In Discussion:
contrast, schwannomas are negative for The cortical projections of the anterior
EMA but strongly positive for S-100 protein. nucleus of the thalamus are to the cingulate
Ultrastructurally, meningiomas show gyrus. This is part of the classical Papez
elongated intertwined cell processes joined circuit that formed the basis of the concept
by large numbers of desmosomes. of the limbic system.
Schwannomas also show elongated cell
processes by electron microcopy, but do not
have the prominent intercellular junctions Reference:
seen in meningiomas; rather, a layer of Haines DE. Fundamental neuroscience. 2nd
basal lamina covers the cell processes of ed. New York: W.B. Saunders, 2002.
schwannoma. Another distinctive _____________________________
ultrastructural feature seen in many Question(s) 174:
schwannomas is the presence of Luse Pharmacology/Chemistry
bodies ("long-spacing collagen"), which are
fusiform cross-striated structures composed Discussion:
of collagen that has an increased banding The medium spiny neurons of the striatum
periodicity. Antoni type A tissue (densely contain, in addition to D1 and D2 dopamine
cellular tumor) and Antoni type B tissue receptors, several other receptors that can
(loose, hypocellular tumor) are characteristic potentiate or antagonize the effects of
of schwannoma. Palisades of nuclei dopamine. The A2 adenosine receptors
separated by fibrillar anuclear zones are antagonize the effects of activation of D2
referred to as Verocay bodies and are receptors. This may have therapeutic
typically seen in Antoni type A areas of implications, as adenosine receptors are
schwannomas. blocked by caffeine.

Reference: Reference:
Burger PC, Scheithauer BW, Vogel FS. Richardson PJ, Kase H, Jenner PG.
Surgical pathology of the nervous system Adenosine A2a receptor antagonists as new
and its coverings. 4th ed. New York: agents for the treatment of Parkinson's
Churchill Livingston, 2002. disease. Trends Pharmacol Sci
_____________________________ 1997;18:338-344.
_____________________________
Question(s) 172:
Pharmacology/Chemistry
Discussion:
Quetiapine is an atypical neuroleptic that,
like clozapine, has 5-HT2 receptor blocking
activity lower D2 blocking potency than the
typical neuroleptic such as haloperidol or
chlorpromazine. Therefore, quetiapine and
clozapine are the neuroleptic of choice for
management of hallucinations in patients
with Parkinson's disease. Risperidone,
another atypical neuroleptic, has higher D2
receptor blocking activity and thus higher
risk of extrapyramidal side effects.

30
 2003 AAN RITE Discussion & Reference Manual

Question(s) 175: Physiology Question(s) 178: Pathology

Discussion: Discussion:
The most common presentation of nemaline Synaptophysin is the most specific and
myopathy is congenital hypotonia. Affected useful immunohistochemical marker for
children are usually quite bright but exhibit a assessing neuronal differentiation; other
characteristic open-mouth appearance. useful neuronal markers include
Extra-ocular muscles are not affected and neurofilament proteins, chromogranin, and
muscle weakness is static throughout life. Neu-N. S-100 protein is positive in neural
crest derivatives, but is not specific for
neurons because Schwann cells and CNS
Reference: glial cells (astrocytes, oligodendrocytes and
Griggs RC, Mendell JR, Miller RG. ependymal cells) are also strongly
Congenital myopathies. In: Griggs RC, Miller immunoreactive. Similarly, despite the
RG, editors. Evaluation and treatment of optimistic name, neuron-specific enolase
myopathies. Philadelphia: FA Davis Co., has a wide expression distribution beyond
1995. the nervous system and glial tumors such as
_____________________________ glioblastoma are often immunopositive. Neu-
Question(s) 176: Clinical Adult N is a more recently introduced neuronal
Discussion: marker with nuclear and cytoplasmic
Optic neuritis is an inflammatory or localization; its potential usefulness is still
autoimmune disease process affecting the under evaluation. Vimentin is a cytoskeletal
optic nerve causing relatively acute impaired intermediate filament protein and cells of
vision, progressing over hours to days. It is many lineages exhibit immunoreactivity for
more common in women and affects this protein.
patients who are 20 to 50 years of age. The
optic disc is normal in approximately two- Reference:
thirds of patients and swollen in one-third. Fuller GN, Goodman JC. Practical review of
Pain in the eye, often exacerbated by neuropathology. Philadelphia: Lippincott,
movement, occurs in greater than 90 Williams & Wilkins, 2001.
percent of patients. _____________________________
Question(s) 179: Behavioral
Reference: Discussion:
Brazis PW, Masdeu JC, Biller J. Localization Wernicke's aphasia is characterized by
in clinical neurology. 4th ed. Philadelphia: impairments in comprehension, repetition,
Lippincott, Williams & Wilkins, 2001. reading, writing, and naming, with fluent
_____________________________ paraphasic speech.
Question(s) 177: Behavioral
Discussion: Reference:
Over half of epileptics have one or more Benson DF. Aphasia, alexia, and agraphia.
episodes of significant depression during the New York: Churchill Livingston, 1979.
course of the disorder and the suicide rate is _____________________________
considerably greater than in the general
population.

Reference:
Mendez MF, Cummings JL, Benson DF.
Depression in epilepsy. Archives of
Neurology 1996;43:766-770.
_____________________________

31
 2003 AAN RITE Discussion & Reference Manual

Question(s) 180: Reference:

Pharmacology/Chemistry Benarroch EE, Westmoreland BF, Daube
Discussion: JR, et al. Medical neurosciences - an
approach to anatomy, pathology and
Tick paralysis usually occurs in young
physiology by systems and levels. 4th ed.
individuals. The paralysis is reversible upon
New York: Lippincott, Williams & Wilkins,
removal of the tick. It is thought to result
1999.
from a presynaptic neuromuscular block.
This results from an impairment of _____________________________
excitation-secretion coupling due to reduced Question(s) 184: Behavioral
calcium availability. Discussion:
Dopamine agonists such as bromocriptine
Reference: have been used to treat the behavioral traits
1. Cooper BJ, Spence I. Temperature- associated with medial frontal syndrome.
dependent inhibition of evoked acetyl This syndrome may be seen after ischemia
choline release in tick paralysis. Nature in the distribution of the anterior cerebral
1976;263:693-695. artery.
2. Bradley WG, Daroff RB, Fenichel GM, et
al, editors. Neurology in clinical practice. 3rd Reference:
ed. New York: Butterworth-Heinemann, Muller U, von Cramon DY. The therapeutic
1999. potential of bromocriptine in
_____________________________ neuropsychological rehabilitation of patients
Question(s) 181: Behavioral with acquired brain damage. Prog
Neuropsypharm Biol Psych 1994;18:1103-
Discussion:
20.
Topographagnosia, usually combined with
_____________________________
some degree of unilateral neglect, most
often follows right parietal damage. Question(s) 185: Behavioral
Discussion:
Reference: Alien hand phenomenon is one of the
associated clinical features of corticobasal
Grusser O-J, Landis T. Visual agnosias and
ganglionic degeneration.
other disturbances of visual perception and
cognition. London: MacMillan Press, 1991.
_____________________________ Reference:
Question(s) 182: Clinical Adult Riley DE, Lang AE, Lewis A, et al.
Corticobasal ganglionic degeneration.
Discussion:
Neurology 1990;40:1203-1212.
Cataplexy, a loss of muscle tone following
_____________________________
emotional stimulation, occurs in about 70%
of patients with narcolepsy. It is not Question(s) 186: Behavioral
associated with other conditions that cause Discussion:
excessive daytime somnolence. Flumazenil may be used to reverse the
sedation associated with benzodiazepines.
Reference: This patient was comatose and ventilator-
dependent prior to flumazenil and became
Kryger MH, Roth T, Dement WC. Principles
alert and "weanable" after treatment.
and practice of sleep medicine. 3rd ed.
Philadelphia: WB Saunders Company, 2000.
_____________________________ Reference:
Question(s) 183: Physiology Kaplan HI, Sadock BJ. Pocket handbook of
psychiatric drug treatment. 2nd ed.
Discussion:
Baltimore: Williams and Wilkins, 1996.
During muscle contraction, the filaments
_____________________________
slide over one another and the Z bands
move closer together.

32
 2003 AAN RITE Discussion & Reference Manual

Question(s) 187: Behavioral Reference:

Discussion: Graham DI, Lantos PL. Greenfield's
neuropathology. 7th ed. New York: Arnold
Alexia without agraphia, also known as pure
Press, 2002.
word blindness, is most often produced by
left posterior cerebral artery occlusion that _____________________________
can cause a right homonymous Question(s) 191:
hemianopsia. Pharmacology/Chemistry
Discussion:
Reference: Glucose transporter type 1 deficiency may
Mesulam MM. Principles of behavioral and present with intractable seizures associated
cognitive neurology. 2nd ed. New York: with low cerebrospinal fluid (CSF) glucose
Oxford University Press, 2000. and low or low-normal CSF lactate. Patients
respond to an alternative fuel source in the
_____________________________
form of ketone bodies, as supplied by the
Question(s) 188: Physiology ketogenic diet. The CSF profile may be
Discussion: differentiated from that seen in mitochondrial
Preganglionic autonomic nerve fibers cytopathies, where low glucose is typically
conduct at a velocity of approximately 3-15 accompanied by high lactate. Glycine
meters/second. encephalopathy can present with intractable
seizures from birth and is associated with
normal CSF glucose and lactate, and with a
Reference: high CSF glycine (and elevated CSF:
Kandel ER, Schwartz JH, Jessel TM. plasma glycine ratio). Glycine
Principles of neural science. 4th ed. New encephalopathy does not respond to the
York: McGraw-Hill, 2000. ketogenic diet.
_____________________________
Question(s) 189: Behavioral Reference:
Discussion: diMauro S, De Vivo DC. Diseases of
Patients with frontotemporal dementia have carbohydrate, fatty acid and mitochondrial
been shown to manifest a variety of metabolism. In: Siegel GJ, Agranoff BW,
behavioral changes including hoarding of Albers RW, et al, editors. Basic
items and nascent musical and/or artistic neurochemistry. 6th ed. Philadelphia:
expression. This combination of behaviors is Lippincott-Raven, 1999.
usually not seen in other degenerative _____________________________
dementias.
Question(s) 192: Physiology
Discussion:
Reference: An asymmetric slowing of conduction
Miller BL, Cummings JL, Boone K, et al. velocities of pattern reversal visual evoked
Emergence of artistic talent in responses with a unilateral delay of 30 msec
frontotemporal dementia. Neurology is most likely to be associated with optic
1998;51:978-981. neuritis.
_____________________________
Question(s) 190: Pathology Reference:
Discussion: Nuwer MR. Fundamentals of evoked
Bone fractures are associated with release potentials and common clinical applications
of fatty bone marrow into the systemic today. Electroencephalogr Clin Neurophysiol
circulation. Pulmonary circulation may be 1998;106:142-148.
compromised, and emboli may travel to the _____________________________
brain leading to ball hemorrhages around
small vessels. The symptoms described in
this patient also fit best with fat embolism,
rather than diffuse axonal injury.

33
 2003 AAN RITE Discussion & Reference Manual

Question(s) 193: Physiology Reference:

Discussion: Kryger MH, Roth T, Dement WC. Principles
and practice of sleep medicine. 3rd ed.
A highly epileptogenic EEG pattern that has
Philadelphia: WB Saunders Company, 2000.
a high incidence of associated seizures is
the 3 Hz spike-and-wave. Benign EEG _____________________________
patterns that are unassociated with seizures Question(s) 197:
include 6 Hz spike-and-wave, small sharp Pharmacology/Chemistry
spikes and wicket spikes. Discussion:
Pyridoxine-dependent seizures are believed
Reference: to result from diminished activity of glutamic
Niedermeyer E, Lopes da Silva F, editors. acid decarboxylase (GAD), which is
Electroencephalography: basic principles, responsive to pharmacologic doses of its
clinical applications, and related fields. 4th cofactor, pyridoxine (vitamin B6). Deficient
ed. Philadelphia: Lippincott, Williams & action of GAD would be expected to
Wilkins, 1998. produce elevated levels of the excitatory
neurotransmitter glutamic acid, with
_____________________________
corresponding low levels of gamma
Question(s) 194: Clinical Adult aminobutyric acid, the major inhibitory
Discussion: neurotransmitter in the brain. Such findings
Nitrous oxide abuse may result in a clinical have been reported in patients with
syndrome of myeloneuropathy pyridoxine-dependent seizures, both prior to
indistinguishable from that of vitamin B12 and when off therapy. The cerebrospinal
deficiency. The mechanism appears to be fluid glucose concentration is normal in this
interference with the vitamin B12-dependent condition.
conversion of homocysteine to methionine.
Reference:
Reference: Gospe SM. Current perspectives on
Bradley WG, Daroff RB, Fenichel GM, et al, pyridoxine-dependent seizures. J Pediatr
editors. Neurology in clinical practice. 3rd 1998;132:919-923.
ed. New York: Butterworth-Heinemann, _____________________________
1999. Question(s) 198: Physiology
_____________________________
Discussion:
Question(s) 195: Physiology Increased fiber density on single fiber EMG
Discussion: occurs when there has been denervation
Nerve conduction velocities are about half and reinnervation, resulting in enlargement
the adult value in term infants and slower in of the motor unit and a greater likelihood
premature infants. They reach adult values that two or more muscle fibers innervated by
by age two. a motor unit will be within the recording
radius of the single fiber EMG electrode.
The correct answer is ALS.
Reference:
Kimura J. Electrodiagnosis in disease of
nerve and muscle. 3rd ed. New York: Oxford Reference:
University Press, 2001. Kimura J. Electrodiagnosis in disease of
_____________________________ nerve and muscle. 3rd ed. New York: Oxford
University Press, 2001.
Question(s) 196: Physiology
_____________________________
Discussion:
In healthy young adults, REM sleep is
approximately 20-25% of total sleep: stage 1
is 5%, stage 2 is 45%, and stage 3 and 4
combined is 25%.

34
 2003 AAN RITE Discussion & Reference Manual

Question(s) 199: Behavioral from visceral cell groups of the brain stem
associated with the oculomotor, facial,
Discussion: glossopharyngeal and vagus nerves. All of
Clinically significant obsessions and these are parasympathetic.
compulsions are seen in about half of the
individuals with Tourette's syndrome.
Reference:
1. Benarroch EE, Westmoreland BF, Daube
Reference: JR, et al. Medical neurosciences - an
Frankel M, Cummings JL, Robertson MM, et approach to anatomy, pathology and
al. Obsessions and compulsions in Gilles de physiology by systems and levels. 4th ed.
la Tourette's syndrome. Neurology New York: Lippincott, Williams & Wilkins,
1986;36:378-382. 1999.
_____________________________ 2. Haines DE. Fundamental neuroscience.
Question(s) 200: 2nd ed. New York: W.B. Saunders, 2002.
Pharmacology/Chemistry _____________________________
Discussion: Question(s) 203: Anatomy
The termination of synaptic actions of L-
Discussion:
glutamate, as with GABA and most
The contralateral cerebellar hemisphere (in
monoamines, depends on its active uptake
this case, the left cerebellar hemisphere) is
by neurons and glia. The glutamate
engaged with motor tasks planned and
transporter is energy-dependent and
executed from the opposite cerebral
coupled to co-transport of Na+ and K+. Loss
hemisphere. Activation of a cerebral cortical
of glutamate uptake occurs in patients with
motor strip is accompanied by activation of
amyotrophic lateral sclerosis. The glutamate
the contralateral cerebellar hemisphere.
transporter has been cloned.
Conversely, if the motor areas of a cerebral
hemisphere are damaged, the contralateral
Reference: cerebellar hemisphere shows a reduction in
1. Rothstein JD, Martin LJ, Kuncl RW. activity.
Decreased glutamate transport by the brain
and spinal cord in amyotrophic lateral Reference:
sclerosis. NEJM 1992;326:1464-1468.
Benarroch EE, Westmoreland BF, Daube
2. Bradley WG, Daroff RB, Fenichel GM, et JR, et al. Medical neurosciences - an
al, editors. Neurology in clinical practice. 3rd approach to anatomy, pathology and
ed. New York: Butterworth-Heinemann, physiology by systems and levels. 4th ed.
1999. New York: Lippincott, Williams & Wilkins,
_____________________________ 1999.
Question(s) 201: Physiology _____________________________
Discussion: Question(s) 204:
50% decrease in CMAP amplitude at the Pharmacology/Chemistry
elbow compared with the wrist is consistent Discussion:
with conduction block below the proximal Ceramide is released in conditions of cell
point of stimulation and above the distal stress and binding of cytokines to death
point of stimulation. receptors and can activate caspase 3,
probably through release of cytochrome c.
Reference:
Kimura J. Electrodiagnosis in disease of Reference:
nerve and muscle. 3rd ed. New York: Oxford McDonald ES, Windebank AJ. Mechanisms
University Press, 2001. of neurotoxic injury and cell death.
_____________________________ Neurology Clinics 2000;3:525-540.
Question(s) 202: Anatomy _____________________________
Discussion:
The cranial preganglionic outflow arises

