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CNS Pathology Flashcards

1
Q

Negri bodies

A

Rabies (enters via peripheral nerves) Negri bodies in purkinji fibers

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2
Q

NFT

A

Alzhiemer

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3
Q

Presenile

A

Alzheimer

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4
Q

Lissencephaly

A

Congenital disorder with smooth cortical surface due to lack of gyri. White matter contains clusters of neurons that could not migrate to gray matter/cortex

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5
Q

holoprosencephal

A

trisomy chr 13 - 15

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6
Q

Subacute combined degeneration

A

B12 def, pernicious anemia – degeneration of posterior-lateral part of cord. Get burning in feet as early sign

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7
Q

Triplet expansion diseases

A

HD, fragile x, freidreich ataxia

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8
Q

Whorled myelin

A

undigested ganglioside in tay sachs

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9
Q

Psammoma bodies

A

Meningiomas

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10
Q

Hirano bodies

A

Rod shaped and crystal like. Made of actin. AD and CJ encephalopathy

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11
Q

Thiamine def.

A

rapid onset of thermal dysreg, altered consiousness, opthalmoplegia, nystagumus, ataxia. (usually in hypothal, MAMILLARY bodies, PAG, and tegmental area)

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12
Q

Spasms of throat

A

Rabies

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13
Q

Hydrophobia

A

Rabies

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14
Q

Potine myelinolysis

A

in chronic alcoholics. Overly rapid correction of hyponatriemia in alcoholics and malnourished

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15
Q

Encephalomalacia

A

after brain infarcts

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16
Q

Duret Hemmorrhages

A

secondary hemmorrhages in pons and cerebellum due to herniation (uncal and tonsillar)

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17
Q

Subdural hematoma

A

from veins (sinus usually)

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18
Q

Epidural hematoma

A

lens shaped, from meningeal arteries

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19
Q

Subarachnoid hemorrhage

A

cerebral arteries or aneurysm rupture (marfan’s or Ehlers-Danlos). Worse headache of their life. Spinal tap is bloody or yellow (xanthochromic)

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20
Q

Pick bodies

A

Tau Protiens. frontotemporal dementia/ atrophy

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21
Q

Lewy bodies

A

alpha-synuclein in PD (in substania nigra) and lewy body dementia (in cortex)

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22
Q

Hexosamindase A

A

tay sachs

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23
Q

Cherry red spots in eye

A

increased macular transparency in tay sachs (and Nieman-pick)

