CNS notes

CNS
Notes
By: Dr. Abdullah Nouh

1
 CNS notes
Anatomy
A) ST Anatomy:
1. Dilated Perivascular Spaces (Virchow-Robins):
• Fluid filled spaces that accompany perforating vessels
• Normal variant
• Contain interstitial fluid (Not CSF)
o Suppress FLAIR, Not restrict DW
• Normal surrounding brain tissue (DD from infarction)
• Ass.with: -
- Mucopolysaccharidoses (Hurlers &Hunters)
- ‘Gelatinous pseudocysts” in cryptococcal meningitis
- Atrophy with age
• Sites: -
o Ant commissure
o Lower 1/3 BG (Lenticulostriate arteries)
o Centrum semiovale
o MB (Gaint)
B) Bony Anatomy
Skull Base:
Foramen Contents
Foramen Ovale O = otic ganglion Pain over jaw & chin
V = V3 Atrophy of muscles of mastication
A = Acc mening A
L = Lesser petrosal N
E = Emissary veins
Foramen Rotundum V2 ("R2V2")
Sup Orbital Fissure V1, III, IV, VI
Inf. Orbital Fissure V2
Foramen Spinosum Middle Mening A
Jug Foramen IX, X, XI & Jug V Vernet $:
Loss of taste to pos 1/3 tongue
Vocal cord paralysis
Dysphasia
Uvula deviate to same side
Weakness of SCM & trapezius
Hypoglossal Canal XII Tongue weakness
Difficulty moaving food to back of throat
Hemiatrophy tongue
Deviation on protrusion
Optic Canal II &Opth A
Cavernous Sinus
• CN3, 4, CN VI, CNV2,CN6, & carotid.
• CN2 &CN V3do NOT run through it.
• CN6 runs next to carotid, therestare along wall (so lateral rectus palsyisearlier than cavernous sinus pathologies).
Misc Brain Conditions
Monro-Kellie Doctrine:
• The head is a closed shell with 3 major components: (1) brain, (2) blood (3) CSF
• As volume of one goes up, volume of another must go down.

2
 CNS notes
Degenerative
Degenerative and White Matter Disease
Classification
- WM disease - GM disease
• Demyelinating: acq (destroyed Nl myelin) • Alzheimer dis
▪ Multiple sclerosis (MS) • Frontotemporal dementia
▪ ADEM • Vascular dementia
▪ Toxin related • Dementia with Lewy bodies
- Central pontine myelinolysis • Amyotrophic lat sclerosis (ALS)
- Paraneoplastic $ - BG disorders
- RTx, CTx • Huntington dis
- Alcoholism
• Wilson dis
• Dysmyelinating: cong (errors of myelin
• Fahr dis
synthesis, maintenance, or degradation) see Ped
• Leigh dis
▪ Lysosomal enzyme disorders
▪ Peroxisomal disorders - Toxic/infectious
▪ Mitochondrial disorders • Creutzfeldt-Jakob
▪ Amino acidopathies • CO
▪ Idiopathic • Alcohol/Wernicke
• Seizure medication
WM Disease
Multiple Sclerosis
• Women 20-40Y. In children, no gender difference
• Cl depends on anatomic location
• Relapsing-remitting (85%) “separated by time & space”
o Monocular visual loss, gait difficulties & sensory disturbances
• Lab: evoked potentials, CSF oligoclonal bands
Revised McDonald Criteria
Dissemination in space
- > 1 high T2 lesion
- > 2 of the following areas: periventricular, juxtacortical, infratentorial, spinal cord
Dissemination in time
- New T2 or enhancing lesion on FU MRI. OR
- Simultaneous presence of enhancing & non-enhancing lesions
Findings:
MRI plaques:
- Multiple #
- 0.5–3 cm
- Active: high T2, DW & enhance: homogeneous, ring-like, or patchy. Inactive: not enhance
- Oblong, elliptical in calloso-septal interface… Ependymal “dot-dash” sign in early MS
- Dawson fingers: perivenular extension into deep WM (sagittal T2/FLAIR)
- Tumefactive MS: like brain tumor or infarct with less mass effect
- T1 black holes = axonal damage
o ± intrinsic rim of high T1 = microglial infiltration “beveled” or “lesion within lesion” appearance
- Central vessel sign:
o Dark vein in middle of high T2 lesion on FLAIR (DD migraine, small vessel dis)
Site of of plaques
• Supratentorial: Periventricular (85%), CC (70%), Juxtacortical, GM (uncommon)
• BS, Cerebellum, Spinal cord, 50%, Optic nerve, chiasm
Other findings
• Cortical central atrophy (20%–80%)
• Atrophy of CC (40%)
• Increased ferritin: low T2 in thalamus & putamen
• Mass effect of very large plaques (>3 cm) like tumors
• Spine: -
▪ May be solitary
▪ # is Cx spine (65%)
▪ Peripherally located

3
 CNS notes
Acute Disseminated Encephalomyelitis (ADEM)
• In children. After viral illness or vaccination.
• Like MS:
- Multiple LARGE high T2 lesions
- Involves CC
- Edema & enhancement
- All lesions enhance at same time
• DD from MS:
- Abrupt onset
- NOT in calloso-septal interface
- Symmetrical
- Less periventricular
- More BG
- Monophasic (MS is polyphasic)
• Acute Hemorrhagic Leukoencephalitis (Hurst Dis):
- Fulminant form of ADEM with massive brain swelling & death
- Hge is only seen on autopsy (not imaging)
Posterior Reversible Encephalopathy Syndrome (PRES)
• Causes: HTN, CTx, eclampsia, pre-eclampsia, immunosuppression drugs
• Parieto-occipital regions: Asymmetric cortical & subcortical WM edema
o Less: BS, BG & cerebellum
o High T2/FLAIR
o NOT restrict DW (DD stroke)
o Variable enhancement
• Not always reversible.
• If Hge occur in normotensive pt… postpartum cerebral angiopathy
• Cl: headaches, visual disturbance, seizure, confusion.
Radiation/Chemotherapy-Induced Cns Abnormalities
- Causes
- Cyclosporine: post high WM… blindness
- RTx & CTx potentiate each other's toxic effects
- DD residual tumor by MR perfusion ***
- May hemosiderin deposition, & mineralizing microangiopathy (Ca in BG & subcortical WM)
- Disseminated necrotizing encephalopathy:
o Methotrexate (Intrathecal) + whole brain radiation, leukemia + RTx & CTx
o Deep WM high T2W, Progressive diffuse
o Fatal
o Ring enhancement
- Acute changes
o Immediately after course & resolve after ends
o Mild edema & inflammation
- Chronic changes
o 6–8 m after non-fractionated therapy
o 2 y after fractionated therapy
o May be permanent
o Occlusion of small vessels, demyelination, proliferation of glial cells & mononuclear cells, atrophy
o High T2W, CT hypodense
Osmotic Demyelination Syndrome (ODS) central pontine myelinolysis
• Symmetric, noninflammatory demyelination of pons
• Dt rapid correction in pt with hyponatremia
• In chronic alcoholics & malnourished pt & in orthotopic liver transplantation
Findings:
• Initial MRI is Nl
• Diffuse central pontine high T2
• Spare corticospinal tracts (periphery)
• No mass effect or enhancement (Patchy enhancement in subacute phase)
• Acute phase: restricted DW
• Extrapontine lesions: in putamina & thalami

4
 CNS notes
GM Disease
Dementia
5% of population >65 y
Types:
Disease Atrophy Characteristics Imaging
Alzheimer (50%) + Temp lobe, hippocampus WM abn not prominent
Vascular demen(45%) + Atrophy Periventr lacunae; cortical & subcort infarc
NPH – Lacunae, BG Com HC
Binswanger + Periventricular WM lesion
Wernicke- Korsakoff + Lacunae, BG, gyral & vermis atrophy Med thalamus high T2W
Alzheimer Disease
• # degenerative br dis. # cortical dementia
• DD: SDH, vascular dementia, Binswanger dis, 1ry brain tumor & NPH
Findings:
• Not specific
• #: diffuse enlargement of sulci & ventricles
• Atrophy of hippocampi, ant temp lobes & sylvian fissures (Disproportionate)
• + High WM
• Regional bilat temporoparietal abn:
o SPECT HMPAO: decreased perfusion
o PET: decreased activity in:
▪ Early: post cingulate gyri, parietal, med temporal lobes & precuneus
▪ Late: in frontal lobe
• Preservation of sensorimotor strip, visual cortex, striatum, thalamus & cerebellum
Vascular Dementia
# 2nd cause of dementia
Types
Multi-infarct dementia
• 2nd# cause of dementia
• Cortical infarctions (territorial vascular infarctions)
• PET: multiple scattered areas of decreased activity
• Enlarged sulci & ventricles
• Prominent high T2
Subcortical dementia (Binswanger disease)
• High periventricular (penetrating vessel ischemia)
• HTN
• >55 Y
• Small vessel vascular dementia
• Spares subcortical U fibers
Dementia with Lewy Bodies:
• 3rd# cause of dementia (2nd# neurodegenerative)
• Cl/P is very similar to Parkinsons (but in DLB comes first)
• NL hippocampi
• Decreased PET in lat. Occ. cortex sparing cingulate gyrus (Cingulate Island Sign)
o Substantia negra atrophy
Crossed Cerebellar Diaschisis (CCD):
• Low bl flow of cerebellar hemisphere after contralat supratentorial insult (infarct, tumor resection, radiation)
Frontotemporal Dementia (Pick Disease)
• Rare
• Before 65 Y
• Frontotemporal horn enlargement & sparing occipital
• DD from Alzheimer:
o Less memory loss
o More personality changes (irritability)
o Loss of function
o Loss of interest
o Difficult word-finding
• PET frontotemporal hypometabolism

5
 CNS notes
Parkinson Disease
Loss of melanin containing neurons in substantia nigra
Clinical Findings: Cogwheel rigidity, Bradykinesia & Tremor
Types
• Parkinson dis
• 2ry parkinsonism: Neuroleptic drugs, Trauma, CO poisoning
Findings:
• MRI is normal
• Narrow& low T2 in pars compacta
• Signal loss in BG
o In T2W spin-echo & GE (black ganglia)
o Dt Fe-induced
o Site:
▪ Parkinson: globus pallidus
▪ Parkinson-plus: putamen
• DD: dementia with Lewy bodies (cerebral atrophy & occipital lobe low PET)

BG Disorders
Basal Ganglia Calcification
• 1% G. population
• No symptoms
Causes
• Idiopathic, physiologic aging (#)
• Metabolic
- Hypoparathyroidism (common)
- Pseudohypoparathyroidism
- Pseudopseudohypoparathyroidism
- Hyperparathyroidism (HPT)
• Infection (common)
- Toxoplasmosis
- HIV infection
• Toxin related (uncommon)
- CO
- Lead poisoning
- Radiation/chemotherapy
• Ischemic/hypoxic injury
• Neurodegenerative diseases (rare)
- Fahr disease
- Mitochondrial disorders
- Cockayne disease
- Hallervorden-Spatz disease