35
 2003 AAN RITE Discussion & Reference Manual

Question(s) 205: Reference:

Pharmacology/Chemistry Bradley WG, Daroff RB, Fenichel GM, et al,
Discussion: editors. Neurology in clinical practice. 3rd
ed. New York: Butterworth-Heinemann,
The selective serotonin transporter (SERT)
1999.
blockers, or SSRIs, include fluoxetine,
fluvoxamine, paroxetine, sertraline and _____________________________
citalopram. Paroxetine is the most potent Question(s) 208: Clinical Pediatrics
SERT blocker, citalopram the most Discussion:
selective, and fluoxetine the longest lasting. ADHD and obsessive-compulsive symptoms
Sertraline is also a potent blocker of the are often associated with Tourette’s
dopamine transporter. Venlafaxine is a SSRI syndrome. Boys are more often affected
at low dose, and at high dose also blocks than girls; 96% have symptoms before 11
the norepinephrine transporter (NET). years. An increase in seizures is not seen.
Coprolalia is not necessary for the
Reference: diagnosis.
Richelson E. Pharmacology of
antidepressants. Mayo Clin Proc Reference:
2001;76:511-527. 1. Berg BO, editor. Principles of child
_____________________________ neurology. New York: McGraw-Hill, 1996.
Question(s) 206: 2. Menkes JH, Sarnat HB, editors. Child
Pharmacology/Chemistry neurology. Philadelphia: Lippincott, Williams
Discussion: & Wilkins, 2000.
The CD4+ helper cells (Th) exist as two _____________________________
distinct subsets, Th1 and Th2. Th1 cells Question(s) 209: Anatomy
produce proinflammatory cytokines, such as Discussion:
interleukin-2 (IL)-2, tumor necrosis factor The adductor pollicis normally brings the
(TNF) alpha, and interferon (IF) gamma, thumb toward the palm. When the ulnar
which are the major players in cell-mediated nerve is non-functional, the flexor pollicis
immunity and proinflammatory responses. In assumes the role of adducting the
contrast, Th2 cells produce IL-10, which metacarpal.
inhibits the inflammatory effectors of Th1
cells. Interferon beta is produced by
fibroblasts and may affect immune balance Reference:
in favor of an ant-inflammatory responses, Brazis PW, Masdeu JC, Biller J. Localization
by inhibiting production of Th1 cytokines, in clinical neurology. 4th ed. Philadelphia:
such as IL-2 and increasing that of Th2 Lippincott, Williams & Wilkins, 2001.
cytokines, such as IL-10. _____________________________

Reference:
Dalakas M. Basic aspects of
neuroimmunology as they relate to
immunotherapeutic targets: present and
future prospects. Ann Neurol
1995;37(supplement 1):S2-S13.
_____________________________
Question(s) 207: Clinical Adult
Discussion:
The primary cancers most responsible for
spinal cord metastasis are lung (49%),
breast (15%), lymphoma (9%), colorectal
(7%), renal and head and neck (6% each).

36
 2003 AAN RITE Discussion & Reference Manual

Question(s) 210: Anatomy Reference:

Discussion: Kryger MH, Roth T, Dement WC. Principles
and practice of sleep medicine. 3rd ed.
The "locked-in" (deafferented state) consists
Philadelphia: WB Saunders Company, 2000.
of quadriplegia, aphonia and horizontal gaze
impairment. Corticospinal, corticobulbar and _____________________________
corticopontine tracts are all involved. The Question(s) 213: Behavioral
quadriplegia is due to bilateral corticospinal Discussion:
tract involvement. Aphonia is due to Achromatopsia follows lesions that involve
involvement of the corticobulbar tract the occipital cortex inferior to the calcarine
destined to the lower cranial nerves. sulcus. Damage in this area produces a
Horizontal gaze paralysis is due to superior visual field defect (upper
involvement of the fascicles of cranial nerve quadrantanopsia), and loss of color vision in
VI. Because the reticular formation is not the preserved inferior visual field.
usually affected, the patient is awake. In
addition, since the supranuclear oculomotor
pathways are dorsal and in the midbrain, the Reference:
patient can look up and blink. A remarkable Damasio A, Tranel D, Rizzo M. Disorders of
first hand account of this horrific syndrome is complex visual processing. In: Mesulam
given in "The Diving Bell and the Butterfly" MM, editor. Principles of behavioral and
by Jean-Dominique Bauby. cognitive neurology. 2nd ed. New York:
Oxford University Press, 2000.
_____________________________
Reference:
1. Parent A. Carpenter's human Question(s) 214: Pathology
neuroanatomy. 9th ed. Baltimore: Williams & Discussion:
Wilkins, 1996. Seizure disorders frequently are associated
2. Brazis PW, Masdeu JC, Biller J. with neuronal loss in the hippocampus, in
Localization in clinical neurology. 3rd ed. both end-folium and Sommer's sector. The
Boston: Little, Brown and Co., 1996. condition is known as mesial temporal
3. Bauby JD. The diving bell and the sclerosis.
butterfly. New York: Alfred A. Knopf, 1997.
_____________________________ Reference:
Question(s) 211: Physiology Graham DI, Lantos PL. Greenfield's
Discussion: neuropathology. 7th ed. New York: Arnold
A common anomaly of innervation in the leg Press, 2002.
involves the accessory peroneal nerve. It is _____________________________
recognized when the amplitude of the Question(s) 215: Physiology
compound muscle action potential is larger
Discussion:
with stimulation at the knee than it is with
Typical triphasic waves would most likely
stimulation at the ankle and confirmed by
suggest the presence of hepatic coma.
obtaining a response with stimulation behind
the ankle.
Reference:
Reference: Niedermeyer E, Lopes da Silva F, editors.
Electroencephalography: basic principles,
Kimura J. Electrodiagnosis in disease of
clinical applications, and related fields. 4th
nerve and muscle. 3rd ed. New York: Oxford
ed. Philadelphia: Lippincott, Williams &
University Press, 2001.
Wilkins, 1998.
_____________________________
_____________________________
Question(s) 212: Physiology
Discussion:
A lesion in the area ventral to the locus
ceruleus in the pontine tegmentum causes
REM sleep without atonia.

37
 2003 AAN RITE Discussion & Reference Manual

Question(s) 216: Anatomy circumference of 45 cm is greater than the

98th percentile. In the absence of other
Discussion: cerebral malformations, congenital
A unilateral lesion of the ventrocaudal pons aqueductal stenosis is the most common
results in ipsilateral lateral rectus and facial cause of noncommunicating hydrocephalus.
paresis and a contralateral facial sparing A familial X-linked form accounts for 2% of
hemiparesis. This is known as the Millard- these. Increased intracranial pressure in the
Gubler syndrome. context of hypoxic ischemic encephalopathy
does not cause increased head
Reference: circumference at birth. Klippel-Feil syndrome
1. Brazis PW, Masdeu JC, Biller J. (fusion of the cervical vertebra) may be
Localization in clinical neurology. 4th ed. associated with Chiari malformation but is a
Philadelphia: Lippincott, Williams & Wilkins, much less common cause of hydrocephalus.
2001. Choroid plexus papillomas are typically
2. Haines DE. Fundamental neuroscience. located in one lateral ventricle and become
2nd ed. New York: W.B. Saunders, 2002. symptomatic after the perinatal period,
usually by obstructing ventricular outflow.
_____________________________
Lissencephaly is typically associated with
Question(s) 217: Clinical Adult microcephaly.
Discussion:
REM-sleep behavior disorder is
Reference:
characterized by motor activity, often violent,
accompanying dreams. Polysomnography Fenichel GM. Clinical pediatric neurology: a
reveals persistent EMG activity during signs and symptoms approach.
periods that are otherwise typical of REM Philadelphia: WB Saunders, 1997.
sleep. The condition is most common in _____________________________
elderly men, and usually responds well to Question(s) 220: Neuroimaging
clonazepam. Discussion:
Enhancing ring lesion is a non-specific
Reference: finding and may be due to abscess, tumor,
Bradley WG, Daroff RB, Fenichel GM, et al, cysticercosis cyst or resolving hematoma.
editors. Neurology in clinical practice. 3rd Although there are certain neuroimaging
ed. New York: Butterworth-Heinemann, characteristics that suggest the cause,
1999. clinical history or pathology findings are
needed for definite diagnosis.
_____________________________
Question(s) 218: Anatomy
Reference:
Discussion:
The locus ceruleus is the principal location Salzman C, Tuazon CU. Value of the ring
of noradrenergic neurons in the central enhancing sign in differentiating
nervous system (CNS). These intracerebral hematoma and brain abscess.
noradrenergic neurons project widely Arch Intern Med 1987;147:951-2.
throughout the CNS and appear to be _____________________________
important in regulation of the sleep-wake Question(s) 221: Physiology
cycle and attentional mechanisms. Discussion:
For depolarization to occur, Na+ flows
Reference: inward and K+ flows outward.
Haines DE. Fundamental neuroscience. 2nd
ed. New York: W.B. Saunders, 2002. Reference:
_____________________________ Kandel ER, Schwartz JH, Jessel TM.
Question(s) 219: Clinical Pediatrics Principles of neural science. 4th ed. New
Discussion: York: McGraw-Hill, 2000.
At birth the median head circumference of a _____________________________
normal term infant is 35 cm. A head

38
 2003 AAN RITE Discussion & Reference Manual

Question(s) 222: Pathology Reference:

Discussion: Bradley WG, Daroff RB, Fenichel GM, et al,
editors. Neurology in clinical practice. 3rd
Ethylene glycol toxicity has birefringent
ed. New York: Butterworth-Heinemann,
calcium oxalate deposits in and around
1999.
blood vessels. Oxalic acid crystals may be
detected in the urine. _____________________________
Question(s) 226: Pathology
Reference: Discussion:
Ellison D, Love S. Neuropathology: a Paracrystalline intramitochondrial inclusions
reference text of CNS pathology. Chicago: are the most definitive ultrastructural
CV Mosby, Inc., 1998. indicators of abnormal mitochondria.
Tubular cristae, calcium inclusions and
_____________________________
fibrillary background may be seen in normal
Question(s) 223: Behavioral mitochondria. Viral inclusions are not seen
Discussion: in mitochondria.
Impulsive and antisocial behavior are often
seen after lesions of the orbitofrontal cortex.
Reference:
Akinetic mutism is seen with medial frontal
Graham DI, Lantos PL. Greenfield's
lesions. Depression and apathy are more
neuropathology. 7th ed. New York: Arnold
commonly seen with left dorsolateral frontal
Press, 2002.
lesions or subcortical caudate lesions.
Hyposexuality is more common with _____________________________
temporal lobe lesions. Question(s) 227: Anatomy
Discussion:
Reference: Ascending fibers that reach the medulla via
Feinberg TE, Farah MJ. Behavioral the fasciculus gracilis and cuneatus
neurology and neuropsychology. New York: terminate in the nucleus cuneatus and
McGraw Hill, 1997. nucleus gracilis. From there, axons sweep
ventromedially as the internal arcuate fibers,
_____________________________
cross the midline and then continue upward
Question(s) 224: Behavioral as the medial lemniscus.
Discussion:
The majority of structural lesions associated
Reference:
with development of obsessive-compulsive
1. Benarroch EE, Westmoreland BF, Daube
behavior have involved the frontal lobe
JR, et al. Medical neurosciences - an
and/or frontal-basal ganglia network
approach to anatomy, pathology and
connections.
physiology by systems and levels. 4th ed.
New York: Lippincott, Williams & Wilkins,
Reference: 1999.
Swoboda KJ, Jenike MA. Frontal 2. Haines DE. Fundamental neuroscience.
abnormalities in a patient with obsessive- 2nd ed. New York: W.B. Saunders, 2002.
compulsive disorder. Neurology _____________________________
1995;45:2130-2134.
_____________________________
Question(s) 225: Clinical Adult
Discussion:
A spinocerebellar syndrome with ataxia,
ophthalmoparesis, and Babinski signs
developing in a patient with a long history of
fat malabsorption suggests a deficiency of
vitamin E.

39
 2003 AAN RITE Discussion & Reference Manual

Question(s) 228: Clinical Adult Reference:

Discussion: 1. Benarroch EE, Westmoreland BF, Daube
JR, et al. Medical neurosciences - an
Cerebral malaria most commonly manifests
approach to anatomy, pathology and
with seizures and altered consciousness.
physiology by systems and levels. 4th ed.
Focal deficits and movement disorders
New York: Lippincott, Williams & Wilkins,
occur less commonly. Cerebral malaria is
1999.
almost always caused by P. falciparum. It
should be suspected in patients with a 2. Haines DE. Fundamental neuroscience.
history of travel to endemic areas. 2nd ed. New York: W.B. Saunders, 2002.
_____________________________
Question(s) 232: Clinical Adult
Reference:
Bradley WG, Daroff RB, Fenichel GM, et al, Discussion:
editors. Neurology in clinical practice. 3rd Chronic paroxysmal hemicrania is
ed. New York: Butterworth-Heinemann, characterized by frequent, short-lived,
1999. unilateral, fronto-temporal headaches with
_____________________________ ocular and nasal autonomic symptoms. Jaw
claudication is a feature of temporal arteritis.
Question(s) 229: Behavioral Horner’s syndrome is present in chronic
Discussion: paroxysmal hemicrania but abducent nerve
Many authorities consider prosopagnosia as palsies do not occur.
a more subtle form of visual agnosia and
almost all patients with visual agnosia show
difficulty in recognizing familiar faces.
Reference:
Victor M, Ropper A. Adams and Victor's
principles of neurology. 7th ed. New York:
Reference: McGraw-Hill, Co., 2001.
Damasio A, Tranel D, Rizzo M. Disorders of _____________________________
complex visual processing. In: Mesulam Question(s) 233: Anatomy
MM, editor. Principles of behavioral and
cognitive neurology. 2nd ed. New York: Discussion:
Oxford University Press, 2000. A lesion in the base of the midbrain
_____________________________ (basipeduncular region) will produce an
ipsilateral third nerve palsy and contralateral
Question(s) 230: Physiology hemiparesis (Weber's syndrome).
Discussion:
A nocturnal seizure in an eight-year-old child
is most likely due to a benign focal epilepsy
Reference:
of childhood, associated with centrotemporal 1. Brazis PW, Masdeu JC, Biller J.
spikes on the EEG. Localization in clinical neurology. 4th ed.
Philadelphia: Lippincott, Williams & Wilkins,
2001.
Reference: 2. Haines DE. Fundamental neuroscience.
Niedermeyer E, Lopes da Silva F, editors. 2nd ed. New York: W.B. Saunders, 2002.
Electroencephalography: basic principles, _____________________________
clinical applications, and related fields. 4th
ed. Philadelphia: Lippincott, Williams &
Wilkins, 1998.
_____________________________
Question(s) 231: Anatomy
Discussion:
The central tegmental tract consists of
descending fibers from midbrain nuclei that
project to the inferior olivary complex and
ascending fibers from the lower brain stem
that project to thalamic nuclei.

40
 2003 AAN RITE Discussion & Reference Manual

Question(s) 234: Physiology could be considered. While antiphospholipid

antibodies are an important cause of
Discussion: elevated prothrombin times, they would not
The diagnosis is brachial plexopathy of the be an important diagnostic possibility in a
upper trunk. In brachial plexopathy, needle patient presenting with spontaneous
EMG demonstrates abnormality in muscles hemorrhage at a young age.
innervated by a trunk or cord and paraspinal
muscles are spared.
Reference:
Brazis PW, Masdeu JC, Biller J. Localization
Reference: in clinical neurology. 4th ed. Philadelphia:
Kimura J. Electrodiagnosis in disease of Lippincott, Williams & Wilkins, 2001.
nerve and muscle. 3rd ed. New York: Oxford
_____________________________
University Press, 2001.
_____________________________ Question(s) 238: Clinical Pediatrics
Question(s) 235: Clinical Adult Discussion:
While spinal muscular atrophy usually does
Discussion: not present with arthrogryposis (congenital
Essential tremor is typically characterized by joint contractures), it is one of the most
bilateral postural tremor involving the upper common etiologies of this finding. Many
extremities. Head titubations and vocal other neuromuscular conditions can present
tremor are also seen. with arthrogryposis including myotonic
dystrophy and congenital fiber type
Reference: disproportion. Uterine restraint and uterine
Bradley WG, Daroff RB, Fenichel GM, et al, bands are also frequent causes of
editors. Neurology in clinical practice. 3rd arthrogryposis, but these conditions do not
ed. New York: Butterworth-Heinemann, usually have severe hypotonia.
1999.
_____________________________ Reference:
Question(s) 236: Anatomy Volpe JJ. Neurology of the newborn. 4th ed.
Discussion: Philadelphia: WB Saunders, 2001.
In the adult, spinal cord segments are not all _____________________________
adjacent to the corresponding vertebral
body. The tenth thoracic spinal cord
segment is adjacent to the eighth thoracic
vertebral body.

