Pathoma Neurology Flashcards

1
Q

PNS develops from?

A

neural crest

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2
Q

CNS develops from?

A

wall of neural tube

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3
Q

ventricles & spinal canal develop from?

A

lumen of neural tube

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4
Q

neural tube defects?

A

lack of folate PRIOR to conception

detected by elevated AFP in amniotic fluid or maternal blood

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5
Q

frog like appearance of fetus

A

anencephaly; lacks swallowing centers in brain - polyhydramnios

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6
Q

meningocele vs meningomyeleocele?

A

meningocele = meninges alone coming out; meningomyeleocele = meninges + spinal cord

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7
Q

what is spina bifida?

A

failure of posterior vertebral arch to close leading to vertebral defect, with cystic protrusion of underlying tissue through the defect

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8
Q

how does spina bifida occulta present?

A

dimple or patch of hair over vertebral defect

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9
Q

what is cerebral aqueduct stenosis?

A

congenital stenosis of the channel that drains cerebrospinal fluid from 3rd to 4th ventricle –> hydrocephalus

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10
Q

Where is CSF produced?

A

choroid plexus lining the ventricles

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11
Q

describe the flow of the CSF

A

from lateral ventricles to the 3rd ventricle via interventricular foramen of monro then to the 4th ventricle via cerebral aqueduct. The CSF then goes into the subarachnoid space via foramina of Magendie and Luschka

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12
Q

How does cerebral aqueduct stenosis present?

A

enlargin head circumferance due to the dilation of the ventricles (cerebral sutures are not fused)

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13
Q

massively dilated 4th ventricle w/absent cerebellum?

A

Dandy-Walker malformation; failure to develop cerebellar vermis

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14
Q

congenital extension of cerebellar tonsils thru foramen magnum?

A

Arnold-Chiari malformation; associated: hydrocephalus, meningimyeleocele and syringomyelia

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15
Q

what is syringomyelia

A

cystic dilation of the spinal cord from trauma or Arnold-Chiari leading to C8-T1 damage.

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16
Q

How does syringomyelia present?

A

loss of pain & temperature sensation in UE, sparing of position & fine touch due to involvement of anterior white commissure of spinothalamic tract with dorsal column sparing

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17
Q

syrinx expansion in syringomyelia can lead to what other symptoms?

A

can damage anterior horn (motor) & lateral horn (sympathetic to face, Horner’s syndrome)

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18
Q

sx of lower motor neuron dysfxn

A

anterior horn dysfxn; poliomyelitis, Werdnig-Hoffman dz

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19
Q

Poliomyelitis is the result of what neuro defects?

A

anterior horn; LMN defects

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20
Q

what are lower motor neuron signs?

A

flaccid paralysis with muscle atrophy, fsciculations, weakness with decreased muscle tone, impaired reflexes and negative Babinski

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21
Q

Werdnig-Hoffman has what defects?

A

inherited degeneration of anterior horn motor neurons, autosomal recessive; floppy baby”, death w/in a few years after birth”

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22
Q

ALS has what defects?

A

UMN & LMN degeneration

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23
Q

patient presenting with motor weakness in hands - what differentiates between ALS vs syringomyelia?

A

ALS is motor only; syringomyelia would also have pain/temp deficits

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24
Q

what are the upper motor neuron signs?

A

spastic paralysis with hyperreflexia, increased motor tone and positive Babinski due to damage to the lateral corticospinal tract

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25
Q

what is defect in familial ALS?

A

zinc-copper superoxide dismutase mutation (important mechnaism of removing free radicals)

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26
Q

difficulty walking, loss of vibratory sense & proprioception, mm weakness in LE, loss of DTR?

A

Friedrich’s ataxia; degenerative disorder of cerebellum

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27
Q

what are characteristics of Friedrich’s ataxia?

A

degenerative disorder of cerebellum & spinal cord tracts; autosomal recessive, trinuc GAA repeat in frataxin gene; HYPERTROPHIC cardiomyopathy

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28
Q

what gene is defective in Friedrich Ataxia? What does it do?

A

frataxin gene, necessary for mitochondrial iron regulation; increase risk of free radicals

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29
Q

what is inflamed in meningitis?

A

leptomeninges - the pia & arachnoid matter

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30
Q

MCC meningitis in neonates?

A

GBS, E coli, Listeria monocytogenes

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31
Q

MCC meningitis in children & teenagers?

A

Neisseria meningitidis; enters thru nasopharynx

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32
Q

MCC meningitis in adults & elderly?