6
 CNS notes
Huntington Chorea
• AD
• Cl: choreiform movements & dementia
• Findings:
o Caudate nucleus atrophy
o Boxcar appearance of frontal horns
Wilson Disease AR
• Abn in copper transport protein, ceruloplasmin
Findings:
• Early: Nl MRI
• Putamen, & thalami: High T2 Low T1
• “Face of giant panda” sign: High tegmentum (except red nucleus) & low sup colliculus
• BG: hypodense on CT
• G. atrophy
• Hepatic cirrhosis
Fahr Disease AD
• Cl: late-onset dementia with extrapyramidal motor dysfunction
• Dentate nuclei & WM Ca
Leigh Disease
• Mitochondrial disorder of oxidative phosphorylation
• Child with lactic acidosis
Diagnosis
• Suggestive:
- Elevated serum pyruvate/lactate levels
- Typical findings on CT
• Definitive:
- Cultured skin fibroblast assay for mitochondrial enzyme deficiency
- Histology
Findings: MRI is more sensitive than CT
• Site: putamen > globus pallidus > caudate nucleus
• CT: bilat symmetric hypodense BG
• High T2W
Hallervorden-Spatz disease
• BG: eye of tiger
• Low T2 in red nucleus & substentia nigra
Neurosarcoidosis
• CNS involved in <5%
• Females, West Africans
• Serum & CSF ACE is elevated (70%–80%) in pul sarcoidosis
• Cl: Aseptic meningitis, fits & MS-like plaques
• Findings:
o Nodular homogeneous thickening & enhancement of dura & leptomeninges
▪ Iso T1W, High T2W
o + CSF spaces & parenchyma
o # N: Facial & acoustic N
o Enhancing optic tracts, optic chiasm, floor of 3rd vent & pit infun
• Dx: meningeal biopsy & raised ACE levels

7
 CNS notes
Toxic/Infectious
Creutzfeldt-Jakob Disease (CJD)
• Classical:
o In old pt
o Cl: progressive dementia over 4–5 m
o High T2, FLAIR & DWI: in caudate, putamen & GM
• Variant:
o In young pt
o Cl: progressive dementia over 4–5 m. More psychiatric & behavioral symptoms
o High T2, FLAIR & DWI: in pulvinar (specific)
o ‘Hockey stick’ appearance
Co Poisoning
• # cause of death from poisoning
• Early: cerebral edema + petechial Hge
• Late: ischemia: high T2/FLAIR & restr DW in Globus Pallidus, cortex, hippocampus, & substantia nigra
• Delayed post anoxic encephalopathy
- Weeks after CO poisoning
- High T2 in CC, subcortical U fibers, int & ext cap
- Low T2 in thalamus & putamen
Alcoholic & Wernicke Encephalopathy
• # cause of cerebellar volume loss
• Damage of BS & BG
• Cl: ataxia & nystagmus (Wernicke). If severe amnesia, confabulation & Korsakoff $
Findings:
1 Alcoholism:
- G.volume loss, # in cerebellum
- Mam bodies enhancement
2 Marchiafava-Bignami:
- Dt ch alchololism
- CC swelling, high T2 (body then genu then splenium)
- Involve central & spare dorsal, ventral fibers… “sandwich sign” on sagittal
3 Wernicke: bilat high T2/FLA1R in Periaqueductal GM, Med thalamus, Para-3rd vent, Mam body
4 Alcohol withdrawl $: volume loss in: temp cortex & ant Hippocampus
Seizure Medication Dilantin, phenobarbital
• Dilatation of cerebellar fissures & 4th vent
• Low uptake FDG PET
• Skull thickening

8
 CNS notes
Infections
Neonatal - HIV related - “characteristic” - Meningitis & cerebral abscess
II. Neonatal Infections (TORCH)
• Harmful in 1st 2 trimesters (No much in 3rd)
• Ca & microcephaly presents in all of them
1. CMV: 2. Rubella:
• # TORCH (3x Toxo) • Less common dt vaccines
• Site: germinal matrix • Less Ca
• Periventricular Ca + tissue necrosis • MR: WM focal high T2 (dt vasculopathy & ischemia)
• Highest ass with polymircogyria
3. Toxoplasmosis: 4. HSV:
• 2nd# TORCH. Ass with cats • 90% HSV-2
• Site: BG. random Ca • In infants: affects endothelial cells … thrombus & Hge
• Freq in 3rd trimester (problem in 1st & 2nd) infarction …encephalomalcia & atrophy
• HC • In adults: affect limbic sys
5. HIV: ‫الزتونة‬
• Not TORCH CMV #, Periventricular Ca, Poly microgyria
• Occur during pregnancy, delivery, Toxo HC, BG Ca
breastfeeding Rubella Vasculopathy/Ischemia. High T2 -Less Ca
• Brain atrophy more in frontal lobes HSV Hge infarct… encephalomalcia
• BG … faint enhancement on CT & MRI then HIV Frontal lobe atrophy
Ca
III. Infections Immunosuppressed Patients (AIDS)
1. HIV Encephalitis:
• 50% of AIDS Pt
• CD4 < 200
• Demyelination + gliosis … brain atrophy
• Symmetric high T2 / FLAIR in deep WM. T1 is Nl
• Spare subcortical U-fibers + GM (DD PML)
• No enhancement
2. PML: # opportunistic infection in AIDS is toxo
• 2-4% & Poor prognosis # fungal infection in AIDS is Cryptococcus
Typical abscess restricts diffusion
• CD4 <50. Caused by JC virus
Atypical infections (Toxo, fungal) not restrict
• Destruct oligodendrocytes
• Pareito-occipital region
• CT: Single or multiple scattered hypodensities
• Low T1 (DD HIV) & High T2/FLAIR (out of proportion to mass effect)
• No enhance Toxo Lymphoma
• Subcortical U-fibers + GM Ring Enhancing (eccentric) Ring Enhancing
• Asymmetry (DD HIV) # mass effect in AIDS # 2nd mass effect in AIDS
3. CMV: BG Perivacular
• Brain atrophy Thallium Cold Thallium HOT
• Periventricular hypodense (high T2/FLAIR) PET Cold Pet Hot
• Ependymal enhancement Hypodense Hyperdense
4. Toxo: Hypovascular Hypervacular
• # opportunistic infection in AIDS Perfusion: low CBV Perfusion: high CBV
• Low T1, high T2, ring enhancing (If > 1cm) Hge common Hge less
• BG Ca
• Marked edema
• Leptomeningeal enhance
• NOT restrict DW
• Th Cold (Lymphoma is hot)
5. Cryptococcus:
• # fungal infection in AIDS
• Cl: # meningitis (leptomeningeal enhance)
• Dilated perivascular spaces… Filed with mucoid gelatinous crap (not enhance)
• Cryptococcomas: BG. Low T1, high T2 & ring enhce

9
 CNS notes
6. Neurosyphillis
• Large & medium sized vessel arterltis … infarcts in BS, BG & MCA territory
HIV Enceph PML CMV Toxo Cryptococcus
Symm high T2 Asymm high T2 Periventric high T2 Ring Enhance Dilated Perivas Spaces
Spare SCU F Involve SCUF Ependymal Enhance Th Cold Basilar Meningitis
Nl T1 Low T1
IV.Characteristic Infections:
7. TB Meningitis:
• Dystrophic Ca
• Basilar meninges Enhance (as sarcoid) with minimal nodularity
• Comps: - Vasculitis & infarct (# in children) &Obs HC (DD sarcoid)
8. HSV:
• Type 1 in adults &Type 2 in neonates
• Swollen med temp lobe (unilateral or bilateral)
o High T2
o Restrict DW (more sensitive > T2)
o If bleed Blooming on Gr (common in adults, rare in neonate)
• Spares BG (DD MCA stroke)
• Limbic Encephalitis:
- Like HSV
- PNS (SC lung CA). Not infection
9. West Nile:
• In BG & Th. High T2 + restrict DW
• May Hge
10. CJD:
• DWI: Cortical Gyriform restrict (most sensitive)
• Hockey Stick / Pulvinar Sign
• Progressive atrophy
• 3 types: sporadic (80-90%), variant (rare), & familial (10%)
• Unilat, bilat, symm, or asymm
• EEG CCC apperance
• 14-3-3 protein assay in CSF
11. Neurocysticercosis:
• Organism is tinea solium. Undercooked pork
• # site: subarachnoid space over cerebral hemispheres > basal cisterns (worst outcome) > vent
• 4 stages:
▪ Vesicular - thin walled cyst (iso-iso T1/T2 + no edema)
▪ Colloidal - hyperdense cyst (bright-bright T1/T2 + edema)
▪ Granular - cyst shrinks, wall thickens (more edema)
▪ Nodular - small Ca lesion (no edema)
V. Meningitis & Cerebral Abscess
• 4 types: bac (acute pyogenic), viral (lymphocytic), ch (TB or Fungal) & non-infectious (sarcoid)
• Thick leptomeingeal enhancement
1- Empyema:
• T1 bright & restrict DW. Types:
a. Subdural #
- Don’t cross falx
- Dt frontal sinusitis
b. Epidural
- Don’t cross dural attachments
- Dt sinusitis (66%)
2- Intraaxial Infections: Abscess
DWI - Restricts
• Cerebritis, Abscess & Ventriculitis
MRS - High Lactate
• Rt to Lt shunts & pul AVMs
PET FDG - + ve
3- Ventriculits:
• Dt: shunt placement, intrathecal chemo, IV ext of abscess (V. serious)
• Ventricle enhance + ventricular fluid-fluid levels
• If septa … obs HC

10
 CNS notes
Brain Tumors
(1) Patient age -> (2) Location of Mass -> (3) Characteristics.
A) Age:
Peds Adults
Supratentorial Infratentorial Supratentorial Infratentorial
- Astrocytoma (including GBM) - JPA - Astrocytoma (including GBM) - JPA
- PXA (Pleomorphic Xanthoastrocytoma) - Medulloblastoma - Oligodendroglioma - Hemangioblastoma
- PNET - Ependymoma
- DNET - BS Astrocytoma
- Ganglioglioma
B) Location
Extra-axial masses Intra-axial masses
- Buckling of GWM interface - Expansion of brain cortex
- GM between mass &WM - No expansion of subarachnoid space
- Expanded subarachnoid spaces - Pial vessels peripheral to mass
- Displaced subarachnoid vessels
- Broad dural base
- Bony reaction
- CSF cleft
- Pial vessels medial to mass