Reference:
Haines DE. Fundamental neuroscience. 2nd
ed. New York: W.B. Saunders, 2002.
_____________________________
Question(s) 237: Clinical Adult
Discussion:
A femoral neuropathy or high lumbar plexus
lesion associated with retroperitoneal pain in
a hemophiliac with prolonged PTT suggests
a retroperitoneal hemorrhage. While imaging
and physiological tests might confirm the
diagnosis, definitive treatment requires
replacement with Factor VIII concentrates.
The amount administered depends on the
patient's Factor VIII level. When adequately
replaced, surgical drainage based on
imaging studies and his clinical condition

41
 2003 AAN RITE Discussion & Reference Manual

Question(s) 239: chromosomes 1p and 19q in anaplastic

Pharmacology/Chemistry oligodendrogliomas is associated with
increased responsiveness to chemotherapy.
Discussion:
Tyrosine hydroxylase (TH) deficiency is an
autosomal recessive disorder of biogenic Reference:
amine synthesis in which the conversion of Kleihues P, Cavenee WK, editors. Pathology
L-tyrosine to L-dihydroxyphenylalanine (L- and genetics of tumours of the nervous
dopa) is impaired. L-dopa is subsequently system. Lyon: International Agency for
metabolized to dopamine, and to Research on Cancer, 2000.
homovanillic acid (HVA) and 3-methoxy-4- _____________________________
hydroxyphenylethyleneglycol (via
Question(s) 242: Physiology
norepinephrine). Thus, HVAS and 5-
hydroxyindole acetic acid (5-HIAA) levels in Discussion:
cerebrospinal fluid are diminished in TH The combination of an abnormality of wave I
deficiency, although 5-HIAA, a metabolite of and subsequent waves on the ipsilateral
serotonin, is normal. Clinical manifestations side and a contralateral prolongation of III-V
include parkinsonian and dystonic features is most often seen with a cerebellpontine
presenting in infancy. Improvement is seen angle tumor that compresses the brainstem.
with the administration of oral L-dopa.
Reference:
Reference: Chiappa KH. Evoked potentials in clinical
Wevers RA, De Rijk-Van Andel JF, et al. A medicine. 3rd ed. New York: Lippincott-
review of biochemical and molecular genetic Raven Press, 1997.
aspects of tyrosine hydroxylase deficiency _____________________________
including a novel mutation (291delC). J Question(s) 243: Behavioral
Inherit Metab Dis 1999;22:364-373.
Discussion:
_____________________________ Diffuse Lewy body dementia is
Question(s) 240: Clinical Adult characterized by dementia with cortical
Discussion: impairments, parkinsonism, vivid visual
Alexia without agraphia (pure alexia) results hallucinations, heightened sensitivity to
from damage to the pathways conveying neuroleptics, and a fluctuating clinical
visual information inputs from both course with periods of episodic confusion
hemispheres to the dominant angular gyrus, mixed with periods of lucidity.
which itself remains intact but disconnected
from visual regions. This syndrome is Reference:
usually due to combined lesions of the
McKeith IG, Galasko D, Levsaka K, et al.
dominant medial occipital region and the
Consensus guidelines for the clinical and
inferior fibers of the splenium of the corpus
pathological diagnosis of dementia with
callosum. It can also be seen with a single
Lewy bodies (DLB): report of the consortium
lesion of the dominant occipitoparietal
on DLB international workshop. Neurology
periventricular white matter beneath and
1996;47:1113-1124.
beside the occipital horn of the lateral
_____________________________
ventricle.

Reference:
Brazis PW, Masdeu JC, Biller J. Localization
in clinical neurology. 4th ed. Philadelphia:
Lippincott, Williams & Wilkins, 2001.
_____________________________
Question(s) 241: Pathology
Discussion:
Combined loss of heterozygosity for

42
 2003 AAN RITE Discussion & Reference Manual

Question(s) 244: Physiology gestures to regulate social interaction. 2.

Failure to develop peer relationships
Discussion: appropriate to developmental level 3. A lack
The convention in EEG is that if the first of spontaneous seeking to share enjoyment,
electrode of the pair is surface negative, the interests or achievements with other people
deflection goes up. If there is a focal area of 4. Lack of social or emotional reciprocity
surface negativity or positivity, there is a B. Restricted repetitive and stereotyped
phase reversal in adjacent channels at the patterns of behavior, interests and activities,
common electrode. as manifested by at least one of the
following: 1. Encompassing preoccupation
Reference: with one or more stereotyped and restricted
Fisch B. Spehlmann's EEG primer. 2nd ed. patterns of interest that is abnormal either in
Amsterdam: Elsevier Science Publications, intensity or focus, 2. Apparently inflexible
1991. adherence to specific, nonfunctional routines
_____________________________ or rituals, 3. Stereotyped and repetitive
motor mannerisms (e.g. hand or finger
Question(s) 245: Pathology flapping or twisting, or complex whole body
Discussion: movements), 4. Persistent preoccupation
Visual disturbances due to lesions around with parts of objects C. The disturbance
the optic nerve; and polydipsia, polyuria, causes clinically significant impairment in
somnolence or obesity due to involvement of social, occupational, or other important
the hypothalamus and pituitary gland are areas of functioning D. There is no clinically
among the most common manifestations of significant general delay in language (i.e.
central nervous system sarcoidosis. single words used by age 2 years,
communicative phrases used by age 3
years). E. There is no clinically delay in
Reference: cognitive development or in the
Graham DI, Lantos PL. Greenfield's development of age-appropriate self-help
neuropathology. 7th ed. New York: Arnold skills, adaptive behavior (other than in social
Press, 2002. interaction), and curiosity about the
_____________________________ environment in childhood. F. Criteria are not
Question(s) 246: Physiology met for another specific Pervasive
Discussion: Developmental Disorder or Schizophrenia.
A given skeletal muscle fiber discharges The criteria do not include delay in cognitive
once in response to an action potential, development, language acquisition, or in
fibrillates following denervation, is part of development of self-help adaptive skills.
only one motor unit, has a conduction Stereotyped and repetitive use of language
velocity of about 5 m/sec, and is innervated is not a criterion for Asperger syndrome.
by either a 'fast' or 'slow' axon, but not both. These are all differences that distinguish
Asperger syndrome from autistic disorder.

Reference:
Reference:
Kandel ER, Schwartz JH, Jessel TM.
Principles of neural science. 4th ed. New American Psychiatric Association.
York: McGraw-Hill, 2000. Diagnostic and statistical manual of mental
disorders. 4th rev. ed. Washington:
_____________________________
American Psychiatric Association, 2000.
Question(s) 247: Clinical Pediatrics _____________________________
Discussion:
DSM IV criteria for the diagnosis of
Asperger’s disorder include: A. Qualitative
impairment in social interaction as
manifested by at least two of the following:
1. Marked impairment in the use of multiple
nonverbal behaviors, such as eye-to-eye
gaze, facial expression, body postures and

43
 2003 AAN RITE Discussion & Reference Manual

Question(s) 248: both the nerve fibers and their termination in

Pharmacology/Chemistry the cochlear nuclei.
Discussion:
Fabry's disease is an X-linked defect in Reference:
alpha-galactosidase. It is characterized by Haines DE. Fundamental neuroscience. 2nd
painful peripheral neuropathy with ed. New York: W.B. Saunders, 2002.
autonomic manifestations, a typical rash in _____________________________
the lower half of the body, and accumulation
Question(s) 252: Pathology
of glycolipids in the endothelium of cerebral
vessels and renal glomerular arterioles. Discussion:
Central neurocytoma is a mature neuronal
intraventricular tumor, which is
Reference: microscopically indistinguishable from
Grewal RP. Stroke in Fabry's disease. J oligodendroglioma. Most tumors called
Neurol 1994;241:153-156. intraventricular oligodendrogliomas in the
_____________________________ past were probably central neurocytomas.
Question(s) 249: Physiology Confirmation is provided by
immunohistochemical demonstration of
Discussion: neuronal antigens or electron microscopic
Generalized beta activity (activity over 14 identification of neuronal features, such as
Hz) can be seen as a consequence of drug cytoplasmic dense core granules.
use, particularly benzodiazepines or
barbiturates.
Reference:
Burger PC, Scheithauer BW, Vogel FS.
Reference: Surgical pathology of the nervous system
Niedermeyer E, Lopes da Silva F, editors. and its coverings. 4th ed. New York:
Electroencephalography: basic principles, Churchill Livingston, 2002.
clinical applications, and related fields. 4th
_____________________________
ed. Philadelphia: Lippincott, Williams &
Wilkins, 1998. Question(s) 253:
_____________________________ Pharmacology/Chemistry
Question(s) 250: Anatomy Discussion:
Gilles de la Tourette's syndrome may be
Discussion: worsened by stimulants such as
Descending fibers in the spinal trigeminal methylphenidate. Drugs used for treatment
tract convey impulses concerned with pain, include haloperidol, pimozide and clonidine.
thermal and tactile sense from the face,
forehead and mucous membranes of the
nose and mouth. The spinal trigeminal tract Reference:
and nucleus, pars caudalis, are the only Jankovic J. Tourette's syndrome. NEJM
parts uniquely concerned with the 2001;345:1184-1192.
perception of pain and thermal sense. _____________________________

Reference:
Haines DE. Fundamental neuroscience. 2nd
ed. New York: W.B. Saunders, 2002.
_____________________________
Question(s) 251: Anatomy
Discussion:
The cochlear nerve originates from the spiral
ganglion, enters the brainstem at the
cerebellopontine angle and terminates on
the dorsal and ventral cochlear nuclei. The
pattern of tonotopic localization is evident in

44
 2003 AAN RITE Discussion & Reference Manual

Question(s) 254: Physiology whose exotoxin selectively inhibits inhibitory

synapses in the CNS. The enhanced
Discussion: excitability of the nervous system produces
Patients with increased intracranial the characteristic muscular spasms with
pressure, such as that caused by ventricular trismus, facial rigidity, opisthotonus and poor
outflow obstruction, typically have EEGs feeding. Children born at home to
which show rhythmic slow activity in the unimmunized mothers are at greatest risk.
theta-delta frequency range. The site is The pupils are spared in neonatal tetanus, in
often distant from the site of obstruction. contrast to infantile botulism, another
These EEG findings are not specific. Clostridial disease.

Reference: Reference:
Ebersole JS, Pedley TA. Current practice of Volpe JJ. Neurology of the newborn. 4th ed.
clinical electroencephalography. 3rd ed. Philadelphia: WB Saunders, 2001.
New York: Lippincott Williams and Wilkins.
_____________________________
2002.
_____________________________ Question(s) 258: Clinical Pediatrics
Question(s) 255: Pathology Discussion:
A minority of children with perinatal asphyxia
Discussion: causing selective injury to the basal ganglia
Homer Wright rosettes contain the does not develop symptoms and signs for
cytoplasmic processes (of the tumor cells) in periods ranging for 7-14 years. (Mean 12.9).
the center of the rosette. This type of rosette Nearly one half of these children have early
is commonly seen in neuroblastoma, normal development, and 80% have normal
medulloblastoma, and other PNETs. In intellect. Dystonia continues to progress for
contrast, Flexner-Wintersteiner rosettes a mean of 7 years after onset, although
have a central lumen formed by the tumor most children do not become wheelchair
cells. This type of rosette is seen in bound.
retinoblastoma as well as other types of
PNET.
Reference:
Volpe JJ. Neurology of the newborn. 4th ed.
Reference: Philadelphia: WB Saunders, 2001.
Fuller GN, Goodman JC. Practical review of
_____________________________
neuropathology. Philadelphia: Lippincott,
Williams & Wilkins, 2001. Question(s) 259: Clinical Pediatrics
_____________________________ Discussion:
Question(s) 256: Clinical Pediatrics Fragile X syndrome exhibits a transmission
pattern of X-linked inheritance with no male
Discussion: to male transmission. However, both males
Prenatal cytomegalovirus infection can and females can be affected. In
cause severe cerebral injury, such as contradistinction to most X-linked recessive
microcephaly, microgyria, cerebral disorders, males carrying the mutant gene
calcifications and chorioretinitis. Minimally do not always have manifestations of the
affected children can have only hearing loss. disorder but can pass the gene on to their
daughters.
Reference:
Menkes JH, Sarnat HB, editors. Child Reference:
neurology. 6th ed. Philadelphia: Lippincott, Swaiman KF, Ashwal S, editors. Pediatric
Williams & Wilkins, 2000. neurology. 3rd ed. St. Louis: Mosby, 1999.
_____________________________ _____________________________
Question(s) 257: Clinical Pediatrics
Discussion:
Neonatal tetanus results from infection of
the umbilical stump by Clostridium tetani,

45
 2003 AAN RITE Discussion & Reference Manual

Question(s) 260: Clinical Pediatrics Question(s) 265 - 269:

Discussion: Pharmacology/Chemistry
Pyridoxine (vitamin B6) dependent seizures Discussion:
usually begin in the neonatal period but can Several brain neoplasms are associated
appear up to a year of age. The diagnosis is with either impaired expression of tumor
made in the infant by cessation of seizures suppressor genes or increased activity of
after parenteral administration of 100 mg of oncogenes. Mutations of the p53 gene
pyridoxine. It is an autosomal recessive (chromosome 17p) occur in 40% of all
disorder and requires life-long treatment with astrocytic tumors, particularly in young
pyridoxine. adults. Mutations in the pRB gene
(chromosome 13q), encoding for a protein
that controls the G1-S phase transition
Reference: during the cell cycle, occurs in patients with
Berg BO. Principles of child neurology. New retinoblastoma. Mutations of the NF-1 gene
York: McGraw-Hill, 1996. (chromosome 17q) encoding for
_____________________________ neurofibromin (which inhibits the ras-
Question(s) 261: Clinical Pediatrics oncogene pathway via activation of ras-
Discussion: GTPase) occurs in neurofibromatosis type 1.
The ketogenic diet is a mainstay in the Other genetic disorders associated with
treatment of glucose transporter type 1 brain tumors are neurofibromatosis type 2,
defect. Acetazolamide in conjuction with the associated with mutations of the gene in
diet can produce a symptomatic metabolic chromosome 22q encoding for merlin (a
acidosis and therefore should should not be cytoskeletal protein), and tuberous sclerosis,
used when the diet is initiated and only with associated with mutations in the gene
caution while a child is on the diet. encoding for tuberin, a protein of unknown
Topirimate also is a weak carbonic function.
anhydrase inhibitor and the physician should
be aware that it also can lead to a metabolic Reference:
acidosis. Hill JR, Kuriyama N, Kuriyama H, et al.
Molecular genetics of brain tumors. Arch
Reference: Neurol 1999;56:439-441.
Swaiman KF, Ashwal S, editors. Pediatric _____________________________
neurology. 3rd ed. St. Louis: Mosby, 1999. Question(s) 270 - 271: Physiology
_____________________________ Discussion:
Question(s) 262 - 264: Physiology The F-wave has a variable latency. The H-
Discussion: reflex is dependent on the integrity of the
Low amplitude compound muscle action proximal segment of the sensory and motor
potentials are characteristic of Lambert- roots.
Eaton myasthenic syndrome. An amplitude
decrement of compound muscle action Reference:
potentials to low rates of repetitive 1. Kimura J. Electrodiagnosis in disease of
stimulation is characteristically seen in nerve and muscle. 3rd ed. New York: Oxford
myasthenia gravis and Lambert-Eaton University Press, 2001.
myasthenic syndrome. F-waves are usually 2. Kleihues P, Cavenee WK, editors.
present in neuromuscular junction disorders. Pathology and genetics of tumors of the
nervous system. 2nd ed. New York: Oxford
Reference: University Press, 2000.
Kimura J. Electrodiagnosis in disease of _____________________________
nerve and muscle. 3rd ed. New York: Oxford
University Press, 2001.
_____________________________

46
 2003 AAN RITE Discussion & Reference Manual

Question(s) 272 - 274: Pathology Reference:

