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BMB W11 > 10-22 L1 Neurodegenerative disease of myelin > Flashcards

10-22 L1 Neurodegenerative disease of myelin Flashcards

1
Q

Whats the most common cause of dementia?

A
  • Alzheimer’s disease (62%)
  • Multi-infarct dementia (17%)
  • Diffuse lewy body disease (4%)
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2
Q

Age related accumulations of cytoplasmic filaments are:

A
  • Neurofibrillary tangles
  • Neuritic (senile) plaques
  • Lewy bodies
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3
Q

Alzheimer’s disease

A
  • 2F:1M
  • Most cases are sporadic
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4
Q

Alzheimeris pt will be asymptomatic until how much neurons are left in the brain?

A

20%

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5
Q

What is gross changes in the brain are seen with AD?

A
  • Brain weight decrease (<1,000 g)
  • Cerebral cortical atrophy
    • Loss of gray and white matter
    • ventricular dilation
  • Brainstem & spinal cord normal
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6
Q

What are the 5 histological changes seen in the cerebral cortex and hippocampus in AD pts?

A
  • Neuritic plaques
  • Neurofibrillary tangles
  • Granulovacuolar degeneration
  • Congophilic angiopathy: Beta-amyloid
  • Hirano bodies
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7
Q

Name the three ways that AB is cleard from teh brain:

A
  1. Edocytosis (astrocytes and microglial cells)
  2. Enzymatic degradation
  3. Drained along periarterial spaces
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8
Q

Describe the frontal lobe degeneration (initial stage and late stage)

A
  • Initial stage
    • Overactive, restless, distractible, disinhibited
    • apathetic, inert, emotionally blunted
  • Late stage
    • Gradual reduction in speech (mutism)
    • Akinesia and rigidity
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9
Q

Huntington’s disease

  • chromosome
  • genetic identification
  • onset
  • gross discription
A
  • Chromosome 4 mutation
    • consists of an expanded unstable trinucleotide (CAG) repeats
  • greater the number of nucleotides greater the onset
  • marked atrophy of the caudate nucleus
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10
Q

Parkinson’s disease

  • pathology
  • treatment
A
  • Pathology
    • substantia nigra: affects dopamine-synthesizing neurons w/loss of balance between direct and indirect pathwyas
    • locus ceruleus
    • other brainstem nuclei
  • Treatment
    • Replacement with L-dopa
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11
Q

ALS

Amyotrophic lateral sclerosis

  • type of lesion
  • m v f
  • onset
  • cause
  • chromosome
    • codes for what
A
  • UMN & LMN
  • M>F
  • >40 yr
  • 90% sporadic, 10% familial
  • chromosome 21
    • superoxide mutase (SOD1)
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12
Q

Progressive bulbar palsy

A

if degeneration involves predominantly the cranial nuclei with resulting dysarthria, dysphasia, and respiratory compromise

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13
Q

Progressive muscular atrophy

A

predominant LMN involvement

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14
Q

Primary lateral sclerosis

A

predominant UMN/corticospinal tract involvement

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15
Q

What is the most common autosomal ataxia?

A

Friedreich’s ataxia

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16
Q

Friedreich’s ataxia

  • def
  • pathology
  • chromosome
    • genetic repeat sequence
A
  • a nuclear encoded protein active in the mitochondria where it is involved in iron homeostasis
  • mitochondrial iron accumulation
  • chromosome 9
    • GAA trinucleotide (200-1000 repeats)
17
Q

Whats the difference b/t Hereditary CJD and iatrogenic CJD?

A
  • Hereditary CJD (5-10%)
    • family hx
    • genetic mutation (+)
  • Iatrogenic CJD
    • Exposure to CNS tissue
      • usually through medical procedures
    • Transmitted vis Human GH treatment
    • Not transmittable through causal contact
18
Q

Fatal Familial insomnia (FFI)

A
  • degeneration of the thalamus
    • (via mutation of a gene and production of an infectious prion.
  • symptoms
    • can’t sleep
    • inability to feel pain
    • poor reflexes
    • dementia
19
Q

What stain is used to stain MS (Multiple sclerosis)

A

Luxol Fast Blue (LFB)

20
Q

Pts with long standing MS present with what?

A
  • cerebral atrophy
  • hydrocephalus ex vacuo
  • optic nerves and chiasm (may be grossly atrophic)
21
Q

What disease is commonly seen with ‘knife like’ gyri of the frontal lobe.

A

Pick’s disease

22
Q

Where is the most distribution of neuritic placues in the cerebral cortex

A

Parietal and temporal lobes

23
Q

Metachromatic leukodystrophy

  • Age
  • Genetics
  • Cellular derangement
  • Morphological feature
A
  • Age: children (1-4 yo)
  • Genetics: autosomal recessive
  • Cellular derangement: def. of aryl-sulfatase-A
  • Morphological feature: Metachromasia
24
Q

(Krabbe’s) Globoid cell leukodystrophy

  • Age
  • Genetics
  • Cellular derangement
  • Morphological feature
A
  • Age: Infants (<1 yr)
  • Genetics: autosomal recessive
  • **Cellular derangement: **def. of galactocerebroside-beta-galactosidase
  • Morphological feature: Globoid cells
25
Q

Adrenoleukodystrophey

  • Age
  • Genetics
  • Cellular derangement
  • Morphological feature
A
  • Age: Young males
  • Genetics: x-linked
  • Cellular derangement: defect in a 70KD peroxisomal membrane protein, prevents transport of LCFA into the peroxisomes for beta-oxidation.
  • Morphological feature: Large plaques

BMB W11 (9 decks)

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