Skull Base Supra-sellar Intraventicular CP Angle Pineal Region

(STARCH-MOE)
- Chordoma (midline) - Sup exten of sellar L - Ependymoma - Schwannoma - Pineocytoma
- Chondrosarcoma (off - Sarcoid - Subependymoma - Meningioma - Pineoblastoma
midline) - Teratoma (other germ- - Choroid Plexus Pap - Epidermoid - PNET
- Esthesioneuroblastoma cell tumours) - CentralNeurocytoma - Arachnoid Cyst - Tectal Glioma
- Sinonasal Carcinoma - Aneurysm, Adenoma - Colloid Cyst - Meningioma
- Mets - Rathke’s Cyst - Meningioma - Dermoid
- Lymphoma - Craniopharyngioma - Giant Cell Astrocytma - Germinoma
- Paraganglioma - Hamartoma
- Hypothalamic Glioma
- Meningioma, mets
- Optic Nerve Glioma
- EG
Cortical Based (DOG):
• Dysembryoplastic Neuroepithelial Tumor (DNET)
• Oligodendroglioma
• Ganglioglioma
Cortical tumor / cortical met have very little edema (occult without IV contrast)
Radiation injury Tumour recurrence
MR spectroscopy Decreased choline Decreased NAA
Increased choline
CT perfusion: (rCBV) Low High
Thallium SPECT Deceased activity Increased activity

11
 CNS notes
C) Signal Characteristics:
I. Mutiple: -
1. Mets (50% solitary !!)
2. Lymphoma, GBM, Gliomatosis Cerebri
3. Seeding tumours: Medulloblastoma, Ependymoma, GBM, Oligodendroglioma
4. $s:
NF1 NF 2 “MSME” TS VHL
Optic Gliomas Multiple Schwannomas Subependymal Tubers Hemangioblastomas
Astrocytomas Meningiomas IV Giant Cell Astrocytomas
Ependymomas
II. Enhancement: -
• No enhancement dt intact BBB: -
1) Low grade astrocytomas (except JPAs)
2) Cystic non tumoral lesions (Dermoids, Epidermoids, Arachnoid Cyst)
• Enhancement: -
1) Outside BBB- extra-axial tumor (meningioma, schwannomas, pineal region, Pit. region)
2) High grade tumors disrupting BBB (GBM)
3) Exceptions (low grade tumors): Gangliogliomas &pilocytic astrocytoma (JPA)
• Ring Enhancement MAGIC DR one.
- Mets, Abscess, GBM, Infarct (subacute phase), Contusion, Demylinating (open ring), Radiation Necrosis /
Resolving Hematoma
III.Restriction: -
• Hypercellular tumor: GBM, lymphoma
• Supratentorial: Abscess, Lymphoma
• CP angle case: Epidermoid
• Temporal horns: HSV
IV. Midline Crossing: -
1. Midline: GBM or Lymphoma
2. Meningioma of falx (simulating midline cross)
3. Radiation
4. Necrosis
5. Tumifactive MS plaque in CC
V. Calcification: -
• Oligodendroglioma (90% by CT & 100% by histopathology)
• Astrocytoma (20%). But it is #> oligodendroglioma
VI. High T1: -
• Fat: Dermoid, Lipoma
• Melanin: Melanoma
• Mucin: mucinous adenoCa
• Blood: Bleeding Met (CTMR: Carcinoid / Choriocarcinoma, Thyroid, Melanoma, Renal). Tumor (Pit apoplexy)
• Cholesterol: Colloid Cyst

Gliomas
Astrocytomas
Oligodendrogliomas Extra-axial post fossa masses
Paragangliomas 1. Choroid plexus papilloma
Ganglogliomas 2. Acoustic neuroma
Medulloblastomas 3. Meningioma
4. Chordoma
5. Epidermoid

12
 CNS notes
Adult – Supratentorial
1- Astrocytoma: 4- Oligodendroglioma:
• # 1ry brain tumor in adults • Ca 90%
• Include: • # frontal lobe
1) Pilocytic Astrocytoma (WHO l) • Minimal edema
2) Diffuse Astrocytoma (WHO 2) • Erode inner table of the skull
3) Anaplastic Astrocytoma (WHO 3) • CC… butterfly glioma
4) GBM (WHO 4) • Lp/19q deletion better outcome
• Enhancement: 5- 1ry CNS Lymphoma:
o Low grade doesn’t (WH02) • 2 # cause of CNS mass in AIDS (1st is
nd
o Higher grades do (GBM & some toxoplasmosis)
Anaplasties)
• # NHL
o Exception is pilocytic astrocytoma
• In periventricular region
(enhancing nodule)
o Cross midline & subependymal spread
2- Gliomatosis Cerebri: o Marked oedema
• Diffuse glioma with extensive infiltration o NECT: hyperdense
• Low grade: Not enhance o Low T2/FLAIR
• Little mass effect o Restrict DW
• Bilateral, involves > 3 lobes o Enhancement:
• Affect Thalami, caudate, lentiform &CC ▪ Immunocomprimised pt: ring dt
• Blurring of GWM differ central necrosis
• Extensive high T2 ▪ Immunocompetent pt: soslid &
3- GBM homogeneous
• # malignant form of astrocytoma o Thallium +ve on Spect (toxo is not)
• # in deep WM 6- Mets:
o Irregular, ill-defined hypodense mass • # CNS neoplasm
o Necrosis, Hge • From Lung (#) or Breast
o Extensive WM edema • >80% in GWJ
o Enhancement, (diffuse, heterog, ring) • More edema (DD infarction by contrast)
o Restrict DW ➢ T1 bright: Melanoma, mucinous adenoCa
o Spread across midline (butterfly glioma) ➢ Bleeding mets “MRCT”
• Ass. with Turcot $ (GI polyp) ➢ Cystic mets: SCC lung & adenoca lung
Adult - Infratentorial
1- Hemangioblastoma:
• Cyst with enhancing nodule in adult 4- Xanthogranuloma
• VHL (if multiple) • Benign choroid plexus mass
• Cause HC • Leave them alone
• No Ca (DD from Pilocytic astrocyt) • Restrict diffusion
• Feedimg vessel (DD from Pilocytic astrocyt) 5- Colloid Cyst
2- Subependymoma: • Inf aspect of septum pellucidum
• Well circumscribed IV masses • Ant.part of 3rd vent behind foramen of Monro
• # at foramen of Monro & 4th vent • Cl:
• Cause HC o Positional headache
• Not enhance o Sudden death (acute HC)
3- Central Neurocytoma: • Well circumscribed
• Benign • CT dense (dt mucinous fluid, desquamated cells
• # IV mass in adult 20-40 Y & proteinaceous debris)
• # Attached to septum pellucidum • High T1, T2 (Protein content/paramagnetic effect
• “Swiss cheese” numerous cystic spaces on T2 of of magnesium, copper & iron)
• Ca 95% (like oligodendrogliomas) 6- Meningioma
• Isointense to GM on all MR • IV # (80%) at trigone of lat vent
• Mild contrast enhance • > at lt
• IV Hge • Middle aged female

13
 CNS notes

Adult - Sellar / Suprasellar

T1 T2 Hormones • No Ca
Ant Pit Iso Iso FLAT: FSH, LSH, ADH, TSH, • CT: Hypodense, rim enhancement
Prolactine • T1W: Hyperintense, T2W: variable
Post Pit High iso OEG: OT, Endorphins, GSH 4- Vestibular Schwannoma
1- Pit. Adenoma • 75% of CPA masses
Microadenoma < 10mm from adenohypophysis (ant 2/3) • If bilateral … NF-2
Macroadenoma > 10mm • Enhances strongly but heterogeneous than
Macroadenomas meningomas
• # tumor of sella in adults • Widen porusac ousticus resulting in a “trumpet
• Widened sella with floor erosion shaped” IAC
• Heterogeneous necrotsis & Hge 5- Meningioma
• Low T1 & high T2 • Very Common extradural mass
• Delayed enhancement (hypointense in normal • # in women
enhancing gland) • # location over cerebral convexity
• Cl: • Ca
o Endocrin dysfunction (< than microadenomas); • Dural tail (pathognomonic). 3 main criteria for
Prolactinoma: definition:
• Fem… menstr irregularity, galactorrhea - Present in > 2 contiguous 5 mm sections
• Male … decreased libido - Greatest thickness adjacent to tumour
o Op chi involvement… bitemporal hemianopia - Enhancement > tumour itself
o Cav sin involv… CN palsies (III, IV, VI) • Hyperostosis of adj bone
Pit. Apoplexy • Enhance strongly
• Hge or Infarction of Pit. • Inward bulging of GWM jun
• Enlarged gland (pregnancy or macroadenoma). • Oedema 60%
• Bromocriptine (prolactin drugs), “Sheehan $”, • NECT: Hyperdense
Cerebral Angiography • # radiation-induced CNS tumour
• T1 bright (adenoma is T1 dark) - In radiation field
• Emergent finding (lack of hormones cause - Ass with low-dose radiation of T.capitis
hypotension) - Latency period: years
Sheehan’s $ - Multiple
• Dt hge -induced hypotension … pit infarction - Higher recurrence rates
• Early: enlarged homogeneous pit • +ve Octreotide & Tc-MDP
- Low T1 & high T2 & ring enhance • Meningiomas around cerebral convexity,
• Later: empty sella Parafalcine, sphenoidal meningiomas fed by ECA
• Cl: • Tentorial or CPA meningiomas fed by Bernasconi
- Visual field loss – Casanari A from ICA
- Headache Meningioma Schwannoma
- Ophthalmoplegia Homogeneous Less homogeneous
- Pit dysfunction (diabetes insipidus) enhancement enhancement
2- Craniopharyngioma Extend to but not Extend to & expand IAC
• # suprasellar mass. expand IAC “trumpeted” appearance
Ca
• Pap in Adults (Papi for Pappi).
Adamantinomatous in Ped Dural based IAC based
• Cl: Isointense T2 Hyperintense T2
- Headache (dt HC) Dural tail, Flaring of porus acousticus
- Bitemporal hemianopia (compression of op hyperostosis
chism) Obtuse angle with Acute angle with petrous bone
- Diabetes insipidus (compression of pit gland) petrous bone
- Growth failure Less Cys deg & Hge More Cystic degen & Hge
• Imaging 6- Arachnoid Cyst
- Ca • Benign
- Cyst formation • In subarachnoid space & contains CSF
- Enhancement of solid component • Mucopolysaccharidoses (perivascular spaces)
• Recur <Adamantinomatous (encapsulated) • Dark on FLAIR (like CSF)
3- Rathke Cleft Cyst • NO restrict DW
• Female: Male =2:1 • If interhemispheric: agenisis of CC
• Intra & suprasellar (70 %), pure intrasellar (20%) • Scallop inner table of skull
14
 CNS notes
7- Epidermoid Autoimmune Pit adenoma
• In comparison ‫في الجدول‬ hypphysisits
8- Dermoid Cyst Age <30 Asymmetrical
• In comparison ‫في الجدول‬ enlarged pit
Related to pregn Size >6 cm
Intracranial lipomas:
Homogenous enhance Sinus mucosal thick
• Well-circumscribed
Loss of post pit
• Fat density
bright spot
• Rim Ca
Enlarged stalk
• 30% in pericallosal region
• In CC: Ass with cong anomalies (# agenesis of CC-
encephalocele & cutaneous frontal lipomas)
Epidermoid Dermoid
Congenital lesions dt inclusion of ectodermal elements during closure of neural tube
Squamous epithelial lining & produce keratin
Only ectodermal elements Both ectodermal & mesodermal elements
(hair follicles, sweat & sebaceous glands)
Off midline Midline
Follow CSF in CT, T1 T2 Follow fat in CT, T1 & T2
Heterogenous FLAIR
Restrict DW
No Ca Ca
Enhance periph No enhance
Lobulated masses Heterogeneous (DD lipoma)
Encased vessels HC
Compress adjacent CN Rupture … IV fat…chemical meningitis
Ass. With NF2
Epidermoid Arachenoid
Lobulated outlines Smoth outlimes
Restrict DW Not restrict DW
High FLAIR Low FLAIR
Adult - Meningeal
1- Meningioma: mentioned
2- Hemangiopericytoma
• Heterogenous enhancement
• Abutt sphenoid sinus
• Displace &enhance lat ventricle
• Mimic aggressive meningioma: -
o Enhance homogeneously
o Dural tail, dural base of dural attachment
• But:
o No Ca
o No hyperostosis
o Invade skull
3- Mets
• # met to dura: breast CA
• 80% in GWJ
• More edema than 1ry tumor
• Melanoma mets is high T1