A

Strep pneumo

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33
Q

MCC meningitis in unvaccinated?

A

Haemophilus influenza

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34
Q

MCC viral meningitis?

A

Coxsackievirus

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35
Q

Meningitis in immunocompromised?

A

think of fungi

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36
Q

Meningitis presentation

A

headache, nichal rigidity, fever; photophobia, vomiting and altered mental status may present

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37
Q

doing LP what layers are pierced?

A

Dura & Arachnoid, NOT Pia between L4 and L5

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38
Q

LP w/lymphocytes & low CSF glucose?

A

Fungal

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39
Q

LP w/lymphcytes & nl CSF glucose

A

Viral

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40
Q

LP w/neutrophils & low glucose

A

Bacterial; positive gram stain & culture

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41
Q

death from meningitis occurs because of:

A

herniation secondary to cerebral edema; seen w/bacterial b/c of pus production, etc

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42
Q

hydrocephalus, hearing loss, & seizures w/meningitis are from?

A

the healing process, fibrosis; primarily seen w/bacterial meningitis

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43
Q

majority of Cerebrovascular Dz?

A

ischemic (85%); hemorrhagic is ~15%

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44
Q

type of CVA caused by hypoglycemia?

A

global ischemia; see w/repeated episodes (insulinoma)

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45
Q

what are the major etiologies of global cerebral ischemia?

A

low perfusion, acute decrease in blood flow, chronic hypoxia, repeated hypoglycemia

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46
Q

highly vulnerable areas of the brain?

A

pyramidal layers of the cortex; layers 3, 5, & 6 leading to laminar necrosis
pyramidal neurons of hippocampus
purkinje layer of cerebellum

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47
Q

cortical lamenar necrosis

A

lines of necrosis; pyramidal layers 3, 5, & 6

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48
Q

focal neural deficits < 24 hours?

A

TIA

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49
Q

what causes a thrombotic stroke?

A

rupture of atherosclerotic plaque

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50
Q

where does atherosclerosis usually develop?

A

branch points, bifurcation of internal carotid and middle cerebral artery

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51
Q

what is the MC source of emboli in an embolic stroke?

A

left side of heart, atrial fibrillation

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52
Q

pale vs hemorrhagic necrosis following ischemia?

A
pale = thrombotic (ruptured plaque, prevents successful lysis, periphery of cortex); 
hemorrhagic = embolic (embolus comes in, causes ischemia, is lysed, periphery of cortex)
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53
Q

MC site of embolic stroke?

A

middle cerebral artery

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54
Q

what is a lacunar stroke?

A

results from hyaline arteriolosclerosis; this occurs w/HTN or diabetes

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55
Q

MC site of lacunar stroke?

A

lenticulostriate vessels (feeding deep structures of the brain) - lake like areas of infarction on gross inspection

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56
Q

order of histologic appearance following ischemic stroke?

A
eosinophillic change: red neurons (~12 hours)
coagulative necrosis (24hrs): PMNs (1-3days), microglial cells (4-7days), granulation tissue (weeks 2-3)
--> fluid-filled cystic space surrounded by gliosis (reactive astrocytes)
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57
Q

what is intracebral hemorrhage? What is it caused by? Complication of? MC site?

A

bleeding into brain parenchyma; due to ruptured Charco-Bouchard microaneurysms of lenticulostriate vessels; complication of HTN; basal ganglia MC site

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58
Q

How does an intracerebral hemorrhage present?

A

severe headach, nausea, vomiting coma

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59
Q

Charcot-Bouchard aneurysms?

A

caused by hyaline arteriosclerosis; can result in intracerebral hemorrhagic stroke

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60
Q

see a bleed on bottom of the brain?

A

subarrachnoid hemorrhage; really only cause

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61
Q

How does subarrachnoide hemorrhage present?

A

worst headach of my life with nuchal rigidity

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62
Q

What do you see on lumbar puncture with subarachnoid hemorrhages?

A

xanthochromia (yellow hue due to bilirubin breakdown)

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63
Q

MCC subarachnoid hemorrhage?

A

berry aneurysms, other cuases are AV malformation and anticoagulated state

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64
Q

MC location of berry aneurysms? Assocaited with?

A

cause subarachnoid hemorrhage; MC located in anterior circle of willis, specifically anterior communciating artery; associated w/marfan & ADPKD

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65
Q

histology of berry aneurysm?

A

thin walled, lack media layer

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66
Q

trauma w/lens shape lesion on CT?

A

epidural hemotoma

67
Q

cause of epidural hematoma?