Discussion: Menkes JH, Sarnat HB, editors. Child
neurology. 6th ed. Philadelphia: Lippincott,
Temporal lobe (uncal) herniation
Williams & Wilkins, 2000.
compresses the posterior cerebral artery,
causing posterior cerebral artery infarction _____________________________
and cortical blindess. Leg weakness follows Question(s) 283 - 287: Behavioral
cingulate herniation and entrapment of the Discussion:
anterior cerebral artery with occasional Lesions in the hippocampus produce a
infarctions. Tonsillar herniation will result in declarative memory deficit. Docility may be
cardiorespiratory arrest. seen with bilateral lesions of the amygdala
(part of the Klüver-Bucy Syndrome). Lesions
Reference: of the anterior cingulate may result in loss of
Graham DI, Lantos PL. Greenfield's motivation. Anosognosia (denial of illness or
neuropathology. 7th ed. New York: Arnold impairment) is seen with right parietal
Press, 2002. lesions. Depression is commonly associated
with left prefrontal lesions.
_____________________________
Question(s) 275 - 279:
Pharmacology/Chemistry Reference:
Discussion: 1. Feinberg TE, Farah M. Behavioral
neurology and neuropsychology. New York:
Genetic disorders affecting distinct subunits
McGraw-Hill, 1997.
of muscle ion channels are associated with
specific disturbances of muscle membrane 2. Mesulam MM. Principles of behavioral
excitability. Hyperkalemic periodic paralysis and cognitive neurology. 2nd ed. New York:
and paramyotonia congenita are due to Oxford University Press, 2000.
different mutations of the alpha subunit of _____________________________
the voltage-gated sodium channel. Question(s) 288 - 291: Pathology
Hypokalemic periodic paralysis is due to a Discussion:
mutation in the dihydropyridine (DHP) Patients with von Hippel-Lindau syndrome
receptor. Myotonia congenita is associated have hemangioblastomas of the central
with a mutation of the chloride channel. nervous system (CNS). Patients with
tuberous sclerosis may develop
Reference: subependymal giant cell astrocytomas that
Lehmann-Horn F, Jurkatt-Roth K. Voltage- protrude into the ventricular system. Patients
gated ion channels and hereditary disease. with neurofibromatosis often have
Physiol Rev 1999;79:1317-1372. neurofibromas and schwannomas. Children
_____________________________ with the "bathing trunk" epidermal nevus
syndrome may develop cutaneous and CNS
Question(s) 280 - 282: Clinical Pediatrics melanomas.
Discussion:
Niemann-Pick disease type A can have
cherry red macula, associated with
Reference:
hepatosplenomegaly and mental Burger PC, Scheithauer BW, Vogel FS.
deterioration. Children with the neuronal Surgical pathology of the nervous system
ceroid lipofuscinosis exhibit progressive and its coverings. 4th ed. New York:
dementia and seizures. All of the various Churchill Livingston, 2002.
forms have abnormal visual evoked _____________________________
potentials and electroretinograms. Hurler
syndrome is one of the
mucopolysacharidoses, characterized by
slowed development, coarse facies and
bony abnormalities. Kyphosis appears early.

47
 2003 AAN RITE Discussion & Reference Manual

Question(s) 292 - 295: Pathology red fibers. In the periodic paralyses, there is
myofiber vacuolization during and
Discussion: immediately following attacks; the muscle
Medulloblastomas frequently disseminate biopsy may be normal in between episodes
via the subarachnoid space to the spinal of weakness. The molecular basis of
cord, forming "drop metastases." hypokalemic periodic paralysis is sodium
Ependymomas comprise about 60% of channel mutation, but the cause of the
primary spinal cord neoplasms. Most vacuoles is still unclear.
originate from within the cord proper;
however, the myxopapillary ependymoma
usually arises from the filum terminale. Reference:
Oligodendrogliomas account for 10-15% of Graham DI, Lantos PL. Greenfield's
all gliomas. They are often slow growing and neuropathology. 7th ed. New York: Arnold
calcified and may undergo spontaneous Press, 2002.
hemorrhage. The most common glioma of _____________________________
older adults is glioblastoma.
Question(s) 303 - 306:
Pharmacology/Chemistry
Reference: Discussion:
Burger PC, Scheithauer BW, Vogel FS. Several biological toxins can produce acute
Surgical pathology of the nervous system or subacute neurologic manifestations.
and its coverings. 4th ed. New York: Botulinum toxin prevents release of
Churchill Livingston, 2002. acetylcholine by hydrolyzing proteins in the
_____________________________ synaptic vesicle (synaptobrevin) or the
Question(s) 296 - 299: Clinical Pediatrics terminal membrane (SNAP-25, syntaxin)
involved in exocytosis. Saxitoxin and
Discussion: tetrodotoxin block voltage-gated sodium
Krabbe's disease and metachromatic channels. Mushroom toxins, such as those
leukodystrophy are autosomal recessive from Amanita muscaria, block both
lysosomal enzyme disorders, occuring in cholinergic and GABA synapses. Latrotoxin,
both males and females. In addition to a the venom of the black widow spider,
central myelin abnormality the myelin of produces acute release of acetylcholine
peripheral nerves is also affected, so that from synaptic terminals. Toxins of the
motor nerve conduction velocities are chickpea (Lathyrus) including alpha-amino-
slowed and CSF protein concentration is beta-oxalaminopropionic acid activate
elevated. Seizures are not prominent in glutamate receptors resulting in
either disorder and if present occur late in excitotoxicity.
the disease.

Reference:
Reference: Bradley WG, Daroff RB, Fenichel GM, et al,
Menkes JH, Sarnat HB, editors. Child editors. Neurology in clinical practice. 3rd
neurology. 6th ed. Philadelphia: Lippincott, ed. New York: Butterworth-Heinemann,
Williams & Wilkins, 2000. 1999.
_____________________________ _____________________________
Question(s) 300 - 302: Pathology
Discussion:
Muscle biopsy findings reflect the underlying
biochemical abnormalities. In carnitine
deficiency, there is a defect of transport of
fatty acids into the mitochondria, and
therefore lipid accumulates in the muscle. In
progressive external ophthalmoplegia, large
scale deletions of mitochondrial DNA lead to
the subsarcolemmal accumulations seen on
modified Gomori trichrome stain as ragged

48
 2003 AAN RITE Discussion & Reference Manual

Question(s) 307 - 311: Behavioral diverse manifestations of this multisystem

disease.
Discussion:
Valproate is commonly used to treat bipolar
disorder. Paroxetine may be used to treat Reference:
obsessive-compulsive disorders. Buspirone 1. Terwindt GM, Ophoff RA, Haan J, et al.
is an anxiolytic agent. Benztropine is Familial hemiplegic migraine: a clinical
indicated for acute dystonic reactions. comparison of families linked and unlinked
Bromocriptine may be used to treat to chromosome 19. DMG RG. Cephalagia
neuroleptic malignant syndrome. 1996;16:153-155.
2. Murakami T, Garcia CA, Reiter LT, et al.
Charcot-Marie-Tooth disease and related
Reference:
inherited neuropathies. Medicine
Arana GW, Rosenbaum JF. Handbook of
1996;75:233-250.
psychiatric drug therapy. 4th ed.
3. Ouahchi K. Arita M, Kayden H, et al.
Philadelphia: Lippincott, Williams & Wilkins,
Ataxia with isolated vitamin E deficiency is
2000.
caused by mutations in the alpha-tocopherol
_____________________________
transfer protein. Nat Genet 1995;9:141-145.
Question(s) 312 - 317: 4. Worton RG, Molnar MJ, Brais B, et al.
Pharmacology/Chemistry The muscular dystrophies. In: Scriver CR,
Discussion: Beaudet AL, Sly WS, et al, editors. The
Several inherited neurologic disorders are molecular and metabolic bases of inherited
due to mutations in genes encoding for diseaase. 8th ed. New York: McGraw-Hill,
critical proteins. Familial hemiplegic 2001.
migraine (as well as episodic ataxia type 2, 5. Sampson JR. Tuberous sclerosis. In:
EA-2) is related to mutation in the gene Scriver CR, Beaudet AL, Sly WS, et al,
encoding for the alpha subunit of the P/Q editors. The molecular and metabolic bases
calcium channel. Spinocerebellar of inherited diseaase. 8th ed. New York:
degeneration occurs in families with a McGraw-Hill, 2001.
genetic defect in vitamin E metabolism. _____________________________
Charcot-Marie-Tooth disease may occur as Question(s) 318 - 321: Pathology
a manifestation of several genetic defects.
The X-linked form is due to mutation in the Discussion:
connexin-2 gene (connexin is a gap junction Epidural hematomas are associated with
protein of peripheral myelin). DOPA- lacerations of the meningeal arteries (most
responsive dystonia is due to a defect in often the middle meningeal artery). They are
guanosine triphosphate (GTP) associated with skull fractures in
cyclohydrolase; this enzyme is necessary for approximately 85% of cases. Subdural
formation of tetrahydrobiopterin, the cofactor hematomas are more common than epidural
for tyrosine hydroxylase (the key enzyme for hematomas, and they are associated with
dopamine synthesis). Emerin is a protein tearing of bridging veins. Subdural
that localizes to the inner nuclear hematomas can be associated with skull
membrane, and is deficient in Emery- fractures as well, but this is a less frequent
Dreifuss muscular dystrophy, an X-linked association (when compared to epidural
disorder that presents in the first decade hemorrhage). Subarachnoid hemorrhage is
with toe walking, followed by progressive seen in traumatic brain injury, but important
joint contractures and cardiac conduction differential diagnostic considerations include
defects that may lead to sudden death. rupture of a saccular aneurysm or vascular
Hamartin is the gene product of TSC-1, one malformation.
of two genes that are associated with the
tuberous sclerosis complex. facial Reference:
angiofibromas, hypopigmented macules, Prayson R. Neuropathology review. Totowa:
periungual and subungual fibromas, cavities, Humana Press Inc., 2001.
cortical tubers, renal angiomyolipomas,
_____________________________
cardiac rhabdomyomas and subependymal
giant cell astrocytomas are included in the

49
 2003 AAN RITE Discussion & Reference Manual

Question(s) 322 - 326: Anatomy Question(s) 332 - 334: Behavioral

Discussion: Discussion:
The entorhinal cortex is the gateway for Apolipoprotein E4 associated Alzheimer's
neocortical multimodal information to enter disease is linked to chromosome 19.
the hippocampal circuit. It projects via the Frontotemporal dementia has been linked to
perforant pathway to the dentate gyrus, and chromosome 17. Presenilin 2 associated
granule cells of this region give rise to dementia is linked to chromosome 1.
mossy fibers that innervate the CA3 region.
CA3 neurons project to CA1 neurons via the
Schaffer collaterals. The subiculum, and not Reference:
the CA1 area, provides most of the axons of 1. Coslett HB. Behavioral neurology/higher
the fornix. The mammillary bodies project to cortical function. Seminars in Neurology
the anterior thalamic nuclei via the 2000;20:4.
mamillothalamic tracts. 2. Grabowski TJ, Anderson SW, Cooper GE.
Disorders of cognitive function. Continuum
2002;8:2.
Reference: _____________________________
Parent A. Carpenter's human
Question(s) 335 - 339: Anatomy
neuroanatomy. 9th ed. Baltimore: Williams &
Wilkins, 1996. Discussion:
_____________________________ The posterior cord of the brachial plexus
gives off the subscapular, thoracodorsal,
Question(s) 327 - 331: radial and axillary nerves. The medial cord
Pharmacology/Chemistry gives off the medial head of the median
Discussion: nerve and ulnar nerve. The lateral cord
The management of parkinsonism includes gives off the lateral head of the median
non-dopaminergic drugs as adjuvants for a nerve and the musculocutaneous nerve.
variety of non-motor manifestations.
Midodrine (and fludrocortisone) helps in the
management of orthostatic hypotension in Reference:
patients with multiple system atrophy; Brazis PW, Masdeu JC, Biller J. Localization
clonazepam helps patients with rapid eye in clinical neurology. 4th ed. Philadelphia:
movement sleep behavior disorder; Lippincott, Williams & Wilkins, 2001.
trazodone is helpful for management of _____________________________
insomnia and depression; quetiapine or
olanzapine aid management of psychosis;
and anticholinergic drugs, such as
trihexiphenydil, may help reduce drooling.
Anticholinergic drugs are relatively
contraindicated in elderly patients as they
can worsen cognitive function and trigger
urinary retention.

Reference:
Olanow CW, Koller WC, editors. An
algorithm (decison tree) for the management
of Parkinson's disease. Treatment
guidelines. Neurology 1998;50(suppl 1):S1-
S57.
_____________________________

50
 2003 AAN RITE Discussion & Reference Manual

Question(s) 340 - 342: Clinical Pediatrics Question(s) 349 - 354:

Discussion: Pharmacology/Chemistry
The seizures in the benign rolandic epilepsy Discussion:
of childhood characteristically occur in sleep Ataxia telangiectasia presents in childhood,
with facial twitching and drooling. Spike and is associated with scleral
discharges arise from the central or telangiectasias, oculomotor apraxia and
centrotemporal region. In Lennox-Gastaut chorea. Blood levels of alpha-fetoprotein
syndrome, the EEG typically demonstrates 2 and carcinoembryonic antigen are high, with
Hz spike-and-wave abnormalities; there variable depression of 1gA, 1gE and 1gG
often multiple types of seizures (tonic, atonic subclasses. Wilson disease may present in
and atypical absence) and most patients are children with hepatic disease, hemolytic
mentally retarded. The classic EEG anemia or renal tubular dysfunction.
abnormality seen in the early stages of Neurologic dysfunction usually begins in the
infantile spasms is hypsarrhythmia. 10-16 teenage years, with basal ganglia and
Hz polyspikes, maximal over frontal areas is cognitive dysfunction. Kayser-Fleischer rings
seen in juvenile myoclonic epilepsy. 3 Hz (representing copper deposition in
spike-and-wave is characteristic of the EEG Descemet’s membrane of the cornea) are
in childhood absence epilepsy. characteristic. Serum copper and
ceruloplasmin levels are [Link]
disease results from phytanic acid oxidase
Reference: deficiency, and is associated with elevated
Pellock JM, Dodson WE, Bourgeois BFD, levels of phytanic acid in the blood. Patients
editors. Pediatric epilepsy, diagnosis and develop peripheral neuropathy with ataxia,
treatment. 2nd ed. New York: Demos, 2001. deafness and visual loss with pigmentary
_____________________________ [Link] leukodystrophy
Question(s) 343 - 348: Clinical Pediatrics classically presents in late infancy or early
Discussion: childhood with ataxia, spasticity, optic
Sodium valproate is a reasonable atrophy and dementia. Reflexes are lost
medication to use first in generalized because of demyelinating peripheral
absence seizures, juvenile myoclonic neuropathy. Arylsulfatase A deficiency is
epilepsy, and Lennox Gastaut syndrome. diagnostic, but must be distinguished from
Some authorities recommend ethosuximide pseudodeficiency. Mitochondrial cytopathies
as the first line medication for generalized have protean manifestations, with
absence seizures because of its low side progressive external ophthalmoplegia,
effect profile, but it does not protect against deafness, pigmentary retinopathy, ataxia
generalized tonic, clonic, or tonic clonic and short stature as frequent findings.
seizures. Lennox Gastaut is very difficult to Deletions or mutations in mitochondrial or
treat but sodium valproate is a reasonable nuclear DNA cause impaired oxidative
first line medication. Carbemazepine is a phosphorylation, usually associated with
reasonable first line medication for simple elevated levels of lactic acid in blood and
and complex partial seizures. Treatment CSF. Both primary sulfite oxidase
with anticonvulsants may not be necessary deficiency, and deficiency of this enzyme
in benign rolandic epilepsy. ACTH is the secondary to molybdenum cofactor
usual starting treatment for infantile spasms deficiency are associated with early onset,
in the United States. Vigabatron also has often intractable seizures, profound
been proven efficacious for infantile spasms. developmental delay, microcephaly and
ectopia lentis (often not apparent until two
years of age). Sulfites are typically present
Reference: in fresh urine specimens.
Swaiman KF, Ashwal S, editors. Pediatric
neurology. 3rd ed. St. Louis: Mosby, 1999.
Reference:
_____________________________
Scriver CR, Beaudet AL, Sly WS, et al,
editors. The molecular and metabolic bases
of inherited disease. 8th ed. New York:
McGraw-Hill, 2001.

