Path - Demyelinating, Neurodegenerative, etc. Flashcards

1
Q

what disease process often follows a viral infection or sometimes a viral immunization?

A

acute disseminated encephalomyelitis (ADEM)

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2
Q

what disease process almost always follows an upper respiratory infection?

A

acute necrotizing hemorrhagic encephalomyelitis (ANHE)

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3
Q

list the inclusions seen in Huntington’s, Alzheimer’s, and parkinsons’

A

HD: expanded polyglutamine repeats

Alz: beta amyloid

PD: alpha-synuclein

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4
Q

describe the morphological hallmark of Alzheimer’s

A

global cortical atrophy starting in the frontal and temporal lobes –> then parietal lobes –> then occipital lobes

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5
Q

congo red stain will stain amyloid, and can be indicative of what dz processes

A
  • alzheimers’s

- cerebral amyloid angiopathy

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6
Q

compare morphology of pick disease and AD

A

pick: only frontal lobes are affected

AD: global

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7
Q

what is the demographic for progressive supranuclear palsy

A

men (2x more likely)

5th-7th decade

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8
Q

compare adult and juvenile Parkinson disease in terms of inheritance and protein

A

adult: autosomal dominant, alpha-synuclein
juvenile: autosomal recessive, alpha-synuclein and parkin

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9
Q

morphology of brain of pt with HD

A

brain is small

  • decreased body of corpus callosum
  • decreased head of caudate nucleus
  • decreased globus pallidus
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10
Q

what is anticipation and how does it affect patients with HD

A

repeat expansions during spermatogenesis lead to earlier onset fo the disease (juvenile form)

  • inherited through the father
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11
Q

describe the pathogenesis of HD

A

there is loss of medium spiny striatal neurons that leads to dysregulation of basal ganglia circuitry that modulates motor output

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12
Q

failure to remove cells with DNA damage is characteristic of what dz process

A

ataxia telangiectasia

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13
Q

SOD1 (superoxide dismutase) mutation is associated with what dz process

A

ALS

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14
Q

bunina bodies are associated with what dz process

A

ALS

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15
Q

decreased side of anterior horn neurons is associated with what disease process

A

ALS

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16
Q

protein and location for Alzheimer

A

protein: beta amyloid and tau
location: extracellular neurons

17
Q

protein and location for progressive supra nuclear palsy

A

Protein: Tau

location: neurons and glia

18
Q

protein and location for Parkinson

A

Protein: alpha-synuclein

location: neurons

19
Q

protein and location for ALS

A

Protein: TDP-43, SOD-1

location: neurons

20
Q

what gene and enzyme are affected in Tay-Sachs

A

gene: HEXA
enzyme: hexosaminidase A (can’t degrade GM2 gangliosides)

21
Q

age of presentation of Tay-sachs

A

about 1 year old, then death around ages 2-3

22
Q

cherry red spots in maculae is indicative of what dz

A

tay sachs

23
Q

in mitochondrial encephalopathies, what is the most commonly affected tissue, and what is the second

A

first: muscle
second: CNS

24
Q

sx of kearn-sayre syndrome

A

cerebellar ataxia

progressive external ophthalmoplegia

pigmentary retinopathy

cardiac conduction defects

progressive inability to move the eyes and eyebrows

25
Q

histologic hallmarks of kearn-sayre syndrome

A

spongiform change in the gray and white matter and neuronal loss most evidently in the cerebellym

26
Q

progressive inability to move the eyes and eyebrows indicates what dz

A

kearn-sayre syndrome

27
Q

what causes beriberi dz

A

thiamine deficiency (B1)

28
Q

pseudolaminar necrosis of the cortex could be indicative what of what problem

A

hypoglycemia