A

fractured temporal bone –> rupture of middle meningeal artery –> bleeding separates dura from the skull

68
Q

cause & pathophys of subdural hematoma?

A

trauma; tearing of BRIDGING veins between dura & arachnoid; increased risk w/atrophy (b/c brain has shrunken back)

69
Q

CT appearance of subdural hematoma

A

crescent shaped

70
Q

clinical course of subdural hematoma?

A

progressive neurologic signs; herniation is lethal complication

71
Q

sub-falcian herniation?

A

herniation of cingulate gyrus under falx cerebria (vertical); compress anterior cerebral artery –> infarction

72
Q

tonsillar herniation?

A

cerebellum thru foramen magnum; compresses brain stem –> cardiopulmonary arrest

73
Q

uncal herniation?

A

displacement of temporal lobe uncus under the tentorium derebelli leading to compression of CN III (down and out, dilated pupil), compression of PCA, rupture of paramedian artery –> Duret (brainstem) hemorrhage

74
Q

deficiency of arylsulfatase?

A

metachromatic leukodystrophy; can’t degrade myelin (which must be done regularly), accumulates in lysosomes of oligodendrocytes

75
Q

Krabbe disease?

A

leukodystrophy; deficiency of galactocerebroside beta-galactosidase; galactocerebroside accumulates in macrophages

76
Q

adrenoleukodystrophy?

A

impaired addition of CoA to long chain FA; FA accumulates damaging adrenal gland & white matter (X linked recessive)

77
Q

what cells are destroyed in MS? What is genetic susceptibility? Environmental trigger?

A

oligodendrocytes; HLA-DR2; more common in regions away from equator

78
Q

Multiple Sclerosis symptoms

A

Multiple Sclerosis; scanning speech - resembles drunk, brainstem; Blurred vision 1 eye - optic nerve; internuclear ophthalmoplegia - MLF, hemiparesis, lower extremity loss of sensation

79
Q

dx of MS?

A

MRI - plaques; LP - increased lymphocytes, increased Ig w/oligoclonal IgG bands, myelin basic protein

80
Q

oligoclonal IgG bands in CSF?

A

Multiple Sclerosis

81
Q

gross inspection of MS brain?

A

grey areas in white matter (demyelinating)

82
Q

tx MS?

A

acute - high dose steroids; slow progression - IFN-beta; immune suppression, natalizumab

baclofen for spasticity
catheterization, musc agonists for neurogenic bladder
opioids for pain

83
Q

subacute sclerosing panencephalitis?

A

measles infection of the brain (demyelinating); slow progressing, persistent - progressive, debilitating, leading to death; infection in infancy, signs arise years later

84
Q

viral inclusions w/in neurons (gray matter) & oligodendrocytes (white matter)?

A

measels; SSPE (subacute sclerosing panencephalitis)

85
Q

immunocompromised pt w/rapidly progressing neurological signs?

A

Progressive Multifocal Leukoencephalopathy (demyelinating); reactivation of latent JC virus; leads to death

86
Q

rapid IV correction of hyponatremia?

A

Central Pontine Myelinolysis; focal demyelination of pons; typically alcoholic / severely malnourished

87
Q

locked in syndrome?

A

only eyes can move; classic for Central Pontine Myelinolysis

88
Q

broadly, in neurodegenerative disorders where is the degeneration? Sx?

A

in the cortex (gray matter outside the white) - dementia; in the deeper brain, by ventricles (basal ganglia) –> movement problems

89
Q

MCC dementia?

A

Alzheimer’s

90
Q

clinical features of alzheimer’s

A

slow onset memory loss
loss of learned motor skills and language
changes in behavior and personality
mute and bedridden

91
Q

genetic associations with alzheimer’s

A

epsilon4 allele of APOE increases risk but epsilon2 decreases risk
familial cases: presenilin 1 and presenilin 2 mutations

92
Q

protein pathophys of alzheimer’s?

A

Amyloid Precursor Protein on chromosome 21; normally broken down to alpha; in alzheimer’s broken down to A-beta amyloid which cannot be broken down –> deposited in the brain

93
Q

what are neuritic plaques?

A

extracellular core comprised of Abeta amyloid with entangled neuritic processes

94
Q

what are neurofibrillary tangles?

A

intracellular aggregates of fibers composed of hyperphosphorylated tau protein which is a microtubule associated protein

95
Q

are there any focal neurlogic deficits with Alzheimer’s?

A

No (no pillrolling tremor; no chorea, etc) (not early)

96
Q

forms of alzheimer’s? risk factors?