15
 CNS notes

Peds Brain Tumors:

<1Y
1- Atypical Teratoma / Rhabdoid • Good prognosis
• Highly malignant tumors (WHO IV) 3- Choroid Plexus Papilloma / CA:
• Contain ectodermal, mesodermal & endodermal • 85% <5 Y or adults
components • 15% of kids brain tumors < 1 Y
• Heterogeneous with fat, Ca & cystic components • IV mass, which makes CSF, causes HC
• Large tumors with necrosis • In Adults in 4thVentricle
• In pineal & suprasellar regions & # in cerebellum • In kids in lat ventricle (trigone)
• 2 Y, rare in > 6 Y • Strong enhancement.
2- Desmoplastic Infantile Ganglioglioma • Ca 25%
/ Astrocytoma “DIG”: • Angiography … enlarged chorodial arteries which
• Large cystic tumors supply tumor
• ALWAYS supratentorial > one lobe (frontal & 4- Mets:
parietal #) • In kids = Neuroblastoma
• In superficial cerebral cortex & leptomeninges o To bones & dura (rare to brain &
• Rapidly increasing head circumference meninges)
o In posterolat orbit (DD EG)
Early Adult Tumors:
1- Juvenile Pilocytic Astrocytoma: • Ca (30%) cyst formation (50%)
• Cyst with avidly enhancing mural nodule in kid • Minimal mass effect & oedema
• 30% solid • Bony remodeling
• WHO grade 1 • Low-grade with good prognosis
• In post. fossa (or optic chiasm) • “13 Y with seizures, temporal lobe cystic & solid
• Arise peripherally & displace 4th vent. mass, focal Ca”
(medulloblastoma arise centrally from vermis) 4- DNET (Dysembryoplastic
Hemangioblastoma Pilocytic astrocytoma Neuroepithelial Tumor):
Adult Child • Benign (arising from GM)
Hyperdense Isodense • # In temporal lobe
No Ca Ca • Kid with drug resistant seizures
2- Brain Stem Glioma: • Multiple cysts
• # in pons • No oedema, No enhancement
• High grade fibrillary glioma • CT: hypodense
• High T2 • High T2 “bubbly lesion”
• No enhancement. 5- PXA (Pleomorphic
• No biopsy needed Xanthroastrocytoma): 25Y
3- Ganglioglioma: • Superficial tumor, ALWAYS supratentorial
• < 30Y • In temporal lobe
• Well-circumscribed, hypo- or isodense • Invades leptomeninges
• In temporal lobe • Cyst with nodule
• Cl: focal seizures (# tumour with refractory • No peritumeral high T2
temporal lobe epilepsy) • Like DIG- but not in infant
6- Subependymal Giant Cell • Metting outside CNS
Astrocytoma: 8- Craniopharyngioma
• Part of TS (+ renal AMLs) • Adamantinomatous: kids, Ca , recur less
• Arise from lat wall of ventricle. At foramen of • “machinery oil”
Monro 9- Hypothalamic Hamartoma Hamartoma
• Ca of tuber cinereum
• Causes HC • Developmental malformations (part of HyTh bet
• Enhances homogeneously mam. bodies & Op. chiasm)
7- PNET(Primitive Neuroectodermal • Male, in 1st or 2nd decade
Tumor): • Well-defined pedunculated
• Histologically same as a medulloblastoma • T1 & T2 iso
• Deep WM • NOT enhance, Ca or necrosis
• Very heterogeneous • Not change size over time
• Cl: Gelastic seizures & precocious puberty
16
 CNS notes

10- Medulloblastoma 11- Ependymoma

More common Less Common
Project to 4th ventricle From floor of 4th ventricle
NOT extend to basal cisterns Extend to basal cisterns like “tooth paste”
Though foramen of Luschka & Magendie “plastic tumor”
Enhance Homogeneously Enhance Heterogeneously
Less Ca (20%) More Ca (50%)
Heterogeneous on T1 & T2 Bimodal (6 Y & 40 Y)
Hperdense Hge (fluid level)
Hypercellular & may restrict
Subarachnoid seeding “drop met”
Linear “icing-like” enhancement of brain surface
“Zuckerguss”
Ass. with Basal Cell Nevus $ & Turcots $
Gorlin $
-medulloblastoma + thick dural Ca + odontogenic cysts
-basal cell skin cancer after radiation
Pineal Region Tumors:
• Aquductal compression: dilation of third and lat ventr & normal forth ventric
1- Germinoma: # 3- Pineoblastoma
• Malignant • In childhood
• Exclusive in boys (If suprasellar: girls) • Solid
• Findings: • Highly invasive
o CT: Hyperdense • = PNETs in pineal gland
o Mass containing fat & Ca • Ass. with retinoblastoma
o Heterogeneous T1 & hypo T2 • Heterogeneous
o Vivid enhancement • Enhance vividly
• Cl acc to site: • Excellent survival rate
- Parinaud $: paralysis of upward gaze due
to compression of mesencephalic tectum Germinima Pineoblastoma
Dt inj of tactum (dorsal MB) In pineal region
- HC: compression of aqueduct of Sylvius Young age
- Precocious puberty in < 10 Y Avid enhancement
• Subarachnoid seeding: Dx by lumbar puncture Hyperdense on CT
• Very radiosensitive Subarachnoid seeding
2- Pineocytoma More common Less common
• Rare in childhood Central Ca (Engulphed) Peripheral Ca (exploded)
• Well-circumscribed, non-invasive
• Cystic + Ca
• Enhance
$s
NF-1 ON Glioma
NF-2 MSME: Multiple Schwannomas, Meningiomas, Ependymomas
VHL Hemangioblastoma (brain & retina)
TS Subependymal Giant Cell Astrocytoma, Cortical Tubers
Nevoid Basal Cell $ (Gorlin) Medulloblastoma
Turcot GBM, Medulloblastoma
Cowdens Lhermitte-Dulcos (Dysplastic cerebellar gangliocytoma)

17
 CNS notes

MR SPECT
• NAA:
- Highest NL peak
- Reduced in most brain lesions (neoplastic, vascular, or demyelinating)
- Super high in Canavans
• Creatine indicates cell metabolism
• Choline:
- Indicates cell turnover (tumor, infarct, inflammation)
- Normally –ve
- Highly increased in high-grade neoplasms
- Choline to creatine ratio > 1.5 = high grade tumour
• Lactate:
- Elevated in first H. of life normally
- Normally –ve
- Elevated in inflammation, infarction & some neoplasms
• Myoinositol is elevated with Alzheimer's & low-grade gliomas
• Alanine elevation is specific for Meningiomas (Not NAA)
• Glutamine is elevated in Hepatic Encephalopathy
- High Grade Tumor = Choline Up, NAA down, Lactate & Lipids Up
- Low Grade Tumor = Choline Down, NAA down, Inositol Up
- Radiation Necrosis: Choline Down, NAA Down, Lactate Up

18
 CNS notes

Trauma
1. Parenchymal Contusion:
• Large & superficial
• Locations: ant. temp &inf. frontal lobes
• Coup contre-coup
• blood + Edema
2. Diffuse Axonal Injury/Shear Injury:
• CT:
o Initial is normal
o Small low attenuation foci (oedema) or high attenuation foci (petechial Hge).
o Late: cerebral atrophy
• MRI:
o GE sequences is of choice
o Multiple small T2 bright foci
• Sites: post. GWJ, CC (spc splenium), inf. frontal & temporal lobes & centrum semiovale,
3. Subarachnoid Hge: see vvascular
Subdural vs Epidural
4. Extradural 5. Subdural Hge
Hge
Skull F Old alcoholic man, atrophic brain, fall alot, & stretch /tear bridging veins
Bi-convex or Bi-concave
Lenticular
Cross midline Not cross midline, extend into IH fissure
Cross dural reflections Not cross dural reflections (falx- tentorium)
NOT cross sutures Cross sutures
Usually arterial Usually venous
Rapidly exp
Mid menin A or V
Position Direction Area viewed
Towne’s AP occipital bone
Caldwell AP orbits
Water’s view Occipitomental facial bones, sinuses & zygomatic arches
Leptomeningeal cyst: `growing fracture'
• Skull defect with scalloped bony margins
• 1 % of paediatric skull F
• Skull F. ass. with dural tears (arachnoid herniation & CSF pulsations cause F diastasis)
• 2, 3 m after injury
• Gliosis of adjacent brain parenchyma
***Swirl Sign ***
• Non-contrast CT appearance of acute extravasation of blood into haematoma
• Unclotted fresh blood (hypodense) surrounded by clotted blood (Hyperdense)