51
 2003 AAN RITE Discussion & Reference Manual

Question(s) 355 - 360: dominant inheritance. Incontinentia pigmenti

Pharmacology/Chemistry is an X-linked dominant disorder (lethal in
males).
Discussion:
The complex, interacting motor control
systems responsible for eye movements are Reference:
susceptible to the effects of drugs and toxins Menkes JH, Sarnat HB, editors. Child
at many levels. The following associations neurology. 6th ed. Philadelphia: Lippincott,
have been reported:benzodiazepines - Williams & Wilkins, 2000.
divergence paralysis; carbamazepine - _____________________________
downbeat paralysis; cocaine – opsoclonus;
Question(s) 367: Clinical Adult
phenothiazines – internuclear
ophthalmoplegia; phenytoin – periodic Discussion:
alternating nystagmus; tobacco – upbeat The diagnosis of multiple sclerosis (MS) is
[Link] should be remembered that considered to be clinically definite when
some agents (such as phenytoin) might there have been two attacks and there is
produce several types of abnormal eye clinical evidence of two separate lesions.
movements. The correct answers to this This evidence must be an abnormality on
item represent the best matches between physical examination or on paraclinical
the drugs or toxins and the eye movements testing such as evoked potential or imaging
listed. techniques. Pattern shift visual evoked
responses reveal abnormalities in over 90%
of patients with a history of optic neuritis,
Reference: even when visual acuity has returned to
Leigh RJ, Zee DS. The neurology of eye normal. In a well-designed clinical trial,
movements. 3rd ed. New York: Oxford subcutaneous beta interferon was
University Press, 1999. demonstrated to reduce the relapse rate of
_____________________________ certain relapsing-remitting patients. No
Question(s) 361 - 363: Clinical Pediatrics beneficial effect on ultimate disability was
demonstrated over the two to three year
Discussion: follow-up period. An expert consensus panel
Subdural effusions are commonly seen with has concluded that beta interferon may be
bacterial meningitis in children under the helpful for patients with clinically definite MS
age of two, regardless of the organism. who have had at least two acute
Tuberculous meningitis most commonly exacerbations in the previous two years.
manifests as a caseous meningitis resulting
from hematogenous spread from a
pulmonary focus with frequent cranial nerve Reference:
involvement. Pyogenic abscesses result 1. Bradley WG, Daroff RB, Fenichel GM, et
from one of three routes of infection: (1) al. Neurology in clinical practice. 3nd ed.
bloodstream, either from sepsis or from Boston: Butterworth-Heinemann, 1999.
cardiopulmonary shunt, most often cyanotic 2. Panitch H. et al. Randomized comparative
congenital heart disease; (2) contiguous study of interferon beta-1a treatment
infections such as those of the sinuses or regimens in MS - the EVIDENCE trial.
middle ear; or (3) penetrating wounds. Neurology 2002;59:1507-1517.
_____________________________
Reference:
Menkes JH, Sarnat HB, editors. Child
neurology. 6th ed. Philadelphia: Lippincott,
Williams & Wilkins, 2000.
_____________________________
Question(s) 364 - 366: Clinical Pediatrics
Discussion:
Neurofibromatosis type I and tuberous
sclerosisis are transmitted by autosomal

52
 2003 AAN RITE Discussion & Reference Manual

Question(s) 368 - 369: Clinical Adult tachycardia, respiratory difficulty, vomiting,

weakness, and hypotension. It is probably
Discussion: produced by buildup of acetaldehyde due to
Trigeminal neuralgia is characterized by inhibition of ethanol metabolism.
paroxysms of shock-like pains lasting about
a second and often occurring in volleys,
usually beginning at the maxilla or mandible. Reference:
The pain can often be provoked by light 1. O'Donoghue JL. Neurotoxicity of industrial
touch in a trigger zone. Baclofen and a and commercial chemicals. Boca Raton:
variety of anticonvulsant medications are CRC Press, 1985.
often effective treatment. Trigeminal 2. Heimburger DC. Nutrition's interface with
neuralgia most often begins in the sixth or health and disease. In: Bennett JC, Plum F,
seventh decade of life; onset below age 40 editors. Cecil textbook of medicine.
increases the likelihood that multiple Philadelphia: WB Saunders, 1996.
sclerosis is the cause. _____________________________
Question(s) 371 - 372: Behavioral
Reference: Discussion:
Bradley WG, Daroff RB, Fenichel GM, et al, Alzheimer's disease is the most common
editors. Neurology in clinical practice. 3rd degenerative dementia. It is progressive with
ed. New York: Butterworth-Heinemann, deficits occurring in memory, visual spatial
1999. skills, language, higher executive function,
_____________________________ and personality. Primary motor and sensory
Question(s) 370: functions are usually preserved until later in
Pharmacology/Chemistry the disease. Language dysfunction early in
the disease often resembles a transcortical
Discussion: sensory aphasia. Frontotemporal dementia
The most common form of disulfiram
usually presents with profound changes in
neurotoxicity is peripheral polyneuropathy
personality with relative early preservation of
which may on occasion affect proximal
other cognitive functions. Progressive
muscles first, leading to potential
posterior cortical atrophy would have
misdiagnosis as myopathy. The neuropathy
profound visual spatial impairment with
typically appears 5-6 months after institution
relative sparing of other cognitive functions.
of disulfiram treatment. Disulfiram-induced
Diffuse Lewy body dementia has prominent
CNS toxicity is less common. A progressive
extrapyramidal features and visual
neuropathy first appearing 6 months after
hallucinations. Pick's disease demonstrates
stopping alcohol consumption is unlikely to
a profound change in personality and
be alcohol-induced. Although thiamine
behavior while other cognitive functions
deficiency can result in neuropathy, it should
continue to remain intact [Link] scan
not occur on thiamine replacement unless
early in Alzheimer's disease demonstrates
there is some block of intestinal absorption.
hypoperfusion in bilateral temporal parietal
Vitamin E deficiency can produce posterior
areas. Anticholinesterases such as
column, spinocerebellar, and peripheral
donepezil, rivastigmine, and galantamine, as
nerve dysfunction, but this also would not
well as alpha-tocopherol (vitamin E) are
occur in the face of replacement therapy
recommended treatments of Alzheimer's
unless a malabsorption process is present.
disease.
Abetalipoproteinemia (Bassen-Kornzweig
disease) is a process where malabsorption
results in severe Vitamin E deficiency. Reference:
Thiamine is an important coenzyme for a 1. Cummings JL, Benson DF. Dementia: a
number of cellular reactions, including those clinical approach. Boston: Butterworth-
responsible for ATP generation. The Heinemann, 1992.
combination of disulfiram and alcohol 2. Moore DP. Textbook of clinical
produces the "Antabuse syndrome" or neuropsychiatry. New York: Oxford
"acetaldehyde syndrome", characterized by University Press, 2001.
the unpleasant combination of vasodilatation _____________________________
of the face and body, headache,

53
 2003 AAN RITE Discussion & Reference Manual

Question(s) 373: arsenic intoxication. Organophosphates

Pharmacology/Chemistry (OP) inhibit acetylcholinesterase and OP
Discussion: intoxication produces an early syndrome
Nitrous oxide abuse can produce (type I) of excessive muscarinic activation,
myeloneuropathy that is clinically virtually followed by an intermediate syndrome (type
indistinguishable from that produced by II) of excessive nicotinic activation and
vitamin B12 deficiency. Typical symptoms muscle weakness. In addition, OP induces a
include paresthesias in the hands and feet, delayed axonal, predominantly motor
gait ataxia, and leg weakness. A so-called neuropathy associated with manifestations
reverse Lhermitte's sign, in which neck of central nervous system involvement
flexion induces electrical shock-like (ataxia, spasticity); this is attributed to
sensations traveling from the feet upwards, phosphorylation and inhibition of a
may occur. In the setting of nitrous oxide neuropathy target esterase (NTE) and
abuse the serum B12 level and the Schilling impairment of axonal transport. Acrylamide
test are almost always normal. Because impairs axonal transport causing
nitrous oxide interferes with the vitamin B12- accumulation of neurofilaments and
dependent conversion of homocysteine to paranoidal swelling mostly in large
methionine and not with the conversion of myelinated axons. This produces a dying
methylmalonyl coA to succinyl coA, only the back axonopathy, affecting both the
homocysteine level will be elevated, in peripheral nerves and the central tracts.
contrast to vitamin B12 deficiency where (eg., gracile tract and dorsal spinocerebellar)
both homocysteine and methylmalonic acid
levels will be elevated. Reference:
Windebank A. Metal neuropathy. In: Dyck
Reference: PJ, Thomas PK, editors. Peripheral
Lockwood AH. Toxic and metabolic neuropathy. 3rd ed. Philadelphia: WB
encephalopathies. In: Bradley WG, Daroff Saunders, 1993.
RB, Fenichel GM, et al. Neurology in clinical _____________________________
practice. 2nd ed. Boston: Butterworth- Question(s) 375 - 377: Clinical Pediatrics
Heinemann, 1996. Discussion:
_____________________________ The spinal muscular atrophies are a group
Question(s) 374: of autosomal recessively inherited disorders,
Pharmacology/Chemistry characterized by progressive weakness and
Discussion: atrophy of muscles due to degeneration of
Lead has direct effects on porphyrin anterior horn cells in the spinal cord. The
metabolism, by inhibiting gamma- examination reveals weakness and loss of
aminolevulinic acid dehydrase. Lead deep tendon reflexes. Serum CK is normal
intoxication produces a motor neuropathy or slightly elevated. EMG and muscle biopsy
that affects predominantly, but not are consistant with denervation. The
exclusively, the radial nerve. Associated majority of children have a homozygous
features include abdominal pain, bluish deletion of exon 7 and 8 on chromosome 5.
discoloration of the gums just below the Early onset SMA (1 and 2) have rapidly
teeth, microcytic hypochromic anemia with progressive weakness with severly
basophilic stippling of the red cells, and shortened life-span, while SMA 3
increased coproporphyrin levels. Arsenic (Kugelberg-Welander disease) is a milder
reacts with sulfhydryl groups of proteins and form of the disease with onset after 18
interferes with several steps of oxidative months of age, often with survival into adult
metabolism in the neuron, producing dying life.
back type axonal degeneration, particularly
in myelinated fibers. Thallium ions act Reference:
interchangeably with potassium in respect to Menkes JH, Sarnat HB, editors. Child
their transport by the Na/K ATPase system. neurology. 6th ed. Philadelphia: Lippincott,
Alopecia, and cranial nerve and autonomic Williams & Wilkins, 2000.
involvement help to distinguish thallium from _____________________________

54
 2003 AAN RITE Discussion & Reference Manual

Question(s) 378: no mass effect.

Pharmacology/Chemistry
Discussion: Reference:
Methanol is metabolized to formaldehyde Belman AL, Preston T, Milazzo M. Human
and formic acid by alcohol dehydrogenase. immunodeficiency virus and acquired
Formic acid is believed to be the cause of immunodeficiency syndrome. In: Goetz GG,
retinal toxicity that mainly affects the Pappert EJ, editors. Textbook of clinical
ganglion cells. Patients usually have neurology. Philadelphia: WB Saunders
metabolic acidosis, but Kussmaul respiration Company, 1999.
is uncommon because of the respiratory _____________________________
depression caused by the intoxication.
Fomepizole is an inhibitor of alcohol Question(s) 381: Behavioral
dehydrogenase that appears to be safe and Discussion:
effective in managing methanol poisoning. The history is suggestive of early
Alzheimer's disease. The MMSE is not a
very sensitive test for making a diagnosis of
Reference: probable Alzheimer's disease in highly
Brent J, McMartin K, Phillips S, et al. intelligent individuals. Patients should be
Fomepizole for the treatment of methanol started early on cholinesterase inhibitors
poisoning. NEJM 2001;344:424-429. and vitamin E.
_____________________________
Question(s) 379: Clinical Pediatrics
Reference:
Discussion: Hake AM. The treatment of Alzheimer’s
Infantile neuroaxonal dystrophy is an disease: the approach from a clinical
inherited neurodegenerative disorder specialist in the trenches. In: Pascuzzi RM,
characterized by psychomotor regression, Roos KL. Therapy in neurology expert
usually beginning in infancy or early clinicians’ views. Seminars in Neurology
childhood, with progression to dementia, 2002;22:71-74.
blindness (associated with optic atrophy) _____________________________
and tetraplegia. Axonal neuropathy is
characteristic, as is the presence of axonal Question(s) 382: Pathology
spheroids in peripheral nerve and brain. MRI Discussion:
shows cerebellar atrophy, with signal The sections demonstrate numerous
hypointensity in the pallida and nigra. enlarged, round axonal swellings which stain
positively with the silver stain. This is the
histologic picture of axonal spheroids, the
Reference: finding in diffuse axonal injury. Also known
Nardocci N, Zorzi G, Farina L. Infantile as "shear injury," patients with this condition
neuroaxonal dystrophy: clinical spectrum are rendered immediately unconscious with
and diagnostic criteria. Neurology trauma, and subsequently have a course of
1999;52:1472-1482 chronic "closed head injury."
_____________________________
Question(s) 380: Clinical Adult
Reference:
Discussion: Ellison D, Love S. Neuropathology: a
Primary central nervous system lymphoma reference text of CNS pathology. Chicago:
in AIDS patients is highly associated with CV Mosby, Inc., 1998.
positive PCR for EBV-DNA in the CSF. _____________________________
Progressive Multifocal
Leukoencephalopathy (PML) is associated
with positive PCR for JC virus DNA in the
CSF. Thallium SPECT scan is positive in
CNS Lymphoma. Increased 14-3-3 protein
in the CSF is a feature of Creutzfeldt-Jakob
disease. PML is visible on CT scans and
MRIs as a non-enhancing brain lesion with

55
 2003 AAN RITE Discussion & Reference Manual

Question(s) 383: Neuroimaging Question(s) 386: Neuroimaging

Discussion: Discussion:
The tumor is located in the region of the The MRI demonstrates posterior frontal and
sella turcica, ruling out glioblastoma, superior temporal atrophy. The anterior
ependymoma and schwannoma. frontal lobes are without significant atrophy.
Meningiomas seldom are this symmetrical. The medial temporal temporal lobes are
intact, a common area for atrophy in
Alzheimer's dementia. There is no evidence
Reference: of vascular insults or normal pressure
Stark DD, Bradley WG. Magnetic resonance hydrocephalus. The personality change is
imaging. St. Louis: C.V. Mosby, 1988. also more indicative of frontal temporal
_____________________________ dementia than Alzheimer's.
Question(s) 384: Neuroimaging
Discussion: Reference:
Bony erosion and opacification of the Greenberg JO. Neuroimaging: a companion
mastoid air cells is seen on the right. The to Adams and Victor's principles of
erosion is adjacent to the proximal aspect of neurology. New York: McGraw-Hill, Co.,
the styloid process, which marks the 1999.
stylomastoid foramen, exit for cranial nerve _____________________________
VII from the skull base. Thus, CN VII is the
most likely involved. Erosion is also Question(s) 387: Pathology
immediately adjacent to the jugular foramen, Discussion:
which carries CN IX, X, and XI. The The photo shows a well-demarcated mass
hypoglossal canal, which carries CN XII is at the cerebellopontine angle. The most
located more medial to the erosion and common tumor at this site is a schwannoma
much less likely involved. CN III, IV and VI of the vestibular branch of the 8th cranial
pass through the cavernous sinus and are nerve.
not near the imaged area.
Reference:
Reference: Burger PC, Scheithauer BW, Vogel FS.
Osborn AG. Diagnostic neuroradiology. St. Surgical pathology of the nervous system
Louis: CV Mosby, Inc., 1994. and its coverings. 4th ed. New York:
_____________________________ Churchill Livingston, 2002.
Question(s) 385: Neuroimaging _____________________________
Discussion: Question(s) 388: Physiology
A large cystic, rim-enhancing mass is Discussion:
present with a more solid component along 14 and 6 positive spikes are sharply
the anterior margin. Porencephaly would not contoured, and occur in the posterior head
enhance, nor would a lipoma. Abscess regions during light sleep. They are best
would be more homogeneously enhancing demonstrated on referential EEG montages,
around the rim, and produce more and are most common in adolescent
prominent hemiparesis. Hemangioblastomas patients.
typically occur in the posterior fossa. The
best answer is cystic astrocytoma.
Reference:
Klass DW, Westmoreland BF.
Reference: Electroencephalography: general principles
Zimmerman RA. Medical imaging of and adult electroencephalograms. In: Daube
pediatric supratentorial tumors. Seminars in JR. Clinical neurophysiology. Philadelphia:
roentgenology 1990;2;225-248. FA Davis Company, 1996.
_____________________________ _____________________________

56
 2003 AAN RITE Discussion & Reference Manual

Question(s) 389: Neuroimaging Question(s) 391: Neuroimaging

Discussion: Discussion:
There is symmetric high signal intensity CT is consistent with subacute subdural
involvement of the putamen and thalami hematoma with a significant mass effect
bilaterally. The globus pallidus is also upon the ventricles. Surgical intervention is
involved but not exclusively as it is in many appropriate.
patients with carbon monoxide poisoning.
The heads of the caudate and nuclei appear Reference:
normal and there is no significant overall
Greenberg JO. Neuroimaging: a companion
atrophy of the brain. These are findings that
to Adams and Victor's principles of
tend to exclude Huntington's, while the high
neurology. New York: McGraw-Hill, Co.,
signal intensity within the globus pallidus
1999.
and putamen is atypical for Parkinson's
_____________________________
disease. Gliomatosis cerebri, an infiltrating
astrocytoma of the white matter is excluded Question(s) 392: Neuroimaging
by the fact that the disease process spares Discussion:
the white matter where the tumor occurs. Noncontrast axial CT reveals a lens shaped
The correct response is Wilson's disease. well localized hyperdense extraaxial lesion
with some surrounding edema. Findings are
Reference: consistent with epidural hematoma. Epidural
hematoma is typically a localized
1. Bakshi R, Lindsay BD, Kinkel PR. Brain
hyperdense lesion because dura is adherent
magnetic resonance imaging in clinical
to the skull. Acute subdural hematoma is
neurology. In: Joynt RJ, Griggs RC, editors.
crescent shaped and more widespread.
Clinical neurology. Philadelphia: Lippincott-
Chronic subdural has low density on CT.
Raven, 1997.
2. DeHaan J, Grossman RI, Civitello L, et al.
High field MRI of Wilson's disease. J Reference:
Comput Tomogr 1987;11:132-135. Greenberg JO. Neuroimaging: a companion
_____________________________ to Adams and Victor's principles of
Question(s) 390: Neuroimaging neurology. New York: McGraw-Hill, Co.,
1999.
Discussion:
_____________________________
The images show a cystic cerebellar
hemispheric mass with an enhancing mural
nodule. T2-weighted images suggest the
presence of small vessels within the mural
nodule as hypointense flow voids. The mass
is intra-axial and infratentorial. The
differential diagnosis includes ganglioglioma,
medulloblastoma, cystic astrocytoma, and
abscess. Demyelinating plaques may
present as neoplasms, but this is unusual.
The finding is most consistent with a
cerebellar cystic hemangioblastoma.