A

Sporadic form - age, increased with E4 & decreased with E2; Early form - younger patients, familial (presenilin 1) & down syndrome - APP on C21 x3

97
Q

what trisomy has increased risk of alzheimer’s? why?

A

Down’s, APP (amyloid precursor protein) is on chromosome 21; most pts get AD by 40 yo

98
Q

what is familial cause of alzheimer’s?

A

mutation in presenilin 1

99
Q

diffuse cerebral atrophy?

A

Alzheimer’s; narrowing of gyri, widening of sulci; get hydrocephalus ex vacuo (dilation of ventricles)

100
Q

histologic findings of alzheimer’s?

A

neuritic plaques; a-beta plaques (extracellular) w/entrapped neuritic processes; & neurofibrrillary tangles (flame shaped edge”) in the cytoplasm, tau (microtubule associated) protein is hyperphosphorylated”

101
Q

where is A-beta plaque found?

A

extracellularly; amyloid is always extracellular

102
Q

vascular complication of alzheimers?

A

cereberal amyloid angiopathy; amyloid plaques can deposit around arteries, weaken them, predispose to hemorrhage

103
Q

characteristic of neurofibrillary tangles in alzheimer’s?

A

hyperphosphorylated tau protein, intracellular

104
Q

2nd MCC dementia? Pathophys?

A

Vascular Dementia; multifocal infarction & injury caused by HTN, atherosclerosis, or vasculitis

105
Q

degenerative dz of frontal & temporal cortex; sparing parietal & occipital lobes?

A

Pick Disease (picks frontal & temporal)

106
Q

dementia, & early behavioral & language symptoms?

A

Pick Disease; frontal - behavior; language - temporal

107
Q

ROUND aggregates of tau proteins?

A

Pick disease (recall alzhemier’s has the triangle shaped flames”)”

108
Q

effect of Dopamine on cortex thru Basal ganglia?

A

Increased movement; D1 - excitatory stimulus; D2 - disinhibition

109
Q

Parkinson’s disease has loss of what?

A

dopaminergic neurons in substantia nigra pars compacta (basal ganglia)

110
Q

illicit drug contaminant that can result in Parkinson’s?

A

MPTP

111
Q

key risk factor in Parkinson’s?

A

aging; don’t know the exact cause though

112
Q

clinical features of Parkinson’s?

A

TRAP - tremor, rigidity, akinesia/bradykinesia, postural instability/shuffling gate

113
Q

histological features of Parkinson’s

A

loss of pigmented neurons in substantia nigra and round, eosinophillic inclusions of alpha-synuclein (Lewy bodies) in affected neurons

114
Q

lewy body? Composed of?

A

round eosinophilic inclusions in pars compacta of substantia nigra; Parkinson’s; composed of alpha-synuclein

115
Q

does Parkinsons’s have dementia?

A

not early (late)

116
Q

lewy body dementia - lewy bodies are located where? Sx?

A

cortical lewy bodies; early onset dementia w/hallucinations & parkinson like features

117
Q

loss of gabergic neurons in caudate nucleus?

A

(basal ganglia) Huntington’s - chorea; lose inhibitory hold on cortex - random firing of system

118
Q

grey matter structure indenting lateral ventricle?

A

caudate nucleus

119
Q

striatum is made of:

A

caudate + putamen, seperated by internal capsule; caudate is by lateral ventricle, putatmen is inferolateral

120
Q

gross inspection of huntingtons?

A

coronal section; loss of caudate nucleus (lateral ventricles enlarge b/c of ths)

121
Q

when does anticipation occur w/Huntington’s?

A

during spermatogenesis

122
Q

slow, involuntary snake-like” movement of fingers?”

A

Athetosis; Huntington’s

123
Q

urinary incontinence, gait instability, & dementia

A

classic triad of Normal Pressure Hydrocephalus; results from stretching of Corona Radiata w/hydrocephalus; Wet, Wacky, & Wobbly””

124
Q

how to tx Normal Pressure Hydrocephalus?

A

ventriculoperitoneal shunt (CSF to peritoneum); notably, NPH improves w/lumbar puncture

125
Q

why is there increased CSF in nl pressure hydrocephalus?

A

decreased absorption of CSF into arachnoid granulations, unknown reason

126
Q

stretching of corona radiata?

A

wet, wobbly, wacky” - urinary incontinence, gait instability, dementia; see in normal pressure hydrocephalus”

127
Q

spongiform encephalopathy?