19
 CNS notes
Vascular Dissection
• Causes: trauma: penetrating (ICA) > blunt (VA)
• PF: FMD, Marfan $, Collagen vascular dis, Homocysteinuria
• Angiography:
o Tapered luminal narrowing & enlargement of diameter
o `String' sign in severe stenosis
o No intimal flap in ICA dissections (no`double lumen' sign)
• CT: Peripheral high density crescent rim sign & No enhance
• MRI:
o High T1-weighted (methaemoglobin)
o No fat suppression (DD intramural haematoma from surrounding periarterial fat).
• Types:
o Carotid dissection:
• Horner's $
• # site: distal to carotid bifurcation
o Verebral dissection:
• Headache, Neck pain, Stroke after hours to weeks
• # site: C1-2 level
Subdural hygroma:
• Traumatic subdural effusion (CSF-fluid collection)
• In old or young children
• 6–30 d after trauma
• Asymptomatic, confusion or headaches
• DD for SAH:
o Devoid of blood products
o No vent. effacement
o No loss of sulci-gyral pattern
Brain Herniation
Subfalcine: # type
• Singulate gyrus herniates under falx
• Dt medially directed supratentorial mass effect
• Midline shift, vent deviation & bowing of falx
• ACA compressed … infarct
Descending Transtentorial:
• Uncus & hippocampus herniated through
tentorial incisura downward to post. fossa
• Effacement of ipsilateral suprasellar cistern
• Enlargement of ipsilateral CPA cistern
• Compression of:
o CN3 … ipsilat pupil dilation & ptosis
o PCA … occip ischaemia & infarction
o Perforatng basilic A brs …“Duret Hges”
(in MB)
o Ipsilat cerebral peduncle … contralat
hemiparesis
o Contralateral cerebral peduncle… Ipsil
hemiparesis (Kernohan’s notch phenomenon, false localizing sign)
Ascending Transtentorial:
• Post. Fossa mass… vermis herniate upward through tentorial incisura
• “Smile” of quadrigeminal cistern is flattened or reversed
• “Spinning Top” of MB dt bilat post compression
• Severe obs HC (at aqueduct level)
Cerebellar Tonsil Herniation:
• Dt severe herniation after downward transtentorial herniation
• (Chiari I = 1 tonsil 5mm, or both tonsils 3mm)
Transforaminal herniation
• Herniation of inf cerebellum downward through foramen magnum
• Obtundation & deaths

20
 CNS notes

Vascular
(Anatomy- Hge.- Stroke- Aneurysm- V.Malf)
Arterial Anatomy:
Branches of ECA: -
Some Administrators Like Fucking Over Poor Medical Students
Sup Th, Asc Phx, Lingual, Facial, Occ, Post. Aur, Max & Superf Temp
Ant.Circ. (Carotids):
1- Cl (Cx): 4 pathologies:
• Atherosclerosis: origin is very common.
• Dissection: spont (women), & in Marfans or Ehlers-Danlos
▪ Partial Homer’s (ptosis & miosis)
▪ Followed by MCA stroke
• Injured by ENT accidentally, If retrophx course
• Pseudoaneurysm due to phx infection
2- C2 (Petrous): - Big aneurysms
3- C3 (Lacerum): Meckel’s cave via transfacial approach
4- C4 (Cavernous): Aneurysms ass. with HTN. cav - carotid fist
5- C5 (Clinoid): aneurysm: compress ON … blindness
6- C6: (Ophthalmic - Supraclinoid): aneurysm (#)
• Origin at “dural ring”
7- C7 (Communicating - terminal): aneurysm: compress CN III …palsy

Cerebellar arteries:
• SCAs: Sup cerebellar surface, sup. vermis, cerebellar WM & dentate nuclei, CN V#.
• AICAs: anterolate cerebellar surface, MCP, flocculus & inferolat pons.
• PICAs: postero-infer cerebellar surface, Inf vermis, tonsils & posterolat medulla.
Vascular Variants:
- Fetal Origin of PCA: # “fetal PCOM”
• 30%
• PCOM is larger (or same size) as PI
• PCOM sup / lat to CN3 (Nl is sup / med)
- Persistent Trigeminal A:
• Fetal connection bet cav ICA to BA
• “Tau sign” on Sagittal MRI
• Risk of aneurysm
- Aberrant CA:
• Pulsatile tinnitus
• DD paraganglioma. Don’tbiopsy it
Venous Anatomy:
SSS sup cerebrum Superficial Deep
Tr. Sinuses temporal, parietal & occipital lobes SCV Basal Vein of Rosenthal
Tr. sinuses … sigmoid sinuses … IJV Vein of Trolard Vein of Galen
St. sinus … confluence of inf sagittal sinus & VOG Vein of Labbe Inf. Petrosal Sinus
VOG CC, BG, thalami & upper BS Superf MCV
Cavernous sinus petrosal sinuses & middle cerebral veins

21
 CNS notes
Veins
• Vein of Labbe: large vein, connect superf MCV & tr sinus
• Vein of Trolard: smaller vein, connect superf MCV & SSS
• Basal veins of Rosenthal: Deep veins, formed in sylvian
fissure, passes lat to MB in ambient cistern & drains into
VOG. Accompanies PCA
• VOG: “great” formed by union of 2 int cerebral veins
• CN 3 Palsy - PCOM Aneurysm
• CN 6 Palsy - Increased ICP
I- Hge
1. SAH:
• Causes:
- Aneurismal rupture 80–90%
- AVM 10%
- Other: HTN, tumour, embolic infarction, blood dyscrasia, eclampsia & intracranial infection
• Sensitive sequences: FLAIR (in acute & subacute), GE (in Ch… blooming)
o Supplemental O2 (50-100%) looks like SAH on FLAIR
• 2 –ve angiograms… bleeding is venous
Acute non-traumatic headache with max intensity in 1 H.
• Symotms: High risk if:
1. # focal neurological signs (33%) o > 40Y
2. Seizures (6:16%) o Neck pain
3. Altered consciousness (2%) o Loss of consciousness
4. Subhyaloid Hge (10%) o Onset with exertion (but not sexual activity)
5. Vertigo (rare) o Arrival by ambulance
• Comp: o Vomiting
1 Vasospasm o Bl/P >160/100 mmHg
o Causes: # SAH. Others: Meningitis, PRES
& Migraine
o In 4-14 d after SAH (NOT immediately)
o Smooth, long segments of stenosis
o Involves multiple vascular territories
o Lead to stroke
o Leading\ cause of death and morbidity
2 Acute obs HC
o Early
o Dt: IV bl, ependymitis … obs aqueduct of Sylvius or outlet of 4th vent
3 Comm HC:
o > 1 w dt:impaired CSF abs
4 Superf Siderosis:
o Late
o Staining surface of brain with hemosiderin
o Curvilinear low signal on Gr
o Sensorineural hearing loss & ataxia
• Fisher scale
- Grade 1: no hge
- Grade 2: SAH < 1 mm
- Grade 3: SAH > 1 mm
- Grade 4: IV Hge or IPH extension

22
 CNS notes
2. IPH:
a. Hypertensive Hge:
- Site: BG (#putamen), pons, &cerebellum
- IV extension
b. Amyloid Angiopathy:
- Old normotensive pt on dialysis
- Multiple peripheral (in CMJ) hge areas sparing BG
c. Septic Emboli:
- Hx of IV drug user, transplant, cyanotic heart dis, AIDS, lung AVMs
- Tiny abscesses surrounded by edema; numerous small foci of restricted diffusion
- Result in abscess & mycotic aneurysms (#in distal MCAs)
- Site: CMJ & BG
- Parenchymal bleed in patient with infection
d. Tumours:
- Causes: Pit. Adenoma, PNETs, Epndymoma, Epidermoid, GBM, Oligodendroglioma & Mets
- 5–10% of tumours
- Features
o Complex CT pattern
o Incomplete haemosiderin ring
o Persisting oedema
e. Vascular
- AVMs, vasculitis
3. IVH: Trauma, tumor, HTN, AVMs, & aneurysms
4. EDH / SDH: Trauma, AVFs & High Flow AVMs
MRI & CT appearance:
Stage Time Biochem Location T1 T2 CT
Hyperacute 6h ‫ ساعات‬6 Oxy-Hb IC I Be 40-60 HU
Deoxy-Hb IC I D 60-80 HU
Acute 8h-3d ‫أيام‬3
+ FF level
Early subacute 3-7d ‫أسبوع‬ Met-Hb IC B D Low periph
Late subacute 1-4 w ‫شهر‬ Met-Hb EC Ba By (D rim) High centre
Chronic > 4w Hemosiderin EC Do Do Low periph

23
 CNS notes

II- Stroke
• Causes
1. Infarction, 80%
- Atherosclerosis, 60% ADC DWI
- Cardiac emboli, 15% Diff. effect only Diff. + T2 effect
- Other, 5% Spcif (less sens) Sens. (less spcif)
2. Hge, 15% Abn = low SI Abn= High SI
3. Non traumatic SAH, 5%
4. Venous occlusion, 1%
5. Drugs: anti HTN
• It is clinical Dx& imaging compliment Dx
Watershed Zones:
• Junction between vascular territories zones
• Prone to ischemia, especially in hypotension or low O2(near drowning, Roger Gracie’s cross choke from mount)
• Watershed Infarcts in Kid = Moyamoya

CT Signs:
Best seen in WW8, WL32
Dense MCA Intraluminal thrombus is dense, in Ml &/or M2 seg
Insular Ribbon Loss of normal high-density insular cortex dt cytotoxic edema
Loss of GM-WMD BG / Int Cap & Subcortical regions
Obscuration of lentiform nucleus
Mass Effect Peaks to 3-5 d
Enhancement Rule of 3s: Starts in 3 d, peaks in 3 w, gone by 3 m
“Fogging”
• When infarction = normal tissue. In non-contrast CT & T2
• 2-3 w post infarct
• Contrast demarcate infarction
Imaging Signs on MRI:
Stage Time T1 T2 DWi ADC FLAIR
Hyperacute 0-6 h ‫ ساعات‬6 iso iso   iso
Acute 6h-4d ‫ أيام‬4     
Subacute 4-14d ‫أسبوعين‬    Pseudo Nl 
Chronic >14d (as CSF)     
Contrast: rule of 3; starts 3 d, peaks 3 w, & gone 3 m
Perfusion:
- MTT:(mean transit time) ‘seconds’ Both perfusion & diffusion abn = infarction
- Time blood takes to reach particular region Perfusion > diffusion = penumbra
- Sensitive
-  in infr. & punumbra
- CBV: (Cerebral bl volume) ‘units’ Restrict DWi (Other than stroke)
- Total volume of circulating blood in voxel - Abscess, CJD (cortical), HSV
-  in infr & punumbra - Epidermoids, lymphoma (Hypercellular Tumor)
- CBF (Cerebral blood flow) ‘units’ - Acute MS, Oxyhemoglobin, & Post Ictal State
- Flow of blood in voxel - Artifacts (susceptibility & T2 shine through)
-  in infr.  in punumbra