Reference:
1. Greenberg JO. Neuroimaging: a
companion to Adams and Victor's principles
of neurology. New York: McGraw-Hill, Co.,
1999.
2. Bakshi R, Glass J, Louis DN, et al.
Magnetic resonance imaging features of
solitary inflammatory brain masses. Journal
of Neuroimaging 1998;8:8-14.

57
 2003 AAN RITE Discussion & Reference Manual

Question(s) 393: Neuroimaging Question(s) 395: Neuroimaging

Discussion: Discussion:
Sagittal T1 and axial T2-weighted MRI show Surgical treatment alone for an AVM this
a hyperintense rounded mass at the large likely carries an inappropriate degree
anterosuperior third ventricle consistent with of morbidity. An intravascular embolization
a colloid cyst. Approximately 60% of colloid procedure could substantially reduce the
cysts exhibit short T1 and variable blood flow within the AVM, so that the other
shortening of T2 relaxation times. This MR interventions such as surgical excision or
appearance may be related to heavy protein radiation therapy might be used more
or mucin content. The signal of many colloid effectively. The malformation extends into
cysts allows differentiation from most both temporal lobes. Right carotid artery
gliomas (long T1 and T2 relaxation times), ligation will not eliminate filling from the
aneurysms (flow-related signal void) and other hemisphere, or the posterior
meningiomas (isointense to the brain in T1 circulation (both present in this case).
and T2). Third ventricle craniopharyngiomas Surgical therapy followed by radiation fails
are rare. They may exhibit short T1 and long for the same reason mentioned above. Prior
T2 relaxation times. to surgery or embolization, the amytal test
can help evaluate the possible outcomes.
Reference:
1. Czervionke LF, Daniels DL, Meyer GA, et Reference:
al. Neuroepithelial cysts of the lateral Rauch RA, Vinuela F, Dion J, et al.
ventricles: MR appearance. AJNR Preembolization functional evaluation in
1987;8:609-613. brain arteriovenous malformations: the
2. Greenberg JO. Neuroimaging: a superselective Amytal test. AJNR
companion to Adams and Victor's principles 1992;13:303-308.
of neurology. New York: McGraw-Hill, Co., _____________________________
1999.
_____________________________
Question(s) 394: Physiology
Discussion:
Nerve conduction studies with temporal
dispersion, conduction block, and slow
conduction are seen in chronic inflammatory
demyelinating polyneuropathy. In Charcot-
Marie-Tooth disease, there are slow
latencies and nerve conduction velocities,
but no temporal dispersion or block. In
amyotrophic lateral sclerosis, nerve
conduction studies may be normal or with
decreased compound muscle action
potential amplitudes. Nerve conduction
studies are normal in fascioscapulohumeral
dystrophy.

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

58
 2003 AAN RITE Discussion & Reference Manual

Question(s) 396: Neuroimaging Question(s) 398: Neuroimaging

Discussion: Discussion:
Of the options mentioned, to have the CT shows multiple parenchymal
patient breath an oxygen-rich mixture, were calcifications with mild atrophy; this is
the headache to return, is the most characteristic of a host of infections
reasonable one. This patient had his first including toxoplasmosis, rubella,
episode of cluster headache, when an cytomegalovirus, and herpes. Calcification
incidental congenital arachnoid cyst of the seen in Sturge-Weber syndrome is typically
right middle fossa was found on MRI. The cortical and gyriform. Although lipomas may
cyst does not create mass effect and calcify, they are extra-axial, usually single,
therefore neither decompression nor and are hypodense on CT. The scan does
drainage is warranted. Angiography might not show malformations of the sulci or
be a good idea to rule out an aneurysm ventricles to suggest schizencephaly or
causing a subarachnoid hemorrhage, but a colpocephaly.
lumbar puncture should be done before this
procedure. The absence of mass effect
precludes risk with an LP. It was specifically Reference:
mentioned that the relevant findings are Greenberg JO. Neuroimaging: a companion
shown and therefore no aneurysm was seen to Adams and Victor's principles of
on MRI. There is nothing in the history to neurology. New York: McGraw-Hill, Co.,
suggest an internal retinal artery occlusion. 1999.
The right middle cerebral artery is not _____________________________
occluded, but, lifted by the cyst, fades into Question(s) 399: Neuroimaging
the higher section. Discussion:
Note the hyperdense pineal. Normally, the
Reference: pineal is not calcified at age 2. The earliest
Greenberg JO. Neuroimaging: a companion pineal calcifications occur on CT around 6-
to Adams and Victor's principles of 1/2 years of age. The patient has
neurology. New York: McGraw-Hill, Co., calcifications in both orbits that indicate
1999. congenital retinoblastoma. The calcification
_____________________________ in the pineal region indicates that a third
tumor, a pineoblastoma is developing.
Question(s) 397: Neuroimaging
Discussion:
The enhancing, nodular, patchy, diffuse Reference:
abnormalities shown are most characteristic Zimmerman RA, Bilaniuk LT. Age related
of neurosarcoidosis. Toxoplasmosis incidence of pineal calcification detected by
produces granulomas, which may enhance, CT. Radiology 1982;142:659-662.
but these are rarely restricted to the gray _____________________________
matter as in this case. Cerebrotendinous Question(s) 400: Pathology
xanthomatosis is a white matter disease. Discussion:
Hemorrhagic encephalopathy typically In the figure, myelin-stained cross sections
shows areas of both low and high signal of the cervical and lumbar spinal cord show
intensity, and is not uniformly enhancing. degeneration of the corticospinal tracts with
The patient's age and race are also sparing of the posterior columns and
suggestive of neurosarcoidosis. spinocerebellar tracts. These findings are
most compatible with amytrophic lateral
Reference: sclerosis.
Osborn AG. Diagnostic neuroradiology. St.
Louis: CV Mosby, Inc., 1994. Reference:
_____________________________ Graham DI, Lantos PL. Greenfield's
neuropathology. 7th ed. New York: Arnold
Press, 2002.
_____________________________

59
 2003 AAN RITE Discussion & Reference Manual

Question(s) 401: Neuroimaging Reference:

Discussion: Woodruff WW. Fundamentals of
In the sagittal T1-weighted image the normal neuroimaging. Philadelphia: W.B. Saunders
flow void (low signal) is replaced by Company, 1993.
intraluminal high signal related to _____________________________
thrombosed superior sagittal sinus. The Question(s) 404: Physiology
signal intensity of the thrombus over time
Discussion:
has the same evolution pattern as
The illustration shows sleep spindles, which
intracerebral hematomas.
are thought to be generated by the reticular
thalamic nucleus.
Reference:
1. Gomori JM, Grossman RI, Goldberg HI, et Reference:
al. Intracranial hematomas: imaging by high
Shaul N. The fundamental neural
field MR. Radiology 1985;157:87-90.
mechanisms of electroencephalography.
2. Greenberg JO. Neuroimaging: a Electroencephalogr Clin Neurophysiol
companion to Adams and Victor's principles 1998;106:101-107.
of neurology. New York: McGraw-Hill, Co.,
_____________________________
1999.
_____________________________ Question(s) 405: Pathology
Question(s) 402: Neuroimaging Discussion:
The photomicrograph shows a trichinella
Discussion: organism acquired by ingestion of
The correct answer is intracranial undercooked pork.
hypotension secondary to a persistent
cerebrospinal fluid leak, resulting in
prominent, abnormal meningeal Reference:
enhancement. The clinical findings are not Graham DI, Lantos PL. Greenfield's
consistent with acute pyogenic meningitis. neuropathology. 7th ed. New York: Arnold
Tuberculosis meningitis is usually a basilar Press, 2002.
meningitis. _____________________________
Question(s) 406: Neuroimaging
Reference: Discussion:
Bakshi R, Mechtler LL, Kamran S, et al. MRI The correct answer is a Chiari malformation
findings in lumbar puncture headache with syringomyelia, both of which are clearly
syndrome: abnormal dural-meningeal and visible on the image and often associated in
dural venous sinus enhancement. Clinical the same patient. Hydrocephalus is also
Imaging 1999;23:73-76. frequently present with a Chiari
_____________________________ malformation (small posterior fossa, with the
Question(s) 403: Neuroimaging cerebellar tonsils descended across the
foramen magnum), so that aqueductal
Discussion: atresia need not be postulated to explain the
The correct answer is epidural metastasis. enlarged lateral ventricles. The aqueduct is
The bone and especially inner table not seen in this image slightly off midline.
demonstrates bony irregularity and Neither of the other two possible answers is
infiltration of tumor into epidural space. present on the MRI.
Homogenous enhancement signifies
disruption of inner and outer table with
infiltration of tumor (metastatic prostate Reference:
cancer) into extradural space. Epidural Greenberg JO. Neuroimaging: a companion
hematoma, focal pachymeningitis, and to Adams and Victor's principles of
hygroma with hemorrhage into it would neurology. New York: McGraw-Hill, Co.,
present with a smooth medial surface. 1999.
_____________________________

60
 2003 AAN RITE Discussion & Reference Manual

Question(s) 407: Neuroimaging IV hemorrhages is much worse that those

with Grades I and II.
Discussion:
Multiple basal ganglia high signal foci are
seen bilaterally. They are more numerous Reference:
than normal Virchow-Robin spaces. 1. Graham DI, Lantos PL. Greenfield's
Toxoplasmosis resides also in basal ganglia, neuropathology. 7th ed. New York: Arnold
but then there is usually also mass effect. Press, 2002.
Cryptococcus enters the intracranial space 2. Duckett S. Pediatric neuropathology.
through the perivascular spaces and the Baltimore: Williams and Wilkins, 1995.
organisms are surrounded by gelatinous _____________________________
material that is seen as high signal foci. This
correlates with "soap-bubble" abscesses
Question(s) 410: Neuroimaging
seen grossly. The correct answer is Discussion:
cryptococcus infection. Axial T2-weighted images 6 months apart
demonstrates development of abnormal
signal in the globus pallidus and white
Reference: matter disease adjacent to the trigone of the
Osborn AG. Diagnostic neuroradiology. St. right lateral ventricle. Cerebral atrophy and
Louis: CV Mosby, Inc., 1994. enlarged CSF spaces are also seen to
_____________________________ develop. The ventricular and subarachnoid
Question(s) 408: Neuroimaging space each have increased, consistent with
parenchymal tissue loss. The findings are
Discussion: consistent with anoxic injury occurring
The MRA demonstrates no flow in the
shortly after the first scan. The globus
basilar artery. Good flow is noted in the
pallidus is especially vulnerable to anoxic
vertebral arteries bilaterally. Both of the
injury. Differential diagnosis includes chronic
anterior cerebral arteries originate off the left
hypoglycemic injury, carbon monoxide
anterior circulation. The internal and
poisoning, AIDS, Wilson's disease and
common carotid arteries demonstrate good
meningitis. Gliomatosis cerebri would result
flow without a dissection or stenoses.
in multiple T2 hyperintense parenchymal
lesions and mass effect. Krabbe's usually
Reference: presents in infancy. Both Canavan's and
1. Parent A. Carpenter's human Krabbe's are expected to cause widespread
neuroanatomy. 9th ed. Baltimore: Williams & white matter lesions.
Wilkins, 1996.
2. Osborne A. Diagnostic neuroradiology. Reference:
St. Louis: CV Mosby, Inc., 1994. Greenberg JO. Neuroimaging: a companion
_____________________________ to Adams and Victor's principles of
Question(s) 409: Pathology neurology. New York: McGraw-Hill, Co.,
Discussion: 1999.
This germinal matrix hemorrhage is a _____________________________
common complication of premature birth. Question(s) 411: Pathology
The germinal matrix, located in the walls of Discussion:
the lateral ventricles, is a cellular area which The graphic illustrates bilateral, nearly
is quite vascular and delicate. These symmetric, parasagittal, cortical and basal
hemorrhages are graded clinically into four ganglia venous infarctions due to thrombosis
different subtypes: Grade I (localized to the of the superior sagittal sinus and deep
germinal matrix only), Grade II (ruptured into cerebral veins.
the ventricle without ventricular dilatation),
Grade III (ruptured into the ventricle with
ventricular dilatation), and Grade IV Reference:
(ruptured into the ventricular system and Graham DI, Lantos PL. Greenfield's
also into the cerebral parenchyma). Clinical neuropathology. 7th ed. New York: Arnold
outcome in neonates with the Grade III and Press, 2002.

61
 2003 AAN RITE Discussion & Reference Manual

Question(s) 412: Pathology characteristic feature of Alexander's

disease.
Discussion:
Methotrexate neurotoxicity is associated
with either high dosage or intrathecal Reference:
administration, particularly in conjunction Duckett S. Pediatric neuropathology.
with irradiation. It may cause multiple Baltimore: Williams & Wilkins. 1995.
discrete or confluent foci of necrosis in the _____________________________
cerebral or spinal white matter. The lesions
Question(s) 416: Pathology
frequently are periventricular in location.
Discussion:
Formation of onion bulbs results from
Reference: repeated episodes of demyelination and
Ellison D, Love S. Neuropathology: a remyelination. Onion bulbs are especially
reference text of CNS pathology. Chicago: conspicuous in hypertrophic Charcot-Marie-
CV Mosby, Inc., 1998. Tooth disease, Dejerine-Sottas disease and
_____________________________ Refsum disease. In addition, approximately
Question(s) 413: Neuroimaging one half of the cases of chronic
inflammatory demyelinative polyneuropathy
Discussion: show substantial numbers of onion bulbs.
Tethered cord syndrome often presents with
back pain and imaging studies usually
demonstate a thickened filum terminale, Reference:
widening of the spinal canal, posterior cord Graham DI, Lantos PL. Greenfield's
lipoma, and a low lying spinal cord. The neuropathology. 7th ed. New York: Arnold
axial MRI demonstrates good cord caliber at Press, 2002.
L1-L2 where you should be seeing filum _____________________________
terminale only. The lipoma can be seen in
Question(s) 417: Neuroimaging
the proton density sagittal views.
Discussion:
The signal of the deformity producing tissue
Reference: is the same as in cortex, thus this lesion
Osborn AG. Diagnostic neuroradiology. St. represents heterotopic gray matter.
Louis: CV Mosby, Inc., 1994. Neoplasm would have had a high signal.
_____________________________ The lesion is not limited to the basal ganglia,
Question(s) 414: Pathology thus eliminating a hemorrhage. There is no
sign of chronic infarction. The study does
Discussion: not include the hippocampi. Therefore,
The myelin-stained axial section of the brain mesial temporal sclerosis cannot be
stem at the level of the inferior olivary nuclei diagnosed.
shows infarction of a wedge-shaped area of
the dorsolateral portion of the medulla in the
distribution of the posterior inferior cerebellar Reference:
artery. This usually results from occlusion of Barkovich AJ. Pediatric neuroimaging. 2nd
the vertebral artery. ed. New York: Raven Press, 1994.
_____________________________
Reference:
Graham DI, Lantos PL. Greenfield's
neuropathology. 7th ed. New York: Arnold
Press, 2002.
_____________________________
Question(s) 415: Pathology
Discussion:
The photomicrograph shows numerous
Rosenthal fibers clustered most densely
around blood vessels. They are a

62
 2003 AAN RITE Discussion & Reference Manual

Question(s) 418: Pathology 1989;10:977.

