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
Q

Polio tropism

A

brainstem nuclei

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26
Q

CMV pathology

A

necorsis and calcification. Intranuclear inclusions distinguish from toxo

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27
Q

Somner’s sector of hippocampus

A

one of first hit in global ischemia

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28
Q

TB tropism

A

base of brain, striate arteries

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29
Q

Krabbe dx

A

def of galactocerebroside b-galactoside. Globoid cells –> macrophages with undigested galactocerebricide. severe motor, sensory, and cog defects. fatal.

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30
Q

Alexander dx

A

GFAP –> condense to rosethal fibers

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31
Q

Hurler dx

A

accum of mucopolysacchardies (GAGs). • shortness, corneal opacities, skeletal deformities, hepatosplenomegaly

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32
Q

Cortical degenerative diseases

A

AD, Pick dx, PD, Lewy body dementia

33
Q

knife edge atrophy

A

pick dx

34
Q

Metachromatic leukodystrophy

A

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
Q

galactocerebroside b-galactoside

A

Krabbe

36
Q

arylsulfatase A

A

metachromatic leukodystrophy

37
Q

Adrenoleukodystrophy

A

adrenal cortex and nervous system demyelination. assoc. with high levels of very long chain fatty acids.

38
Q

Cingulate herniation complications

A

ACA displaced beneath falx –> infacrtion –> contralateral lower extremity weakness and urinary incont.

39
Q

Uncal herniation complications

A

ipsilateral CN III crushed –> ipsilateral pupillary dilation and fixed

40
Q

Cerebellar tonsillar herniation

A

compressed brainstem – resp. centers – bad news bears.

41
Q

Gaucher dx

A

glucocerebroside defect –> accumulates in macrophages. Neronal loss –> diffuse astrogliosis

42
Q

Neimann-Pick dx

A

intraneuronal storage of sphigomyelin.

43
Q

PKU

A

phenyalanine accumulates b/c conversion to tyrosine blocked. Early onset. Mental retardation, seizures, impared physical development. Tx. is restricted diet

44
Q

Wilson’s dx

A

hepatolenticular degeneration. Defective excretion of copper in bile –> deposits in brain. Movement disorder with choreoathetosis

45
Q

Opthalmoplegia, ataxia, confusion

A

wernicke’s syndrome from thiamine (b1) def.

46
Q

Syringomyelia

A

cystic enlargement of central canal of spinal cord. cape like bilateral loss of pain and temp in upper extremities. horner’s syndrome

47
Q

Loss of Oligodendroglia

A

MS

48
Q

Loss of Schwann cells

A

Guillain-Barre syndrome and acoustic neuroma (CN VIII)

49
Q

wet wobbly and wacky

A

normal pressure hydrocephalus

50
Q

hydrocephalus ex vacuo

A

increase in CSF due to brain paranchyma atrophy (in AD, advanced HIV, Pick’s disease)

51
Q

left hemianopia with macular sparing

A

PCA infarct of occipital lobe (sparing b/c macula supplied bilaterally)

52
Q

left homonymous hemianopia

A

right optic tract before geniculate nucleus

53
Q

bitemproal hemianopia

A

optic chiasm (pituitary tumor/ cyst). Craniopharyngioma (benign childhood tumor, derived from remnants of Rathke’s pouch.

54
Q

Guillain-Barre

A

autoimmune. symmetric ascending muscle weakness/paralysis beginning in lower extremitiesa)

55
Q

Craniopharyngioma

A

benign childhood tumor, derived from remnants of Rathke’s pouch. bitemporal hemianopia.

56
Q

Hemangioblastoma

A

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
Q

Vasogenic vs cytotoxic edema

A

VE - most common, bbb loosens due to neoplasms, abscesses, meningitis. CE - water is driven across BBB by osmotic forces

58
Q

disruption of myelin production

A

leukodystrophies

59
Q

alzheimer type II astrocytes

A

altered astrocytes in hepatic encephalopathy. enlarged nuclei

60
Q

rosettes

A

histo feature of neroblasts.

61
Q

von Hippel-Lindau syndrome

A

hereditary disorder in which cerebellar hemangioblastomas are associated with retinal angiomas and other tumors due to mutations in the VHL tumor suppressor gene.

62
Q

Glioblastoma multiforme

A

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
Q

perivascular pseduorosettes

A

ependymoma

64
Q

Kayser-Fleischer rings

A

depostion of copper in descemet membrane of iris. wilson disease

65
Q

antibody against myelin basic protein

A

MS

66
Q

periventricular calcification is caused by what virus

A

CMV (congenital)

67
Q

histo findings of HIV encephalitis

A

multinucleatd microglial cells

68
Q

medulloblastoma

A

kids. neuroectodermal tumors. cerebellum-midline vermis. drop metastasis. small round blue cells.

69
Q

pilocytic astrocytoma

A

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
Q

Charcot-Marie Tooth disease

A

most common hereditary neuropathy. peroneal nerve neuropathy - cause atrophy of muscles of lower legs

71
Q

Werdnig-Hoffman Disease

A

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
Q

Freidrich’s ataxia

A

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
Q

Cafe au lait spots associated with

A

NF1. Schwanommas too

74
Q

Gray white junction lesions

A

metastatic cancers

75
Q

Failure to generate myelin but no DISCRETE plaques like in MS =

A

leukodystrophies

76
Q

Cerebral contusion

A

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
Q

Brown-Sequards syndrome

A

hemisection of spinal cord

78
Q

SOD1

A

ALS

79
Q

Oligodendroglia are injured in (x3)

A

MS, progressive, multifocal leukoencephalopathy (PML), leukodystrophies