24
 CNS notes
Sroke in neonates:
- In term infant
- Thrombosis > embolism
- # in MCA
- Causse: traumatic delivery, vasospasm dt meningitis & emboli dt cong HD
- US: echogenic parenchyma in arterial territory
Hemorrhagic Transformation –(50%)
• 6 h to 4 days, TPA (tissue plasminogen activator) within 24 h
• Forms:
o Tiny petechae in GM (90%)
o Hematoma (10%)
• Occur in anticoagulation, TPA, embolic strokes, venous infarcts
Hemorrhagic Transformation with TPA
1. Multiple Strokes
2. Prox MCA occlusion
3. >1/3 of MCA territory
4. >6 H since onset “delayed recanalization”
5. Absent collateral flow
Venous Infarct:
• Dt dural sinus or deep cerebral vein thrombosis (venous occlusion)
• Not conform to arterial territories
• Midline veins occlusion … bilateral infarction
• Risk for Hge transformation
• Causes:
o In babies: dehydration
o In children: mastoiditis
o In adults: coagulopathies (protein C & S def) & oral contraceptives
• # site: sagittal sinus (75%)
• Findings:
o Brain edema: early & marked (DD: art infarcts edema starts later at 3-5 d)
o Unenhanced CT: hyperdense veins, GW jun Hge & brain oedema
o CT venography: ‘empty delta’
o MRI Heterogeneous restricted diffusion
o Arterial stroke = Cytotoxic Edema
o Venous Stroke = Vasogenic + Cytotoxic Edema
Stigmata of ch venous thrombosis Dural AVF, or increased CSF pressure from impaired drainage
Lacunar infarction Vs
TIA
Roth-well classification:
1- Age (~ 60): 1 Management plan for high risk TIAs:
2- HTN (~140/90): 1 1. 300 mg aspirin immediately
3- DM: 1 2. Assessment & investigation in 1 day
4- Symptom: 10-60 min: 1 (7 days for low risk patients)
5- Unilateral weakness: 2 3. Carotid imaging in 7 days
6- Symptom: > 60 min: 2 4. Endarterectomy (if appropriate) in 2 weeks
7- Speech disturbance: 2 5. Measures for 2ry prev
Scores 5 = high risk 6. MR (with DWI) only if uncertain
Risk of stroke within 4 w is 20%

25
 CNS notes

III- Aneurysm
CTA in 1ry detection. DSA for difficult cases
Types:
1. Saccular (Berry): 80%
• 20% pop, 20% multiple, 20% gaint
• Types: Developmetal/ deg.- Traumatic – infective (Mycotic) – neoplastic – flow related – vasculopathy
• RF: Smoking, PCKD, Tissue disorders (Marfans, Ehlers-Danlos), Aortic coarctation, NF, FMD & AVMs
• Site: Branch points (persistent trigeminals)
- Ant. Circ (90%) (A.com #)
- Post. Circ… Basilar is #. PICA origin is 2nd #
• Rupture risk; Size, Post. Location, Hx of prior SAH, Smoking & Female.
• Site:
- SAH of multiple aneurysms
- Locationof SAH/Clot
- Locationof vasospasm, size, &which is most irregular (Focal out-pouching Murphy’s tit”)
Maximum Bleeding-Aneurysm Location
ACOM Interhemispheric Fissure
PCOM Basal Cistern
MCA Trifurcation Sylvian Fissure
Basilar Tip Interpeduncular Cistern / IV
PICA Post. Fossa or IV
2. Fusiform Aneurysm
- Ass. with PAN, CT Disorders, or Syphilis.
- Site: post. circ. mimic CPA mass.
3. Pseudo Aneurysm
- Irregular (often sacular) at a strange location.
- Focalhematoma next to vessel on non-contrast.
▪ Traumatic - distal 2ry to penetrating trauma or adjacent F.
▪ Mycotic - distal (#in MCA), ass. history ofendocarditis, meningitis, or thrombophlebitis.
4. Pedicle Aneurysm
- Found on artery feeding AVM (75%).
- Higher risk to bleed thanAVM itself (high flow).
5. Blister Aneurysm
- Angio is negative
- Broad-based at a non-branch point (supraclinoid ICA is # site).
6. Infundibular Widening
- Not a true aneurysm, but funnel-shaped enlargement at origin of P.Com Artery at jun. with ICA.
- < 3mm
Saccular (Berry) Branch Points - in Ant.Circ
Fusiform Post.Circ.
Pedicle Aneurysm Artery feeding AVM
Mycotic Distal MCAs
Blister Aneurysm Broad Based Non-Branch Point(Supraclinoid ICA)

26
 CNS notes

IV- Vascular Malformation

1) AVMs:
- Abn network of arteries & veins with no capillary bed.
- 98% solitary.
- age is 20 to 40 Y.
Types:
- Parenchymal, 80% (ICA & VA; congenital)
- Dural, 10% (ECA; acquired)
- Mixed, 10%
Imaging:
• Intraarterial angioraphy … gold standard
• MRI
o MR DSA: heplfull
o MRA/ MRV may be normal
- Serpiginous high & low signal (depending on flow rates) within feeding & draining vessels
- Replaces but does not displace brain tissue (No mass effect unless complicated by Hge or edema)
- Edema only in recent Hge or V. thrombosis with infarction.
- Aneurysm 10%
- Ca+ 25%
- “Susceptibility artifacts” on MRI in old hemorrhage
- Early venous drainage (DD other vascular lesions)
Compl:
1. # Hge (3%/Y). Risk in:
Spetzler's Criteria Eloquent =
1) Small size AVM (higher pressure)
0 1 2 3 sensorimotor
2) Single draining vein cortex, visual
Eloquence No Yes — —
3) Aneurysm in cortex, HTh,
Draining Superficial Deep — —
a. Intranidal/perinidal Th, int cap, BS,
vein
b. Circle of Willis cerebellar
Size — <3 3-6 <6 peduncles, deep
c. Arterial feeder
4) Venous stasis cm cm cm nuclei
5) BG/thalamic/ periventricular
- Not pregnancy
2. Seizure is 2nd#comp
3. Adjacent atrophy& gliosis dt vascular steal & ischemia
Dural AVM:
• Abn connections bet arteries supplying meninges (not brain) & small venules in dura
• High or low flow
• 2ry to dural sinus thrombosis
• In 50s-60s
• Cl: Pulsatile tinnitus (sigmoid sinus) vision problems (cavernous sinus)
• Dilated cortical veins
• No nidus
• Occult on MRI/MRA –need catheter angio
• Bleeding: Direct cortical venous drainage
2) Cavernous Malformation: “cavernomas” “cavernous angiomas.”
• Dilated endothelial cell-lined spaces with no normal brain within lesion
• Location: 80% supratentorial
• Multiple 60%-80%
• Any age
• Ass with DVA
Findings:
- MRI is of choice. Best sequence: Gradient (hemosiderin… susceptibility effect)
o Complex SI dt bl products in varying age
o Popcorn lesion: bright lobulated center with black (hemosiderin) rim
o + Ca+
- CT: Hypodense areas
- Angiography is normal

27
 CNS notes

3) DVA:
• Normal variant
• If resected leads to venous infarct
• “caput medusa” dilated medullary veins or “large tree with multiple small branches”
• Ass with cavernous malformations.
4) Capillary Telangiectasia:
• Slow flow lesion
• Normal intervening brain tissue
• Don’t bleed
• Totally incidental
• Single lesion in pons
• Best sequence is gradient (slow flow & deoxyhemoglobin)
• “brush-like” or “stippled pattern” of enhancement
• Comp of radiation therapy
5) Mixed
• DVA with AV shunting or DVAs with telangiectasias
VOG malformation:
• Enlarged V of galen…Abn midline AV communications
• Types: in utero- neonatal- infantile- adult
• In utero & ineonatal: dt high output cardiac failure & mass effect of VOG:
- Hydrops
- Cardiomegaly
- Obst HC
- Seizures, focal neurologic deficit & Hge
- Spontaneous hge
Vasculitis
1- 1ry 1ry Angiitis of CNS (PACNS)
2- 2ry (infection, sarcoid) Meningitis (bacterial, TB, Fungal), Septic Embolus, Sarcoid
3- Systemic vasculitis + CNS involvemen PAN, Temporal Arteritis, Takayasu, Wegeners (PNS, lung nodules)
4- CNS vasculitis from Systemic Dis Cocaine, RA, SLE, Lyme’s
• “Beaded appearance”
• Focal vascular occlusions
• PAN is # systemic vasculitis to involve CNS (but late)
• SLE is # Collagen Vascular Dis
Misc Vascular Conditions
1- Moyamoya
• Non-atherosclerotic (in Japan)
• Progressive stenosis of supraclinoid ICA, ACA, MCA … occl
• Watershed Distribution
• Puff of Smoke in angiography
• Multiple flow voids dt hypertrophied lenticulo-striate arteries
• Moyamoya $: when ass. with: NF, radiation, Downs $, SCD (child)
• Bi-Modal Age (early childhood, & middle age)
• Children Stroke, Adults Bleed
2- CADASIL (Cerebral AD Arteriopathy with Subcortical Infarcts & Leukoencephalopathy).
• 40 Y with migraine, TIA then dementia
• MRI: WM dis in multiple vascular territories
• Subcortical infarcts in ant. Temporal, frontal lobe & ext cap
• Occipital lobes are spared
3- NASCET Criteria: North American Symptomatic Carotid Endarterectomy Trial:
• For carotid stenosis
• Degree of stenosis: max ICA stenosis (A) / parallel (non-curved) dis Cx ICA (B)
• Formula [1- A/B] X 100%
4- Sheehan’s $:
• Pit infarction gt hge-induced hypotension in pregnancy
• Early: enlarged homogeneous pit (low T1, high T2) contrast ring enhancement
• Late, empty sella
• Cl: visual field loss, headache, ophthalmoplegia & pit dysfunction (diabetes insipidus)
28
 CNS notes

5- Spontaneuos Intracranial Hypotension:

• Leaking CSF, decrease overall volume, volume of venous blood will increase to maintain equilibrium
• Postural headache exacerbated by upright position
• Result is
- Diffuse dural enhancement (100%)
- Cerebellar tonsils & MB herniation
- Sagging of brain
- Flattening of pons against dorsal clivus
- Increased enhancement of Pit
- Depression of optic chiasm on sella
- Distention of dural venous sinuses
- Subdural hematoma & subdural hygromas
• Fast spin-echo T2W imaging of spine: source of leak
• Rx: epidural `blood patch'
6- Idiopathic Intracrainal Hypertension (Pseudotumor Cerebri):
• Fatty middle-aged women with headache
• Unkwon etiology (too much CSF, less absorption)
• Ass with hypothyroid, cushings, vit A toxicity
• Increased CSF:
1. Slit like ventricles
2. Pit. shrinks (partially empty sella)
3. Compressed venous sinuses
4. Vertical tortuosity of optic nerves & flattening of post. sclera
7- profound hypoxia
• Diffuse cerebral oedema
• Loss of GW distinction
• Surface blood vessels brighter than Nl brain (confused for SAH)
• Sparing of cerebellum ss(bright cerebellum' sign)
Doppler imaging of CA:
Stenosis
0-50% 50-69% >70% Total occlusion
ICA PSV (cm/s) < 125 125 -230 > 230 undetectable
ICA EDV (cm/s) < 40 40 - 100 > 100 NIA
ICA/CCA PSV ratio <2 2-4 >4 NIA
Reversal CCA flow
Edema:
Cytotoxic:
• Intracellular swelling, arrested metabolism of cells
• Stroke (or trauma)
• Conforming to vascular territory Cytotoxic Vasogenic
• Well-demarcated edges Intracellular Extracellular
• In cortex, loss of GWD Stroke Tumor & infection
• Not cross CC GM WM
• Restricted diffusion (Low ADC) Not cross CC Cross CC
Vasogenic: Low ADC High ADC
• Extracellular, 2ry to disruption of BBB Restrict DW Not restrict DW
• Increased vascular permeability
• Tumor & infection
• In WM (Not in cortex)
• Extend across vascular territories & CC
• Respond to steroids
• Unrestricted diffusison (High ADC)