2. Greenberg JO. Neuroimaging: a
Discussion: companion to Adams and Victor's principles
The photomicrograph shows acutely
of neurology. New York: McGraw-Hill, Co.,
branching septate hyphae which most likely
1999.
represent an opportunistic Aspergillosis
_____________________________
infection. The organism initially infects the
lung and then spreads hematogenously to Question(s) 421: Pathology
involve other organs. The brain is second Discussion:
only to the lung as a site of visceral Degeneration restricted to the dorsal
involvement, and frequently manifests as columns is characteristic of tabes dorsalis.
hemorrhagic infarctions, as shown.

Reference:
Reference: Graham DI, Lantos PL. Greenfield's
Graham DI, Lantos PL. Greenfield's neuropathology. 7th ed. New York: Arnold
neuropathology. 7th ed. New York: Arnold Press, 2002.
Press, 2002. _____________________________
_____________________________
Question(s) 422: Neuroimaging
Question(s) 419: Neuroimaging Discussion:
Discussion: The scan shows Canavan's disease. The
The scan shows typical findings of Dandy- abnormality is the high signal intensity in the
Walker malformation, with absent vermis, white matter that goes from the
large posterior fossa cyst and absent periventricular region to the cortex in a
posterior corpus callosum. diffuse fashion, involving all of the white
matter without sparing subcortical U-fibers.
Reference: Periventricular leukomalacia is in the
periventricular region in the frontal parietal
Barkovich AJ, Maroldo TV. Magnetic
region. Metachromatic leukodystrophy does
resonance imaging of normal and abnormal
not extend out into the subcortical U-fibers,
brain development. Top Magn Reson
while adrenoleukodystrophy favors the
Imaging 1993:5;96-122.
occipital and parietal regions sparing the
_____________________________ frontal lobes in all but a small percentage of
Question(s) 420: Neuroimaging cases. Adrenoleukodystrophy also tends to
Discussion: spare the subcortical U-fibers. Krabbe's
The axial T2-weighted image shows large disease has significant atrophy.
cerebrospinal fluid collection expanding the
posterior fossa with absence of the inferior Reference:
vermis and hypoplasia of the two cerebellar
1. Barkovich AJ. Pediatric neuroimaging.
hemispheres. In an arachnoid cyst, the
New York: Raven Press, 1990.
cerebellar hemispheres should be relatively
2. Greenberg JO. Neuroimaging: a
well preserved as to size and the inferior
companion to Adams and Victor's principles
vermis would not be absent. Cystic
of neurology. New York: McGraw-Hill, Co.,
astrocytoma is not a consideration as the
1999.
fluid is extra-axial. A giant cisterna magna
would not be associated with both vermian _____________________________
and bilateral cerebellar hypoplasia.
Schizencephaly (a pial ependymal cleft seen
in the supratentorial space) is not present.

Reference:
1. Barkovich AJ, Kjos BO, Norman D, et al.
Revised classification of posterior fossa
cysts and cyst-like malformations based on
the results of multiplanar MR imaging. AJNR

63
 2003 AAN RITE Discussion & Reference Manual

Question(s) 423: Pathology Question(s) 426: Pathology

Discussion: Discussion:
Colloid cyst of the third ventricle is a round The graphic shows a circumscribed
circumscribed grape-like lesion occurring in collection of purulent material (empyema) on
the third ventricle where it may produce the surface of the brain (i.e., subdural as
position-dependent CSF obstruction due to opposed to epidural or subarachnoid).
ball-valving. Acute lethal hydrocephalus may Subdural empyema may complicate head
occur leading to sudden death. injury or sinus infections.

Reference: Reference:
Burger PC, Scheithauer BW, Vogel FS. Graham DI, Lantos PL. Greenfield's
Surgical pathology of the nervous system neuropathology. 7th ed. New York: Arnold
and its coverings. 4th ed. New York: Press, 2002.
Churchill Livingston, 2002. _____________________________
_____________________________ Question(s) 427: Anatomy
Question(s) 424: Pathology Discussion:
Discussion: The arrow is pointing to the head of the
In this picture, the collections of multiple caudate nucleus. Lesions of this structure
bubbly cysts in the white matter, caudate are associated with chorea. A lesion of the
and putamen are typical of parenchymal subthalamic nucleus leads to hemiballismus.
involvement by cryptococcal meningitis. Lesions of the substantia nigra result in
Aspergillosis produces gray irregular parkinsonism. Hemiparesis results from
necrotic masses. Tuberculoma and interruption of the upper motor neurons
metastases tend to be discrete solid firm anywhere in their path above the pons.
masses.
Reference:
Reference: Benarroch EE, Westmoreland BF, Daube
Graham DI, Lantos PL. Greenfield's JR, et al. Medical neurosciences - an
neuropathology. 7th ed. New York: Arnold approach to anatomy, pathology and
Press, 2002. physiology by systems and levels. 4th ed.
_____________________________ New York: Lippincott, Williams & Wilkins,
Question(s) 425: Neuroimaging 1999.
_____________________________
Discussion:
The patient presented here has multiple Question(s) 428: Pathology
neurofibromas of spinal nerves, and a Discussion:
plexiform neurofibroma infiltrating the nerves The atrophic cerebellar vermis and loss of
forming the left brachial plexus. Although both Purkinje and granule cell neurons
multiple spinal tumors can be seen in both indicate a degeneration of neurons seen in
NF-1 (pathology is neurofibroma) and in NF- alcoholic cerebellar degeneration. No tumor
2 (pathology is schwannoma), plexiform or volume-expanding lesion is present.
neurofibromas are not seen in NF-2. The Dandy Walker causes agenesis of the
remaining diagnoses are not consistent with cerebellar vermis with formation of a cyst.
the images. Methanol intoxication affects deep white
matter and putamen, not cerebellar vermis.
Reference:
Osborn AG. Diagnostic neuroradiology. St. Reference:
Louis: CV Mosby, Inc., 1994. Graham DI, Lantos PL. Greenfield's
_____________________________ neuropathology. 7th ed. New York: Arnold
Press, 2002.
_____________________________

64
 2003 AAN RITE Discussion & Reference Manual

Question(s) 429: Neuroimaging 1999.

3. Osborne AG. Diagnostic neuroradiology.
Discussion: St. Louis: CV Mosby, Inc., 1994.
T1-weighted MRI shows a mixed signal
_____________________________
destructive mass involving the clivus and
nasopharynx extending to the spinal canal. Question(s) 431: Neuroimaging
The lesion is consistent with a clivus Discussion:
chordoma. The ventral pontine surface is There is a hypodense, CSF density,
compressed. Primary tumors, such as collection that lies between the two leaves of
chordomas and cartilaginous tumors of the the septum pellucidum, separating the
skull base are rare. Chordomas arise from lateral ventricles to each side of the midline.
remnants of the embryonic notochord which The finding is consistent with a cavum
is a mesodermal derivative. Cranial septum pellucidum and cavum vergae. A
chordomas are most common in the third colloid cyst would occur only in the region of
and fourth decades of life and males are the foramen of Monro and be slightly to
affected more often than females. markedly hyperdense; a craniopharyngioma
Cartilaginous tumors occur between 20 and would not lie between the leaves of the
60 years of age. They are extradural and septum pellucidum, but would displace the
over half arise in or adjacent to the body of ventricle around the mass of the
the sphenoid bone. Radiologically the craniopharyngioma, and would likely have
normal, high signal marrow cavity of the calcification in its wall. An intraventricular
clivus is replaced by lower signal intensity meningioma would lie adjacent to the
tumor. Large areas of calcification may be choroid plexus of the lateral ventricle or that
seen as void-phenomenon. Radiographically within the third ventricle, would be
the distinction between chordomas, hyperdense in a significant portion of its
chondrosarcomas and chondromas may be mass. An arachnoid cyst would lie external
impossible. Meningiomas are isointense to to the ventricular system.
the brain before contrast. This is an extra
pontine lesion; the brainstem is not involved.
The pituitary gland is normal. Reference:
Parapharyngeal abscesses are usually 1. Rao K, Harwood-Nash D. Craniocerebral
smoothly contoured. anomalies. In: Lee SH, Rao K, editors.
Cranial computed tomography and MRI.
New York: McGraw-Hill, 1987.
Reference: 2. Greenberg JO. Neuroimaging: a
1. Ham JS, Huss RG, Benson JE, et al. MRI companion to Adams and Victor's principles
imaging of the skull base. J Comput Assist of neurology. New York: McGraw-Hill, Co.,
Tomogr 1984;8:944-952. 1999.
2. McGinnis BD, Brady TJ, New PF, et al. _____________________________
MR imaging of tumors of the posterior fossa.
J Comput Assist Tomogr 1984;7:575-584.
_____________________________
Question(s) 430: Neuroimaging
Discussion:
Both the location of the lesion and the
presence of precocious puberty favor a
hamartoma in the tuber cinereum.

Reference:
1. Williams AL, Mosby VM. Cranial
computed tomography: a comprehensive
text. St. Louis: C.V. Mosby, 1985.
2. Greenberg JO. Neuroimaging: a
companion to Adams and Victor's principles
of neurology. New York: McGraw-Hill, Co.,

65
 2003 AAN RITE Discussion & Reference Manual

Question(s) 432: Neuroimaging 2. Greenberg JO. Neuroimaging: a

companion to Adams and Victor's principles
Discussion: of neurology. New York: McGraw-Hill, Co.,
The MRIs show mass in the prepontine 1999.
space indenting the pons. The signal
_____________________________
intensity characteristics are those of fat, the
finding being most consistent with a lipoma. Question(s) 435: Anatomy
The bright line posterior to the mass is due Discussion:
to chemical shift artifact, which is usually The structure indicated by the arrow is the
best seen on conventional spin-echo T2- lateral geniculate body.
weighted image.
Reference:
Reference: Haines DE. Fundamental neuroscience. 2nd
1. Truwit CL, Barkovich AJ. Pathogenesis of ed. New York: W.B. Saunders, 2002.
intracranial lipoma: an MR study in 42 _____________________________
patients. AJNR 1990;11:665.
Question(s) 436: Pathology
2. Greenberg JO. Neuroimaging: a
companion to Adams and Victor's principles Discussion:
of neurology. New York: McGraw-Hill, Co., The tumor illustrated is a low grade
1999. oligodendroglioma; the calcifications and
_____________________________ "fried-egg cells" are characteristic of
oligodendroglioma. These tumors typically
Question(s) 433: Neuroimaging present in patients in their 30s or 40s. Due
Discussion: to the high tendency of these tumors to
The extensive enhancement of the invade the overlying cortex, seizures (often
ependyma is most suggestive of ventriculitis. of one to five years duration) are one of the
Choroid plexus papilloma with CSF spread, most frequent clinical presentations. A dural-
ependymoma, transependymal flow of CSF, based mass in a 60-year-old woman is most
and tuberous sclerosis with candle guttering likely to be a meningioma. The prototypical
do not produce this pattern, with the profile of a smoker with a lung mass is
possible exception of transependymal CSF metastatic lung carcinoma and the male with
flow (which is non-enhancing). a posterior fossa mass is more likely to be a
Enhancement of the choroid plexus is patient with medulloblastoma.
normal and does not suggest a papilloma.
Candle guttering has the appearance of
enhancing, strings or beads that tend to Reference:
protrude into the ventricles. Burger PC, Scheithauer BW, Vogel FS.
Surgical pathology of the nervous system
and its coverings. 4th ed. New York:
Reference: Churchill Livingston, 2002.
Osborn AG. Diagnostic neuroradiology. St. _____________________________
Louis: CV Mosby, Inc., 1994.
_____________________________
Question(s) 434: Neuroimaging
Discussion:
MR is effective in demonstrating a displaced
optic chiasm because the optic chiasm is
usually slightly more intense than pituitary
tumors.

Reference:
1. Daniels DL, Haughton VM, Czervionke
LF. MR of the skull base. In: Bradley WG,
Stark D. Magnetic resonance imaging. St.
Louis: CV Mosby, Inc., 1988.

66
 2003 AAN RITE Discussion & Reference Manual

Question(s) 437: Neuroimaging Question(s) 440: Neuroimaging

Discussion: Discussion:
Axial MRI scans demonstrate the high signal Predicting the chances of a good outcome is
intensity lesion which is of low signal notoriously inaccurate, but duration of
intensity in the T1-weighted image. The dementia is one of the more important
lesion is located in the vascular territory of considerations. Depressive symptoms may
the anterior (superior) branches of the improve with shunting, but this may occur
middle cerebral artery. The ability of the MRI whether or not his dementia syndrome
scan to detect ischemic lesions is mainly improves. If his shunt results in a decrease
related to changes in water content of in ventricular size, that is indeed favorable,
developing infarct. Earliest changes are but the duration of dementia is a more
related to cytotoxic edema which is followed critical variable. Persons with chronic NPH
by vasogenic edema. Encephalitis is usually may not respond to the shunt, even if follow-
not restricted to a vascular territory. up CT scans suggest that the hydrocephalus
is improved. The duration of urinary
incontinence reflects the degree of damage
Reference: to the fibers of the medial frontal lobe
Greenberg JO. Neuroimaging: a companion micturition centers. The bilateral Babinski
to Adams and Victor's principles of signs, severe gait abnormality, and
neurology. New York: McGraw-Hill, Co., spasticity all suggest the same type of
1999. damage. The duration of incontinence has
_____________________________ little predictive value. Finally, subcortical
Question(s) 438: Pathology types of dementia are more likely to be
treatable than cortical varieties. This by itself
Discussion: is no guarantee that the shunt will produce
The face demonstrates cyclopia with a fused clinical improvement in the dementia
single eyeball and a superior proboscis. syndrome.
Cyclopia occurs with midline cleavage
defects and clefting of the lip or the palate is
usually also present. Alobar Reference:
holoprosencephaly is also part of this Cummings JL, Benson DF. Dementia: a
spectrum of midline clevage abnormalities clinical approach. Boston: Butterworth-
with a single, globular hemisphere, a single Heinemann, 1992.
ventricle and fused basal ganglia. _____________________________

Reference:
Duckett S. Pediatric neuropathology.
Baltimore: Williams & Wilkins, 1995.
_____________________________
Question(s) 439: Neuroimaging
Discussion:
The diffusion and ADC images give clear
evidence of an acute stroke. The
hyperintensity on the diffusion with
corresponding hypointensity on the ADC
map are shown. These findings would not
be present on the other choices.

