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Neuro Week 2 > White Matter Diseases > Flashcards

White Matter Diseases Flashcards

1
Q

what happens to myelin in MS?

A

degenerates

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2
Q

what happens to axons in MS?

A

nothing

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3
Q

what cells are lost in MS?

A

oligodendrocytes

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4
Q

what is imaging used for MS?

A

MRI

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5
Q

do acute or chronic lesions enhance on an MRI with contrast?

A

acute

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6
Q

what drives MS?

A

IgG antibodies…autoimmune disease

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7
Q

where can IgG abs be found in MS?

A

CSF only…none in blood

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8
Q

name four ways MS can present (specific symptoms0

A

monocular vision loss
ataxia
limb weakness or sensory loss
double vision

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9
Q

MS presenting with monocular vision loss is caused by demyelination where?

A

optic nerve (optic neuritis)

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10
Q

MS presenting with ataxia is caused by demyelination where?

A

cerebellum

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11
Q

MS presenting with double vision is caused by demyelination where?

A

brainstem

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12
Q

MS presenting with muscle loss and sensory weaknes is caused by demyelination where?

A

spinal cord

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13
Q

what is acute Rx for MS?

A

3-5 day course of steroids

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14
Q

over time what happens with MS? 3 things

A

gate difficulty
loss of bladder control
impaired cognitive function

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15
Q

what is a cardinal pathologic finding with MS around veins?

A

perivascular inflammation

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16
Q

where do you see changes in MS on MRI most often?

A

periventricular plaques…right around the ventricles the MRI will be bright white

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17
Q

what is ADEM?

A

acute disseminated encephalomyelitis

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18
Q

what causes acute disseminated encephalomyelitis?

A

post infection or vaccine

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19
Q

what happens in acute disseminated encephalomyelitis?

A

immune mediated demyelination and polyfocal neuro deficits

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20
Q

what are common viral infections that lead to acute disseminated encephalomyelitis?

A

measles

varicella and rubella

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21
Q

what are the two patho findings in acute disseminated encephalomyelitis?

A

demyelination and perivenous inflammatory infiltrates

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22
Q

what is PML?

A

progressive multifocal leukoencephalopathy

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23
Q

what causes progressive multifocal leukoencephalopathy?

A

JC virus

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24
Q

who gets progressive multifocal leukoencephalopathy?

A

immunocompromised

25
Q

what happens in progressive multifocal leukoencephalopathy?

A

destruction of oligodendrocytes

26
Q

what lobes does progressive multifocal leukoencephalopathy prefer

A

usually temporal and occipital

27
Q

since progressive multifocal leukoencephalopathy prefers temporal and occipital, what symptom is therE?

A

vision changes

28
Q

what white matter is destroyed in progressive multifocal leukoencephalopathy?

A

subcortical white matter

29
Q

what can you see in oligodendrocyte in progressive multifocal leukoencephalopathy?

A

intranuclear inclusions of the JC virus

30
Q

what is AIDS leukoencephalopathy?

A

diffuse pallor of cerebral white matter on myelin stain with little evidence of myelin degeneration

31
Q

what is seen in histo stain for AIDS leukoencephalopathy?

A

multinucleated giant cells

32
Q

what do they think plays role in AIDS leukoencephalopathy development?

A

toxic cytokines from activated microglia

33
Q

what is another name for central pontine myelinolysis?

A

osmotic myelinolysis

34
Q

what happens in central pontine myelinolysis?

A

rapid axonal demyelination in pons

35
Q

what is cause of central pontine myelinolysis usually?

A

rapid osmotic changes

36
Q

what is iatrogenic cause of central pontine myelinolysis?

A

correction of hyponatremia

37
Q

what are symptoms of central pontine myelinolysis?

A

acute paralysis
dysphagia
diplopia
loss of consciousness

38
Q

what is leukodystrophy?

A

inherited metabolic disease affecting white matter

39
Q

what genes cause leukodystrophy?

A

genes that are important for myelin production or myelin lipid metabolism

40
Q

where is demyelination in leukodystrophy?

A

CNS and PNS

41
Q

what are three leukodystrophys to know?

A

ALD MLD and GLD

42
Q

what do macros in ALD have in them?

A

long chain fatty acid

43
Q

what do macros in MLD have in them?

A

sulfatide…

44
Q

what do macros in GLD have in them?

A

galactocerebreside

45
Q

describe the progression of leukodystrophies

A

progressive and symmetric

46
Q

what is inheritance of ALD?

A

x linked recess

47
Q

where does demyelination primarily occur in ALD?

A

occipital white matter and brainstem/spinal cord

48
Q

what fibers are always preserved in leukodystrophy?

A

subcortical U fibers…just under the cortex of the brain

49
Q

what do macros look like in ALD?

A

stuffed with lipids

50
Q

what is inheritance of MLD?

A

AR

51
Q

what is MLD an issue with?

A

lysosomal storage disease

52
Q

what causes destruction of myelin in MLD?

A

cerebroside sulfate

53
Q

what are symptoms of MLD?

A

dementia and ataxia

54
Q

what is another name for GLD?

A

Krabbe disease

55
Q

what is inheritance of GLD?

A

AR

56
Q

what causes demyleination in GLD?

A

accumulation of galactocerebriside

57
Q

what are symptoms of GLD?

A

DD, peripheral neuropathy

optic atrophy

58
Q

what can happen post radiation for brain tumor?

A

get demyelination and radiation necrosis…this is not a new tumor but lights up white on MRI

Neuro Week 2 (14 decks)

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