29
 CNS notes

Pediatertics
Anatomy
2. Cavum Variants:
• Cavuin Septum Pellucidum (5th ventricle)
o 80% term infants - 15% adults
o Dilate & cause obs HC
• Septooptic dysplasia (SOD) = absent septum pellucidum + ON hypoplasia + Schizencephaly
• Cavum Vergae - post. continuation of cavum septum pellucidum
o Ass with CSP
• Cavum Velum Interpositum - Extension of quadrigeminal cistern (In sagittal)
o To foramen of Monro
o Above 3rd vent & below fornices

Basal cisterns:
• Suprasellar cisterns like pentagon
o Top … Interhemispheric fissure
o Ant.… Sylvian cisterns
o Post. … Ambient cisterns.
• Quadrigeminal plate like smile
3. Brain Myelination:
• T1 of baby is similar to T2 of adult
Immature Myelin Mature Myelin
High Water, Low Fat Low Water, High Fat
Tl dark, T2 bright Tl bright, T2 dark
• T1 proceed T2 changes (1 y for T1, 2 y for T2)
• Caudal to cranial, post to ant, deep to superficial, central to periph, & sensory then motor
• Milestones:
- Term birth: BS, cerebellum, post limb IC
- 2 m: ant limb IC
- 3 m: splenium of CC
- 6 m: genu CC
- 40 m Subcortical fronto-temporo-parietal region “peri-trigonal region: Terminal zones”
Brain Development
• Ant& Post Pit are Bright at Birth (only post is bright in 2m - 2 y)
• Calverial BM is active (low T1) in young kids & fatty (high T1) in older kids
• Sinuses form in following order: Maxillary, Ethmoid, Sphenoid, & Frontal Last
• Brain Iron increases with age (globus pallidus darkens)

30
 CNS notes

H&N
Choanal Atresia:
• Dt oronasal membrane separating nasal cavity from oral cavity
• Unilat or bilat (symptomatic immediately)
• "can't pass NG tube "

• “Thicked vomer”
• Ass with CHARGE $
Congenital Piriorm Aperture Stenosis:
• Dt abn development of med nasal eminences & failure of formation of primary palate
• Piriform aperture of nasal cavity: stenotic & narrow palate
• Ass with central maxillary "MEGA-incisor"
• Ass with Midline defects of brain (CC agenesis & holoprosencephaly)
• Next Step: brain image
Branchial Cleft Cyst
• Several types # is 2nd (95%)
• At angle of mandible
• “Extend between ICA & ECA (notch sign) just above carotid bifurcation”
• Comp: infection, (asymptomatic)
Fibromatosis Coli
• Benign mass in sternomastoid in neonates
• Present with torticollis (chin points towards opposite side) Lat neck cyst = branchial cleft cyst
• US: two SCM Midline neck cyst = thyroglossal duct cyst
• Resolve spontaneously (may needs passive physical therapy)
Brain:
Periventricular Leukomalacia: PVL
• Ischemic /Hge dt ch. hypoxia during birth
• In premature (< 1500g)
• Watershed areas
• Adjacent to trigone of lat vent
• Spare: BS, cerebellum & deep GM structures in mild cases.
• Periventricular cystic changes (1-3 w)
o Symmetrical
o Never septated
o Resolve over time
• 50% cerebral palsy
• US is not sensitive early
Acute hypoxic ischaemic cerebral injury:
• Reversal sign:
o Hypodense GM & WM density, decreased GWD
o Hyperdense BG, thalami & cerebellum
• Causes: accidental trauma, near drowning, cardiac arrest, status asthmaticus & status epilepticus
• Ass with NAI
• Poor prognosis.
Porencephaly
• Destroyed brain tissue
• Congenital or dt vascular or infection
• Asymmetrical
• Rarely disappears over time
• Extension of vent or sub-arachnoid space
Hypoxic damage in full term infant: in most metabolicaliy active areas; putamen, thalami & adjacent WM

31
 CNS notes

Germinal Matrix Hge:

• Only premature. No GM Hge in full term infant
• Ant to caudothalamic groove & inf to lat vent
• Common earlier (40% in 1st 5 h & 90% in day 4)
• Grading:
o Grade I: Subependymal Hge mortality rate: 0%
o Grade II: IV Hge mortality rate: 10%
o Grade III IV Hge with ventr dilat mortality rate: 20%
o Grade IV: IC Hge 20% mortality rate: > 50%
Chord plexus Hge
• Inferomedial aspect of ventricular floor
• Tapering toward cardio-thalamic groove
• Never ant to foramen of Monro

32
 CNS notes

Hydrocephalus
Indicators of HC:
- Dilatation of recesses of 3rd vent
- Convexity of 3rd vent
- Expansion of temporal horns
- Effacement of sulci
- Narrowing of mamillo-pontine distance
- Enlargement of ventricles out of proportion to sulcal dilatation
Specific MR findings
- Transependymal CSF exudation (high FLAIR)
- Accentuation of aqueductal flow void in normal pressure HC
1- Obstructive
A. Non-Communicating:
• Obstruction; dilated some ventricles & some normal sized.
• Causes:
1) Aqueductal Stenosis: Enlarged lat & 3rd vent (4th is normal)
• Congenital #: Dt web, diaphragm or gliosis
• Acquired:
o Extrinsic: tectal plate glioma & pineal tumor
o Intrinsic: ventriculitis or SAH
• Stenosis at level of sup colliculi or at intercollicular sulcus
• Findings:
o Funnel-shaped aqueduct (best diagnostic clue)
o Lack of CSF flow in aqueduct on phase contrast MRI (most specific)
o Ballooning of lat & 3rd ventr
o Nl 4th ventr
o Others:
▪ Thinning of CC
▪ Downward displacement of int cereb veins, 3rd vent floor
2) IV Mass:
• Colloid cyst in 3rd vent… obs f. of Monro… sudden death / thunderclap headache
3) Outlet Obs of 4th Ventricle
• All ventricles are dilated (Like com HC)
• Congenital: DWM
• Acquired: basilar meningitis or post hge ependymitis
B. Communicating:
• All ventricles are dilated (25% 4th vent is Nl).
• Obst in villi / granulation, blocking reabsorption
• 4 main causes:
1) Normal Pressure HC:
• Idiopathic
• Findings:
o Ventriculomegaly (sparing of 4th vent) out of proportion to sulcal enlargement
o Nl hippocampi
o Upward bowing of CC
o Aqueductal ‘flow-void’ sign: increased CSF velocity in aqueduct (observed in Nl individuals)
o MRI: transependymal flow &/or flow void in aqueduct & 3rd ventricle
• Wet, wacky & wobbly = triad of urinary incontinence, confusion, &ataxia
• Rx: surgical shunting
•
2) Blood; SAH: Most sensitive sign on non-contrast CT is enlarged temporal horns of lat vent
3) Pus: Meningitis
4) Cancer: Carcinomatous Meningitis
2- Non- Obstructive.
• Lesion producing CSF…. Choroid Plexus Papilloma
• Transependymal flow in acute HC > in ch HC

33
 CNS notes

Developmental / Congenital
1. Failure to form
2. Failure to cleave
3. Failure tomigrate
4. Normal forming but massive insult makes you look like you didn’t form
5. Herniation $s
6. Craniosynostosis
7. Neurocutaneous $s (phakomatoses)
I. Failure to Form:
CC Dysgenesis / Agenesis:
• CC is formed front to back (last is rostrum)
• Hypoplasia of CC = absence of splenium with intact genu
• # anomaly ass with other CNS malformations
• Ass. with Schizencephaly, Lissencephaly, Porencephaly, Holoprosencephaly, Chiari II, DW $,
Encephalocoele, Lipoma, Polymicrogyria
• Isolated in <10%
• Finding:
o Absent cavum septum pellucidum
o Colpocephaly (asymmetric dilation of occipital horns)
▪ DD: Pericallosal Lipoma
o Steer horn in coronal
o Vertical ventricles widely spaced (racing car) in axial
o High riding 3rd vent
o Enlarged foramen of monro
o Sunburst gyral pattern
o Wide interhemispheric fissure cyst
o Sulcation delay (if <30 w): global WM dysgenesis
o Heterotopia: periventricular nodular + parenchymal low T2
o Post fossa abn: Cerebellar hemispheric abn
Intracranial Lipoma:
• Congenital malformations, not true neoplasms
• Ass. with CC dysgenesis
• In interhemispheric fissure (50%)
• Ca in tubulonodular type
Iniencephaly:
• Rare
• Deficit occipital bones
• Enlarged foramen magnum
• “Star Gazing Fetus” (hyper-extended Cx spine, short neck, & upturned face)
Arhinencephaly:
• No olfactory bulbs & tracts
• Ass with Kallmann $ (hypogondaism, mental retardation)
Rhombencephalosynapsis: In cerebellum
• No vermis & fused cerebellum
• Transversely oriented singe ‫ قرد‬lobed cerebellum
Joubert $:
• “Molar Tooth” appearance of sup cerebellar peduncles
• Small cerebellar vermis
• Absent pyramindal decussation
• Ass with:
- Retinal dysplasia (50%)
- MCDK (30%)
- Liver fibrosis (COACH $)

34
 CNS notes

Dandy Walker: = Absent Vermis

“torcular-Iambdoid inversion” torcular is above level of lambdoid
Ventriculocele” DWM Cystic Dilation of 4th Complete or partial Enlarged Post.Fossa + occipital bone
Variant Agenesis Vermis erosion > encephalocele
“Classic” DWM Cystic Dilation of 4th Complete or partial Enlarged Post. Supply Rotated
Agenesis of Vermis Fossa vermian remnant
“Variant” DWM Partially obs 4th Vent Variable Hypoplasia of Post. Fossa NOT enlarged
Vermis (less severe)
Persistent Blake Cyst below &post. to Normal Vermis Post. Fossa NOT tentorium is
Pouch thevermis enlarged elevated

Mega Cisterna Magna Retro-Cerebellar CSF Normal Cerebellum Enlarged post. Fossa caused by Enlarged
Space >10mm cisterna magna