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24
Q

HSV tropism

A

temporal lobe

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25
Polio tropism
brainstem nuclei
26
CMV pathology
necorsis and calcification. Intranuclear inclusions distinguish from toxo
27
Somner's sector of hippocampus
one of first hit in global ischemia
28
TB tropism
base of brain, striate arteries
29
Krabbe dx
def of galactocerebroside b-galactoside. Globoid cells --> macrophages with undigested galactocerebricide. severe motor, sensory, and cog defects. fatal.
30
Alexander dx
GFAP --> condense to rosethal fibers
31
Hurler dx
accum of mucopolysacchardies (GAGs). • shortness, corneal opacities, skeletal deformities, hepatosplenomegaly
32
Cortical degenerative diseases
AD, Pick dx, PD, Lewy body dementia
33
knife edge atrophy
pick dx
34
Metachromatic leukodystrophy
accum of cerbroside (galactosyl sulfatide) caused by def in arylsulfatase A --> involved in degredation of myelin sulfatides --> accumulated in schwann and oligodendrocytes...impaired production of myelin sheath.
35
galactocerebroside b-galactoside
Krabbe
36
arylsulfatase A
metachromatic leukodystrophy
37
Adrenoleukodystrophy
adrenal cortex and nervous system demyelination. assoc. with high levels of very long chain fatty acids.
38
Cingulate herniation complications
ACA displaced beneath falx --> infacrtion --> contralateral lower extremity weakness and urinary incont.
39
Uncal herniation complications
ipsilateral CN III crushed --> ipsilateral pupillary dilation and fixed
40
Cerebellar tonsillar herniation
compressed brainstem -- resp. centers -- bad news bears.
41
Gaucher dx
glucocerebroside defect --> accumulates in macrophages. Neronal loss --> diffuse astrogliosis
42
Neimann-Pick dx
intraneuronal storage of sphigomyelin.
43
PKU
phenyalanine accumulates b/c conversion to tyrosine blocked. Early onset. Mental retardation, seizures, impared physical development. Tx. is restricted diet
44
Wilson's dx
hepatolenticular degeneration. Defective excretion of copper in bile --> deposits in brain. Movement disorder with choreoathetosis
45
Opthalmoplegia, ataxia, confusion
wernicke's syndrome from thiamine (b1) def.
46
Syringomyelia
cystic enlargement of central canal of spinal cord. cape like bilateral loss of pain and temp in upper extremities. horner's syndrome
47
Loss of Oligodendroglia
MS
48
Loss of Schwann cells
Guillain-Barre syndrome and acoustic neuroma (CN VIII)
49
wet wobbly and wacky
normal pressure hydrocephalus
50
hydrocephalus ex vacuo
increase in CSF due to brain paranchyma atrophy (in AD, advanced HIV, Pick's disease)
51
left hemianopia with macular sparing
PCA infarct of occipital lobe (sparing b/c macula supplied bilaterally)
52
left homonymous hemianopia
right optic tract before geniculate nucleus
53
bitemproal hemianopia
optic chiasm (pituitary tumor/ cyst). Craniopharyngioma (benign childhood tumor, derived from remnants of Rathke's pouch.
54
Guillain-Barre
autoimmune. symmetric ascending muscle weakness/paralysis beginning in lower extremitiesa)
55
Craniopharyngioma
benign childhood tumor, derived from remnants of Rathke's pouch. bitemporal hemianopia.
56
Hemangioblastoma
most often cerebellar. Associ with von-Hippel-Lindau syndrome when found with retinal angiomas. Can produce EPO -- secondary polycythemia. High vascularity. Often found in kids
57
Vasogenic vs cytotoxic edema
VE - most common, bbb loosens due to neoplasms, abscesses, meningitis. CE - water is driven across BBB by osmotic forces
58
disruption of myelin production
leukodystrophies
59
alzheimer type II astrocytes
altered astrocytes in hepatic encephalopathy. enlarged nuclei
60
rosettes
histo feature of neroblasts.
61
von Hippel-Lindau syndrome
hereditary disorder in which cerebellar hemangioblastomas are associated with retinal angiomas and other tumors due to mutations in the VHL tumor suppressor gene.
62
Glioblastoma multiforme
invasive properties and vascular changes (artertitic obliteration) the tumors have patchy yellow areas of necrosis and red zones of hemorrhage. histo display palisading tumor cells (multi nucleated) around necrotic area
63
perivascular pseduorosettes
ependymoma
64
Kayser-Fleischer rings
depostion of copper in descemet membrane of iris. wilson disease
65
antibody against myelin basic protein
MS
66
periventricular calcification is caused by what virus
CMV (congenital)
67
histo findings of HIV encephalitis
multinucleatd microglial cells
68
medulloblastoma
kids. neuroectodermal tumors. cerebellum-midline vermis. drop metastasis. small round blue cells.
69
pilocytic astrocytoma
most common cns tumor of kids. benign. cerebeller. rosenthal fibers. cyst is well circumscribed mass on MRI. often described as cystic with mural nodules. ataxia and clumsiness of limbs. sometimes associated with type 1 NF.
70
Charcot-Marie Tooth disease
most common hereditary neuropathy. peroneal nerve neuropathy - cause atrophy of muscles of lower legs
71
Werdnig-Hoffman Disease
spinal muscle atrophy. 2nd most common lethal, autosomal recessive disease in Caucasians neuron apoptosis inhibitor protein LOF --> loss of anterior horn cells --> muscle weakness and atrophy --> aspiration pneumonia and respiratory failure. infants can't sit on their own, can't suckle well and have trouble swallowing. severe cases don't live past 2 years. ATROPHY AND FASICULATIONS OF TONGUE
72
Freidrich's ataxia
most common cause of inherited ataxia. triplet expansion. degeneration of spinal cord -- ataxia, dysarthria, loss of refelxes, loss of sensation. associated hypertrophic cardiomyopathy and DB
73
Cafe au lait spots associated with
NF1. Schwanommas too
74
Gray white junction lesions
metastatic cancers
75
Failure to generate myelin but no DISCRETE plaques like in MS =
leukodystrophies
76
Cerebral contusion
Cause: Accel/Decel Injuries. Brain impacts bone. Seepage of blood Coup: On side of impact (Stationary headImpact) Contrecoup: Opposite side of impact. ( Moving head Stops) Patho: Permanent marks on brain Local scar formation (astrocytes) with residual hemosideren (orange brown color)
77
Brown-Sequards syndrome
hemisection of spinal cord
78
SOD1
ALS
79
Oligodendroglia are injured in (x3)
MS, progressive, multifocal leukoencephalopathy (PML), leukodystrophies

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