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Wrap Up Block > Path: Chapter 22: CNS Pathology > Flashcards

Path: Chapter 22: CNS Pathology Flashcards

1
Q

‘fried egg’ cells in a brain tumor

A

oligodendroglioma

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2
Q

biopsy shows: “sheets of regular cells with spherical nuclei containing finely granular chromatin surrounded by a clear halo of cytoplasm”

A

oligodendroglioma

“fried egg” cells

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3
Q

Genetics of oligodendroglioma?

A

most common genetic findings are deletions of 1p and 19q, typically occuring together.

tumors w/ these deletions are usually highly responsive to chemo and radiotherapy

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4
Q

calcification of a CNS tumor

A

calcification is present in 90% of oligodendrogliomas

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5
Q

populations of neurons most susceptible to global cerebral ischemia?

A
  • pyramidal neurons of hippocampus and neocortex

- Purkinje cells of cerebellum

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6
Q

Intracellular vs. extracellular lesions of Alzheimer’s?

A

intracellular - hyperphosphorylated tau forms neurofibrillary tangles

extracellular - beta amyloid plaques

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7
Q

Order of brain areas in which pathologic changes are observed in Alzheimer’s?

A

first in ERC, then in hippocampal formation, finally in the neocortex

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8
Q

Neuromyelitis Optica?

A

inflammatory demyelinating dz centered on optic nerves and spinal cord.

antibodies to AQ4

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9
Q

Presentation of CPM?

A

central pontine myelinolysis;

because of the involvement of fibers in the pons carrying motor signals to motor neurons in spinal cord, pts often present w/ rapidly evolving quadriplegia

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10
Q

What is in a Lewy body?

A

alpha-synuclein, neurofilaments and ubiquitin

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11
Q

Genetics of Parkinson’s?

A

can be caused by mutations in alpha-synuclein, parkin (an E3 ubiquitin ligase), and UCHL-1, an enzyme involved in recycling of ubiquitin from proteins targeted to the proteasome)

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12
Q

Lewy bodies may be found where in Parkinson’s even before nigral involvement?

A

dorsal motor nucleus of vagas and in reticular formation

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13
Q

Most common primary sites of brain mets?

A

lung, breast, skin (melanoma), kidney, GI tract

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14
Q

What is the Syndrome of Rapid-Onset psychosis, cataonia, epilepsy, and coma?

A

a paraneoplastic syndrome associated w/ ovarian teratoma and antibodies against the NMDA receptor

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15
Q

Genetics of glioblastoma?

A

loss-of-function in p53 or Rb

gain-of-function in PI3K

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16
Q

Genetics of lower-grade astrocytomas?

A

mutations altering enzymatic activity of isocitrate dehydrogenase (IDH1, IDH2)

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17
Q

Immunostaining for mutated forms of IDH1 has become an important diagnostic tool in evaluating biopsy specimens for the presence of…

A

low-grade astrocytoma

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18
Q

Genetics of Tuberous Sclerosis?

A 
disruption of either TSC1 (encodes hamartin)
                      or    TSC2 (encodes tuberin)

the two TSC proteins form dimeric complex that negatively regulates mTOR, a kinase that “senses” cell’s nutrient status and regulates cellular metabolism.

increased mTOR activity disrupts nutritional signalling and increases cell growth

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19
Q

increased mTOR activity is central to…

A

tuberous sclerosis

and everything that it causes

20
Q

What are shagreen patches?

A

leathery thickenings in localized patches seen in Tuberous sclerosis

21
Q

What are ash leaf spots?

A

hypopigmented areas seen in tuberous sclerosis

22
Q

Triad of CNS toxoplasmosis?

A

hydrocephalus, chorioretinitis, intracranial calcifications

23
Q

What bug causes neurocysticerosis?

A

Tenia Solium

pork tapeworm; typically presents as a mass lesion

24
Q

Recurrent HSV-1 encephalitis may be caused by what genetic problem?

A

mutations involving TLR3

25
Q

What do you call the intranuclear inclusions of HSV-1?

A

Cowdry type A bodies

26
Q

Which type of prion disease features abundant amyloid plaques?

A

variant CJD

27
Q

Which type of prion disease can be spread by blood transfusions?

A

variant CJD

28
Q

Lesions of the _____________ seem to best correlate with the memory loss observed in Korsakoff syndrome.

A

medial dorsal nucleus of the thalamus

29
Q

What are Alzheimer type II cells?

A

not related to Alzheimer’s!

they are a consequence of hepatic encephalopathy and the resultant hyperammonemia. an Alzheimer type II cell is an astrocyte in the cortex and/or basal ganglia w/ swollen, pale nuclei due to lots of ammonia processing

30
Q

Timeline of healing for subdural hematoma?

A

lysis of the clot (1 week)
growth of granulation tissue from dural surface into hematoma (2 weeks)
fibrosis (1 - 3 months)

31
Q

Most common sites for Berry aneurysm?

A

40% - junction of AComm and ACA
34% - junction of M1 of MCA and M2 of MCA
20% - juntion of internal carotid and PComm

32
Q

Only one type of aneurysm is found more commonly in posterior circulation:

A

atherosclerotic aneurysm (in the basilar artery)

33
Q

Cytoplasmic inclusions in ALS are composed of what?

A

TDP-43 (except in those cases of ALS caused by a mutation in SOD-1 [super oxide dismutase -1]

34
Q

What does neurofibromin normally do?

A

neurofibromin is a negative regulator of the oncoprotein Ras

neurofibromin is on chromosome 17 and is mutated in the condition Neurofibromatosis 1

35
Q

Cutaneous signs of Neurofibromatosis 1?

A

cafe-au-lait spots (areas of hyperpigmentation)

and Lisch nodules (pigmented nodules of the iris)

36
Q

Which artery may be compressed by an uncal herniation?

A

PCA

and don’t forget about kinking of basilar artery -> Duret hemorrhages

37
Q

There is an association b/t meningiomas and what congenital CNS-tumor disorder?

A

NF2

mutation on chromosome 22. even 50% of sporadic meningiomas have mutations in NF2 gene

38
Q

‘Midline tumor on cerebellar vermis’

A

medulloblastoma (typically kids get midline medullos, lateral tumors are seen in adults but this is rare)

39
Q

Genetics of medulloblastoma?

A

in general, tumors associated w/ MYC mutations have poor prognosis

tumors w/ WNT mutations have a better course

mutations in sonic hedgehog also occur but no one knows what this means

40
Q

Most common demyelinating disorder?

A

MS

41
Q

Polymorphisms in what cytokines may predispose to MS?

A

IL-2 and IL-7

and don’t forget about HLA-DR2

42
Q

Type of T cells involved in MS?

A

Th17 and Th1 cells

43
Q

In what form of dementia would you see microglial nodules w/ multinucleate giant cells?

A

AIDS

44
Q

Locations of ependymomas?

A

first 2 decades of life - 4th ventricle

adults - spinal cord

45
Q

Genetics of NF2?

A

loss of function mutation in merlin gene of chromosome 22

cytoskeletal protein that facilitates E-cadherin mediated contact inhibition

46
Q

Genetics of pilocytic astrocytoma?

A

BRAF mutations

often V600E

47
Q

Neurons most affected by hypoglycemia?

A

hippocampal pyramidal neurons most affected

purkinje neurons of the cerebellum relatively spared

Wrap Up Block (17 decks)

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