A

Prion dz; PrPc –> PrPsc; accumulates & creates vacuations in brain

128
Q

what form is pathologic prion protein in?

A

beta-pleated; converted from alpha form

129
Q

intracellular vacuoles?

A

spongiform encephalopathy; accumulation of pathologic proteins in vacuoles causing damage

130
Q

MCC spongiform encephalopathy?

A

CJD; usually sporadic

131
Q

EEG of CJD?

A

spike waves on EEG

132
Q

course of CJD?

A

rapid dementia (weeks to months); death w/in year; can get ataxia & startle myoclonus

133
Q

insomnia & exaggerated startle response?

A

familial fatal insomnia; inherited form of spongiform encephalopathy

134
Q

MCC metastasis TO the brain?

A

Lung, Breast, Kidney cancer

135
Q

Metastasis or primary tumors of CNS more common?

A

equal, 50/50

136
Q

will primary CNS tumors metastasize?

A

No, not outside CNS (usually); are locally destructive

137
Q

MCC CNS tumor in adults? Type of cell?

A

Glioblastoma multiforma; astrocyte derived (glial cell = GFAP positive)

138
Q

where do CNS tumors in adults usually occur? Kids?

A

adults - supratentorial; kids - infratentorial

139
Q

most common tumors in children

A

pilocytic astrocytoma, ependymoma, and medulloblastoma

140
Q

tumor crossing midline of brain?

A

butterfly glioma, Glioblastoma Multiforma; MCC adult CNS tumor; derived from astrocytes”

141
Q

pseudopalisading necrosis”? Other histological feature?”

A

histological feature of GBM; central area of necrosis w/living cells sort of” lining up around (pseudopalisading); endothelial cell proliferation; derives from glial cell (astrocyte)”

142
Q

what is the intermediate filament present w/in glial cells? Why care?

A

GFAP - marker for type of tumor - eg GBM

143
Q

MCC benign tumor in adults?

A

Meningioma; Females > Males (note: cell expresses estrogen receptor)

144
Q

seizures in female woman w/round mass on imaging?

A

Meningioma; tumor compresses but does NOT invade the cortex

145
Q

whorled appearance” w/psammoma bodies on histology?”

A

Meningioma

146
Q

tumor of pt presenting w/tinnitus/loss of hearing - type? Marker? Association?

A

Schwannoma - benign; inovles CN or spinal nerves, esp CN 8 at cerebellopontine angle; S100 positive; bilateral seen in NF2

147
Q

bilateral acoustic schwanoma?

A

NF2

148
Q

calcified tumor in the white matter of frontal lobe?

A

Oligodendroglioma, malignant, present w/ seizures

149
Q

fried egg appearance”?”

A

classic for oligodendroglioma or MS

150
Q

cystic brain lesion w/nodule growing on wall?

A

pilocytic astrocytoma (piloCYSTic)

151
Q

astrocytes w/thick eosinophilic processes?

A

pilocytic astrocytoma; Rosenthaal fibers; children

152
Q

biopsy of pilocytic astrocytoma shows

A

rosenthaal fibers (thick eosinophillic processes of astrocytes and eosinophillic granular bodies

153
Q

S100?

A

Schwannoma (benign)

154
Q

GFAP positive?

A

glial derived - pilocytic astrocytoma; GBM

155
Q

medulloblastoma?

A

children; derived from granular cells of cerebellum; small round blue cells & Homer-Wright rosettes; poor prognosis, spreads via CSF

156
Q

tumor deriving from granular cells of cerebellum?

A

Medullablastoma (children, poor prognosis)

157
Q

small, round blue cells & Homer-wright rosettes?

A

medulloblastoma (children, poor prognosis)

158
Q

how does medulloblastoma spread?

A

via CSF, 4th ventricle –> spinal cord

159
Q

drop metastasis” of CNS lesion to spinal cord?”

A

Medullablastoma (children, poor prognosis)

160
Q

Ependymoma?

A

malignant, usually children, ependymal cells lining 4th ventricle; presents w/hydrocephalus

161
Q

perivascular pseudorosette?

A

ependymoma; blue cells lining up around a vessel

162
Q

bitemporal hemianopsia in child or young adult?

A

supratentorial mass –> optic chiasm compression; Craniopharyngioma, arising from epithelial remnants of Rathke’s pouch

163
Q

where do craniopharyngioma’s come from?

A

epithelial remnants of Rathke’s pouch (floor of mouth); seen in children, supratentorial mass

164
Q

craniopharyngioma on imaging? After resection?

A

calcifications on imaging; tends to recur after resection