II. Failure To Cleave

1. Holoprosencephaly (HPE):
• Brain fails to cleave into two separate hemispheres.
• Cleavage occurs back to front (opp to CC); mild form post. cortex is normal &ant. cortex is fused
• Types:
Lobar (mild) Semi-Lobar Alobar (severe)
Brain formation is nearly normal Variable cleavage No cleavage at all
- Absent Septum pellucidum - Partial falx, small 3rd vent - Absent falx, absent 3rd vent
- Falx, CC & vents are normal - Variable cleavage of hemispheres, thalami - Fusion of cerebral hemispheres,
- Hemispheres cleavage & lat vent thalami & BG
- Post. brain is normal - Single large lat ventricle
Pit Problems No olfactory tracts & bulbs No Falx. No CC
• Ass with:
1. Single Midline Eye (Cyclop) “face predicts brain, but brain doesn’t predict face”
2. Solitary Median Max Incisor (MEGA-Incisor)
3. Nasal Process Overgrowth leading to Pyriform Aperture Stenosis
• Meckel-Gruber $: triad: Holoprosencephaly + Multiple Renal Cysts + Polydactyly
2. Anencephaly:
• “No -brain” (only BS), no skull, & no scalp.
• Extreme end of Holoprosencephaly spectrum.
• Not compatible with life
• AFP is elevated
• Polyhydramnios
• “frog eye” appearance on coronal plane (dt absent cranial bone / brain with bulging orbits)

35
 CNS notes

III. Failure to Migrate / Proliferate:

1. Hemimegalencephaly:
• Enlargement of all or part/s of one cerebral hemisphere
• Focal or diffuse neuronal migration defects (polymicrogyria, pachygyria, & heterotopia)
• Big Ventricle + Small Side = Atrophy (Rasmussen’s Encephalitis)
• Big Ventricle + Big Side = Hemimegalencephaly
2. GM Heterotopias: “Nl Neurons in Abn Location”
• Follows GM on all sequences
• # in periventricular location & centrum semiovale
• Types:
3. Lissencephaly-Pachygyria Spectrum: smoothness of
brain surface
Agyria = no gyri
Pachygyria = broad gyri
Lissencephaly = smooth brain surface
a. Type 1 /Classic:
• Smooth Brain. Dt arrest of migration
• “figure 8”, “H. glass”, “vertically oriented shallow Sylvian fissures”
• Ass. with band heterotopias
b. Type II cobblestone brain
• Dt over migration
• Cortex is thinner than type I
• No band heterotopia
4. Schizencephaly:
• CSF cleft lined with GM connecting subarachnoid space with ventricles
• Types: Open Lip (80%) & Closed Lip (20%)
• Ass:
- Optic Nerve Hypoplasia (30%)
- Absent Septum Pellucidum (70%)
- Epilepsy (demonic possession) (50-80%)
Open Lip Schizencephaly Porencephalic Cyst
Lined with GM Lined by WM
True malformation False malformation
5. Hydranencephaly:
• Destruction of cerebral hemispheres. skull is bag of CSF
• Causes:
- Vascular: in utero insult (double MCA infarct)
- TORCH: causing necrotizing vasculitis (HSV)
• Normal brain & falx, but no GM

36
 CNS notes

IV. Herniation $ Vocab

Chiari Malformations: small post. Fossa Herniation:
a. Type 1: headache - Cephaloceles Brain
• Cerebellar tonsil herniates > 5mm below - Meningocele: Meninges
foramen magnum - Meningoencephalocele: Mrain + Meninges
• Crowding of post. fossa
• Craniosynostosis
• Ass with: ‫شبكة‬
- Syringohydromyelia: 50%; (LMN in UL & UMN in LL)
- HC: 25%
- Basilar invagination: 30%
- Klippel-Feil $: 10%
- Atlantooccipital fusion: 5%
b. Type 2:
• Vermis herniates into foramen magnum
• More complicated (>20 findings)
• Towering Cerebellum, Tectal Plate Beaking
• Long Skinny 4th Vent (elongated craniocaudally, short in other dimensions)
• Hypoplastic/ fenestrated falx
• Lemon sign: flat inwardly scalloped both frontal bones & post curve of cerebellum
• Ass with: ‫موساد‬
- Myelomeningocele: 90%
- Obs HC: 90%
- Syringohydromyelia: 50%
- Abnormal cortical gyration
- Dysgenesis of CC
b. Type 3: Chiari II + Encephalocele (high Cx or low occipital)
V. Craniosynostosis
Normal skull fusion:
• Anatomy:
o Brain double in size in 1st 6 m
o Double again by 2nd Y
o Sutures fuse 3 Y
o Petro-occipital remain open into adulthood
• Standard View is Townes’, AP & lat
• Causes:
o 1ry: (idiopathic)
o 2ry:
▪ Metabolic: rickets, hypercalcemia, hyperthyroidism, hypervitaminosis D
▪ Hematological: thalassemia, SCD
▪ Bone dysplasia: achondroplasia, metaphyseal dysplasia
▪ Following shunt
▪ $:
- Aperts: Brachycephaly + Fused Fingers
- Crouzons: Brachycephaly + 1st Arch Hypoplasia (Maxilla &Mandible)
- Cleidocranial Dysostosis: Brachycephaly + Wormian Bones + Absent Clavicles
o Sagittal affects boys (80%), Coronal affects girls
o Lambdoid favors rt side (70%)
Premature Suture Closure:
Sagittal # Scaphocephaly (long & skinny)
Metopic Trigonocephaly (pointed forehead)
Brachycephaly
Bilat Coronal + Lambdoid “harlequin-eye”
Ass. with $
Unilat coronal or Lambdoid Plagiocephaly
Bilat coronal & Lambdoid Turricephaly (tower)
Sagittal, coronal & lambdoid Oxycephaly

Cephalhaematoma
37
 CNS notes

• Birth trauma
• After poor instrumentation & skull F. during delivery
• 1–2% of deliveries
• Weeks to months to resolve
• Not cross sutural lines
• + Ca
Caput succedaneum
• Localized scalp oedema
• Cross sutural lines
VI. Misc Peds Brain Conditions:
1. MELAS
• Mitochondrial disorder with lactic acidosis & stroke like episodes
• SPECT: increased lactate, decreased NAA
2. Leukodystrophies:
• Affect WM in kids
• All untreatable & fatal
• DW has no Dx or DD role
Head Size Age Territory Trivia
Metachromatic (#) Normal 1 -2y/5-7 Diffuse WM, tigroid appearanc Def of enzyme arylsulfatase A
5-10 y Symm occip & splenium WM Sex-linked reces (peroxisomal Enzyme def)
Adreno Begins in post central WM Only in boys
Normal
Leukodystrophy progresses to corticospinal tracts Adrenal gland insufficiency
& visual - auditory pathways High ACTH, Pigmentation
<5y Focal subcortical WM. BG & = subacute necrotiz encephalomyelopathy
Leigh Normal
periaqueductal GM Mitoch enz defect
Big (Great < 1 y Frontal WM Personality disord
Alexander
Alexander) Metaphorical speaking
<1y Inv: Bilat. SCU fibers/ Spare High NAA in MRS & Urine
Canavan Big int. & ext cap & CC Skin culture: aspartoacyclase def
Inv: GB spare putam & caudate

38
 CNS notes

VII. Phakomatoses
1- Neurofibromatosis
• # phakomatosis
• 50% AD, 50% spontaneous mutations
• Types
NF1 NF2 Dx of NF1
von Recklinghausen Bilateral acoustic neuroma At least 2 of the following:
disease 1. 6 cafe-au-lait spots
Peripheral NF Central NF 2. 2 neurofibromas of any type (or 1 plexiform type)
Chromosome 17 Chromosome 22 3. Ax or ing freckling
90% 10% 4. Optic glioma
Prominent Minimal 5. 2 Lisch nodules (pigmented iris hamartomas)
Hamartomas (iris) MISME: 6. Osseous lesion (sphenoid dysplasia or thinning of long bone
Optic nerve Gliomas Schwannoma cortex)
Malignancies: astrocytoma Meningiomas 7. First-degree relative with NF-1
Malignant nerve sh tum Ependymoma
Wilms’
Rhabdomyosarcoma
Leukaemia
Thyroid CA
Pheochromocytoma
Spine: Spine:
NF Schwannoma
Scoliosis
Paraspinal masses
Widening of IV foramina
Post scalloping of VB
2- Tuberous sclerosis (Bourneville disease)
• AD (20%-50%) sporadic (50%) inherited (50%)
• Triad: adenoma sebaceum (facial angiofibroma), seizures & mental retardation
• Imaging:
o CNS (4 major lesions)
1- Hamartoma, in:
- Cortical GM tubers
- Subependymal (candle drippings), near foramen of Monro
2- Tubers:
- Cortical location
- Ca (DD: CMV, toxoplasmosis)
- Non-calcified tubers: low T1, high T2; variable enhancement on MRI
3- Subependymal giant cell astrocytoma
- At foramen of Monro
- Obs HC
4- Disorganized WM lesions:
- Radial bands in cerebral hemisphere
- Near 4th vent in cerebellar hemispheres “perpendicular to ventricles”
o Kidney
▪ AML: 50%; multiple & bilat
▪ Multiple cysts
o Bone: 50%
▪ Multiple bone islands
▪ Periosteal thickening of long bones
▪ Bone cysts
o Other
▪ Pul LMAs
▪ Pneumothorax: 50%
▪ Chylothorax
▪ Cardiac rhabdomyomas: 5%
▪ Aortic aneurysm

39
 CNS notes

3- Von hippel-lindau (VHL)

• AD with 100% penetrance
• Ass with chromosome 3
• Imaging:
o Hemangioblastoma, 50%: Cerebellum (# site), BS, SC & Retina
▪ Multiple is Dx
▪ If solitary: no VHL
o Renal:
▪ Benign renal cysts: 60%
▪ RCC: 50% (bilat 65%, multiple 85%)
o Adrenal glands: Pheochromocytoma: 15% (bilat in 40%)
o Pancreas
▪ Multiple cysts: 70%
▪ Cystadenocarcinoma
▪ Islet cell tumor
o Scrotum: Epididymal cysts:10%
o Other
▪ Hepatic cysts, 20%
▪ Splenic cysts, 10%
• MRI is of choice. CT to evaluate kidneys, adrenals & pancreas
• Entire CNS must be imaged (brain & SC)
• Family screening is necessary
4- Sturge-weber-dimitri $
• Capillary venous angiomas of face & ipsilateral cerebral hemisphere
• Cl:
o Port-wine nevus of V CN dermatome (# V1), unilateral
o Seizures: 90%
o Mental retardation
o Ipsilat glaucoma
o Hemiparesis: 50%
• Imaging
o Tramtrack cortical Ca in parietal & occipital lobes
o Atrophic cortex with enlarged subarachnoid space
o Ipsilat thickening of skull & orbit
o Leptomeningeal venous angiomas & enhancement
o Ipsilateral choroid plexus: enlargement & enhancement
o Glaucoma (30%)ss

40