Reference:
Bakshi R, Ketonen L. Brain MRI in clinical
neurology. In: Joynt RJ, Griggs RC, editors.
Baker’s clinical neurology. Philadelphia:
Lippincott, Williams & Wilkins, 2001.
_____________________________

67
 2003 AAN RITE Discussion & Reference Manual

Question(s) 441: Pathology parietal-occipital lobes. Calcification

secondary to hyperparathyroidism occurs in
Discussion: a symmetric fashion in the basal ganglia,
The tissue section from the resected dentate nuclei of the cerebellum, and
intraventricular mass showed large cells with periventricular white matter. The
abundant eosinophilic cytoplasm. Many also calcifications in tuberous sclerosis occur in
had prominent single nucleoli. Although the subependymal tubers lining the
these features closely resemble those of a ventricles.
number of primary central nervous system
tumors, including gemistocytic astrocytoma
and ganglion cell tumor, the intraventricular Reference:
location eliminates these entities, which are 1. Atlas S. Magnetic resonance imaging of
intraparenchymal tumors, and points to the brain and spine. New York: Raven
subependymal giant cell astrocytoma as the Press, 1991.
correct diagnosis. Of the remaining choices 2. Bakshi R, Lindsay BD, Kinkel PR. Brain
listed, the differential diagnosis of an magnetic resonance imaging in clinical
intraventricular tumor would include neurology. In: Joynt RJ, Griggs RC, editors.
subependymoma and choroid plexus Clinical neurology. Philadelphia: Lippincott,
papilloma; however, the tumor in the present Williams & Wilkins, 1998.
case does not display the papillary _____________________________
architecture of choroid plexus papilloma or
the multilobulation and abundant fibrillar
Question(s) 443: Pathology
matrix of subependymoma. Subependymal Discussion:
giant cell astrocytomas are commonly The biopsy of this cystic mass showed
associated with tuberous sclerosis and may layers of flattened, anucleate squames. This
be the presenting clinical feature, as in the type of “flaky” keratin is characteristic of
present case. epidermoid and dermoid cysts. In contrast,
the keratin formed by adamantinomatous
craniopharyngiomas typically consists of
Reference: nodular clusters of very plump keratinocytes
Burger PC, Scheithauer BW, Vogel FS. referred to as “wet” keratin. The lining of
Surgical pathology of the nervous system Rathke cleft cysts, colloid cysts, and
and its coverings. 4th ed. New York: neurenteric cysts consists of ciliated
Churchill Livingston, 2002. pseudostratified columnar epithelium with
_____________________________ scattered goblet cells.
Question(s) 442: Neuroimaging
Discussion: Reference:
Multiple calcified lesions are seen the brain Burger PC, Scheithauer BW, Vogel FS.
parenchyma, involving both cerebral Surgical pathology of the nervous system
hemispheres and the cerebellum, primarily and its coverings. 4th ed. New York:
at the gray-white junction. This is a typical Churchill Livingston, 2002.
appearance and location for healed _____________________________
toxoplasmosis lesions, in this case seen in a
patient with AIDS. Additional CT
characteristics of HIV encephalopathy are
also seen in this patient, including
generalized atrophy and diffuse, confluent
low-density of the white matter. Sarcoidosis
can be manifested on imaging studies as
either a diffuse meningeal process or
multiple parenchymal lesion; however,
calcification is not a feature. Sturge-Weber
syndrome results in intracranial calcification
secondary to pial angiomatosis, with
curvilinear calcification following the contour
of the cortex, typically in the occipital or

68
 2003 AAN RITE Discussion & Reference Manual

Question(s) 444: Pathology Reference:

Discussion: Orrison WW. Neuroimaging. Philadelphia:
WB Saunders, 2000.
Neuritic plaques consist of an amyloid core
and dystrophic neurites, with reactive _____________________________
astrocytes and microglia. They are a classic Question(s) 447: Neuroimaging
finding in Alzheimer's disease; in the Discussion:
commonly used CERAD grading system, The sagittal T1-weighted image shows a
neuritic plaque counts are used as histologic small pons and cerebellar atrophy. The axial
criteria for making the diagnosis of T2-weighted image through the pons shows
Alzheimer's disease. the pons to be reduced in size. The axial
image through the medulla and lower
Reference: cerebellum show that the cerebellum is of
Graham DI, Lantos PL. Greenfield's high signal intensity relative to the more
neuropathology. 7th ed. New York: Arnold posteriorly placed occipital lobes, and that
Press, 2002. the medullary olives are not normal in size.
Thus the findings are that of cerebellar,
_____________________________
pontine and olivary atrophy. There is no
Question(s) 445: Pathology intrinsic high signal intensity lesion within the
Discussion: substance of the pons so that an infarct is
The photo shows a poorly circumscribed not present. The hallmark of
variegated mass with foci of necrosis and olivopontocerebellar atrophy is loss of the
hemorrhage most consistent with belly of pons, which is also clearly affected.
glioblastoma multiforme. The lack of central Friedreich's ataxia is usually primarily seen
purulent material is against an abscess. The with spinal atrophy.
lesion is not confined within a single
vascular territory, nor is it hemorrhagic,
Reference:
arguing against embolic infarct. The intra-
1. Nabatame H, Fukuyama H, Akiguchi I, et
axial location argues against most
al. Spinocerebellar degeneration: qualitative
meningiomas. The lesion is a mass, ruling
and quantitative MR analysis of atrophy.
out Huntington's disease.
JCAT 1988;12:298.
2. Bakshi R, Lindsay BD, Kinkel PR. Brain
Reference: magnetic resonance imaging in clinical
Burger PC, Scheithauer BW, Vogel FS. neurology. In: Joynt RJ, Griggs RC, editors.
Surgical pathology of the nervous system Clinical neurology. Philadelphia: Lippincott,
and its coverings. 4th ed. New York: Williams & Wilkins, 1998.
Churchill Livingston, 2002. _____________________________
_____________________________ Question(s) 448: Pathology
Question(s) 446: Neuroimaging Discussion:
Discussion: The graphic shows severe pontine atrophy
Combining the history and the image, a and would be most likely seen in
Tolosa-Hunt syndrome is the most likely olivopontocerebellar atrophy (OPCA). OPCA
diagnosis. A meningioma "en plaque" would is part of multiple system atrophy. Glia
not have caused a similar syndrome two (especially oligodendroglia) in multiple
years previously in the contralateral eye system atrophy exhibit cytoplasmic, flame-
leaving no trace. Myasthenia and the Miller- shaped, silver-positive inclusions. The
Fisher syndrome do not show on MRI the inclusions are alpha-synuclein
enhancing lesion in right cavernous sinus immunoreactive.
and meninges present in this case. A
pseudotumor of the orbit sometime extends
into the retro-orbital meninges, but in this
Reference:
case there is no mass effect in the intraconal Graham DI, Lantos PL. Greenfield's
fat and the process clearly extends much neuropathology. 7th ed. New York: Arnold
beyond the orbit. Press, 2002.
_____________________________

69
 2003 AAN RITE Discussion & Reference Manual

Question(s) 449: Neuroimaging vermis. Susceptibility effect with T2

Discussion: shortening and hypointensity is due to
The scans show schizencephaly with septo- hemosiderin from prior repeated
optic dysplasia. Porencephalic cyst would be subarachnoid hemorrhage. The etiology of
lined with white matter, whereas the cavity in the hemorrhagic process was a cervical
schizencephaly is lined by heterotopic grey neoplasm.
matter. Lissencephaly refers to brains with
absent or extremely poor sulcation. Reference:
Holoprosencephaly results from the failure Offenbacher H, Fazekas F, Schmidt R, et al.
of lateral cleavage into distinct cerebral Superficial siderosis of the central nervous
hemispheres and failure of transverse system: MRI findings and clinical
cleavage into diencephalon and significance. Neuroradiology 1996;38:S51-
telencephalon. S56.
_____________________________
Reference: Question(s) 452: Pathology
Osborn AG. Diagnostic neuroradiology. St. Discussion:
Louis: CV Mosby, Inc., 1994. Centronuclear myopathy is characterized by
_____________________________ the presence of centrally located nuclei,
Question(s) 450: Neuroimaging often accompanied by a perinuclear
Discussion: vacuole. The muscle usually shows type I
The sagittal MR images post-gadolinium myofiber predominance.
injection show multiple round enhancing
lesions that are on the surface of the Reference:
cervical spinal cord. Elongated irregular Graham DI, Lantos PL. Greenfield's
plaques of tumor enhance along the dorsal neuropathology. 7th ed. New York: Arnold
aspect of the upper thoracic cord. The Press, 2002.
findings are consistent with tumor seeding. _____________________________
Post-operative changes are present in the
posterior fossa. Astrocytoma of the cervical Question(s) 453: Anatomy
cord would expand the cord and enhance Discussion:
more homogeneously throughout. The arrow is pointing to the substantia nigra
Syringohydromyelia would be a cavity within which normally functions to faciliatate
the cord that expands it. Cord contusions voluntary motor activity originating in the
would have high signal intensity within the prefrontal and motor cortex ipsilateral to the
substance of the cord. Arachnoiditis can substantia nigra. Damage to one substantia
enhance, but does not have a nodular nigra results in hemiparkinsonism. Since the
appearance as in this case. upper motor neurons of the motor strip are
the motor output elements and these cross
at the medullary decussation, the
Reference: hemiparkinsonism resulting from a unilateral
1. Sze G, Abramson A, Krol G, et al. substantia nigra lesion is contralateral to the
Gadolinium-DTPA in the evaluation of lesion.
intradural extramedullary spinal disease.
AJNR 1988;9:153.
2. Greenberg JO. Neuroimaging: a Reference:
companion to Adams and Victor's principles Benarroch EE, Westmoreland BF, Daube
of neurology. New York: McGraw-Hill, Co., JR, et al. Medical neurosciences - an
1999. approach to anatomy, pathology and
_____________________________ physiology by systems and levels. 4th ed.
New York: Lippincott, Williams & Wilkins,
Question(s) 451: Neuroimaging
1999.
Discussion: _____________________________
T2-weighted images show hypointense
signal changes on the pial surface of the
brain stem, temporal lobes and cerebellar

70
 2003 AAN RITE Discussion & Reference Manual

Question(s) 454: Anatomy structures. The ring enhancement in brain

abscess is fairly uniform in thickness and the
Discussion: ring enhancement appears to be under
The photomicrograph shows a coronal tension. Herpes encephalitis usually
section of the corpus callosum and cingulate originates in temporal lobes. Major brain
gyri. The arrows identify the indusium infarction usually also involves the gray
griseum. The indusium (also called the matter, which is spared in this patient.
supracallosal gyrus) is a direct continuation
of the hippocampal formation located dorsal
to the corpus callosum. Reference:
1. Kelly WM, Brant Zawadski M. Magnetic
resonance imaging and computed
Reference: tomography of supratentorial tumors. In:
Parent A. Carpenter's human Radiology: diagnosis-imaging intervention.
neuroanatomy. 9th ed. Baltimore: Williams & Philadelphia: J.B. Lippincott Co., 1986.
Wilkins, 1996.
2. Greenberg JO. Neuroimaging: a
_____________________________ companion to Adams and Victor's principles
Question(s) 455: Neuroimaging of neurology. New York: McGraw-Hill, Co.,
Discussion: 1999.
The correct response is thrombosed _____________________________
aneurysm. Arachnoid cyst would be filled Question(s) 457: Pathology
with cerebrospinal fluid-like signal on all
Discussion:
sequences. Hamartomas of tuber cinereum
Herniation of the cerebellar vermis and
are isointense with grey matter on T1
medulla, with kinking of the upper cervical
sequences and hyperintense on T2. A
cord and beaking of the quadrigeminal plate
lipoma would also show hyperintensity on
are components of the Arnold-Chiari
T1 and could occur in this location, however,
malformation.
they are usually associated with chemical
shift artifact on conventional spin-echo T2.
This thrombosed aneurysm contains blood Reference:
breakdown products in the early subacute Graham DI, Lantos PL. Greenfield's
stage. neuropathology. 7th ed. New York: Arnold
Press, 2002.
Reference: _____________________________
Bakshi R, Lindsay BD, Kinkel PR. Brain Question(s) 458: Neuroimaging
magnetic resonance imaging in clinical Discussion:
neurology. In: Joynt RJ, Griggs RC, editors. MR angiogram demonstrating good flow in
Clinical neurology. Philadelphia: Lippincott, all major vessels. An anatomical variant of
Williams & Wilkins, 1998. both anterior cerebral arteries originating
_____________________________ from the left internal carotid artery is shown.
Question(s) 456: Neuroimaging In addition, the right posterior cerebral artery
originates from the anterior circulation (fetal
Discussion: origin). There are no aneurysms or stenoses
The contrast enhanced axial CT
evident.
demonstrates ill-defined ring enhancement
with significant edema. Findings are typical
for malignant brain neoplasm. The axial Reference:
proton density MR image demonstrates an Osborn AG. Diagnostic neuroradiology. St.
ill-defined mixed signal lesion in the right Louis: CV Mosby, Inc., 1994.
posterior parietal region. Intratumoral _____________________________
hyperintense necrotic foci are seen in
addition to hyperintense edema. Mass effect
upon the right lateral ventricle is seen. Some
tubular signal void regions are seen, they
are most likely consistent with vascular

71
 2003 AAN RITE Discussion & Reference Manual

Question(s) 459: Pathology The appearance of intramedullary

coccidiomycosis is different and in general,
Discussion: infections tend to affect the intervertebral
The illustration shows an eosinophilic discs, relatively spared by this tumor.
intracytoplasmic inclusion in a cortical
neuron. This is a Negri body and is
diagnostic of rabies encephalitis. Reference:
Greenberg JO. Neuroimaging: a companion
to Adams and Victor's principles of
Reference: neurology. New York: McGraw-Hill, Co.,
Graham DI, Lantos PL. Greenfield's 1999.
neuropathology. 7th ed. New York: Arnold
_____________________________
Press, 2002.
_____________________________ Question(s) 462: Pathology
Question(s) 460: Neuroimaging Discussion:
The picture shows marked caudate atrophy
Discussion: diagnostic of Huntington disease, which
The most likely diagnosis is Leigh's disease. results from a trinucleotide repeat
The abnormal areas are seen as high signal amplification mutation in the huntingtin gene
intensity within the brain stem and basal residing on chromosome 4.
ganglia. They are symmetric. Such
abnormalities are found in patients with
metabolic acidosis and elevated lactate Reference:
including Leigh's disease. Herpes usually Graham DI, Lantos PL. Greenfield's
involves the medial temporal lobe, insular neuropathology. 7th ed. New York: Arnold
cortex, and inferior frontal lobes, areas that Press, 2002.
are not involved in this case. There is also _____________________________
no brain swelling or mass effect, findings
Question(s) 463: Neuroimaging
that are common in herpes. Carbon
monoxide poisoning involves principally the Discussion:
globus pallidus and does not involve the Noncontrast CT reveals a nearly isointense
brain stem. Infarctions in sickle cell disease extra-axial mass in left parietal area. The
do not have such symmetry and are enhancement is homogeneous and intense
uncommon in the brainstem. consistent with meningioma.

Reference: Reference:
1. Geyer CA, Sartor KJ, Prensky AJ, et al. 1. Yock DH. Imaging of CNS disease: a CT
Leigh's disease: CT & MR in five cases. J and MR teaching file. Baltimore: Mosby,
Comput Assist Tomogr 1988;12:40-44. 1991.
2. Osborne AG. Diagnostic neuroradiology. 2. Greenberg JO. Neuroimaging: a
St. Louis: CV Mosby, Inc., 1994. companion to Adams and Victor's principles
_____________________________ of neurology. New York: McGraw-Hill, Co.,
1999.
Question(s) 461: Neuroimaging _____________________________
Discussion:
Ependymoma is the most likely diagnosis,
confirmed here by surgery. The tumor arises
from the spinal canal and molds the
vertebral bodies. A chordoma would arise
from the vertebral bodies, or more likely,
from the sacrum, and compress the spinal
canal. Although at the sacral level it may be
difficult to appreciate the origin of the tumor,
it clearly appears intramedullary in higher
sections. Similar arguments could be made
against the diagnosis of osteochondroma.

72
 2003 AAN RITE Discussion & Reference Manual

Question(s) 464: Neuroimaging Question(s) 475 - 479: Neuroimaging

Discussion: Discussion:
Common carotid artery injection (arterial Straightforward identification of cervical and
phase, lateral view) reveals a flame-shaped cerebral vessels.
"stump" of internal carotid artery consistent
with dissection. The external carotid artery
fills with contrast. The internal carotid artery Reference:
(ICA) is relatively unprotected in its cervical 1. Netter FH. The CIBA collection of medical
course and therefore vulnerable to trauma. illustrations: nervous system. Volume 1, Part
Trauma to ICA may result in thrombosis, 1. New York: Donnelley & Sons Company,
intimal tears, stenosis, arteriovenous 1986.
fistulas, pseudoaneurysms or dissection. 2. Parent A. Carpenter's human
neuroanatomy. 9th ed. Baltimore: Williams &
Wilkins, 1996.
Reference: _____________________________
1. Stringer WL, Kelly DL Jr. Traumatic
Question(s) 480: Anatomy
dissection of the extracranial internal carotid
artery. Neurosurg 1980;6:123-130. Discussion:
2. Greenberg JO. Neuroimaging: a The visual field defect is a quadrantic
companion to Adams and Victor's principles sectoranopia and localization is the lateral
of neurology. New York: McGraw-Hill, Co., geniculate nucleus of the thalamus. The
1999. anterior choroidal artery is the main artery to
_____________________________ the lateral geniculate.
Question(s) 465 - 469: Neuroimaging
Discussion: Reference:
MR midline sagittal images show signal void 1. Parent A. Carpenter's human
due to flowing blood in the internal cerebral neuroanatomy. Baltimore: Williams &
vein. Thin soft tissue forming the roof of the Wilkins, 1996.
cranial aspect of the fourth ventricle 2. Brazis PW, Masdeu JC, Biller J.
represents the superior medullary velum. Localization in clinical neurology. Boston:
Little, Brown & Co., 1996.
_____________________________
Reference:
Daniels DL, Haughton VM, Naidich T.
Cranial and spinal magnetic resonance
imaging: atlas and guide. New York: Raven
Press, 1987.
_____________________________
Question(s) 470 - 474: Neuroimaging
Discussion:
Axial T2-weighted image through suprasellar
cistern. Normal anatomy.

Reference:
Hanaway J, Woolsey TA, Gado MH, et al.
The brain atlas. Baltimore: Fitzgerald
Science Press, 1998.
_____________________________

73
AMERICAN ACADEMY OF NEUROLOGY
Executive Office
1080 Montreal Avenue
St. Paul, MN 55116
Direct RITE questions to:
Phone: 651-695-2706
Fax: 651-361-4806
E-mail: lwiener@[Link]