White Matter Diseases

Question 1

what happens to myelin in MS?

Answer 1

degenerates

Question 2

what happens to axons in MS?

Answer 2

nothing

Question 3

what cells are lost in MS?

Answer 3

oligodendrocytes

Question 4

what is imaging used for MS?

Answer 4

MRI

Question 5

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

Answer 5

acute

Question 6

what drives MS?

Answer 6

IgG antibodies…autoimmune disease

Question 7

where can IgG abs be found in MS?

Answer 7

CSF only…none in blood

Question 8

name four ways MS can present (specific symptoms0

Answer 8

monocular vision loss
ataxia
limb weakness or sensory loss
double vision

Question 9

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

Answer 9

optic nerve (optic neuritis)

Question 10

MS presenting with ataxia is caused by demyelination where?

Answer 10

cerebellum

Question 11

MS presenting with double vision is caused by demyelination where?

Answer 11

brainstem

Question 12

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

Answer 12

spinal cord

Question 13

what is acute Rx for MS?

Answer 13

3-5 day course of steroids

Question 14

over time what happens with MS? 3 things

Answer 14

gate difficulty
loss of bladder control
impaired cognitive function

Question 15

what is a cardinal pathologic finding with MS around veins?

Answer 15

perivascular inflammation

Question 16

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

Answer 16

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

Question 17

what is ADEM?

Answer 17

acute disseminated encephalomyelitis

Question 18

what causes acute disseminated encephalomyelitis?

Answer 18

post infection or vaccine

Question 19

what happens in acute disseminated encephalomyelitis?

Answer 19

immune mediated demyelination and polyfocal neuro deficits

Question 20

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

Answer 20

measles

varicella and rubella

Question 21

what are the two patho findings in acute disseminated encephalomyelitis?

Answer 21

demyelination and perivenous inflammatory infiltrates

Question 22

what is PML?

Answer 22

progressive multifocal leukoencephalopathy

Question 23

what causes progressive multifocal leukoencephalopathy?

Answer 23

JC virus

Question 24

who gets progressive multifocal leukoencephalopathy?

Answer 24

immunocompromised

Question 25

what happens in progressive multifocal leukoencephalopathy?

Answer 25

destruction of oligodendrocytes

Question 26

what lobes does progressive multifocal leukoencephalopathy prefer

Answer 26

usually temporal and occipital

Question 27

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

Answer 27

vision changes

Question 28

what white matter is destroyed in progressive multifocal leukoencephalopathy?

Answer 28

subcortical white matter

Question 29

what can you see in oligodendrocyte in progressive multifocal leukoencephalopathy?

Answer 29

intranuclear inclusions of the JC virus

Question 30

what is AIDS leukoencephalopathy?

Answer 30

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

Question 31

what is seen in histo stain for AIDS leukoencephalopathy?

Answer 31

multinucleated giant cells

Question 32

what do they think plays role in AIDS leukoencephalopathy development?

Answer 32

toxic cytokines from activated microglia

Question 33

what is another name for central pontine myelinolysis?

Answer 33

osmotic myelinolysis

Question 34

what happens in central pontine myelinolysis?

Answer 34

rapid axonal demyelination in pons

Question 35

what is cause of central pontine myelinolysis usually?

Answer 35

rapid osmotic changes

Question 36

what is iatrogenic cause of central pontine myelinolysis?

Answer 36

correction of hyponatremia

Question 37

what are symptoms of central pontine myelinolysis?

Answer 37

acute paralysis dysphagia diplopia loss of consciousness

Question 38

what is leukodystrophy?

Answer 38

inherited metabolic disease affecting white matter

Question 39

what genes cause leukodystrophy?

Answer 39

genes that are important for myelin production or myelin lipid metabolism

Question 40

where is demyelination in leukodystrophy?

Answer 40

CNS and PNS

Question 41

what are three leukodystrophys to know?

Answer 41

ALD MLD and GLD

Question 42

what do macros in ALD have in them?

Answer 42

long chain fatty acid

Question 43

what do macros in MLD have in them?

Answer 43

sulfatide...

Question 44

what do macros in GLD have in them?

Answer 44

galactocerebreside

Question 45

describe the progression of leukodystrophies

Answer 45

progressive and symmetric

Question 46

what is inheritance of ALD?

Answer 46

x linked recess

Question 47

where does demyelination primarily occur in ALD?

Answer 47

occipital white matter and brainstem/spinal cord

Question 48

what fibers are always preserved in leukodystrophy?

Answer 48

subcortical U fibers...just under the cortex of the brain

Question 49

what do macros look like in ALD?

Answer 49

stuffed with lipids

Question 50

what is inheritance of MLD?

Answer 50

AR

Question 51

what is MLD an issue with?

Answer 51

lysosomal storage disease

Question 52

what causes destruction of myelin in MLD?

Answer 52

cerebroside sulfate

Question 53

what are symptoms of MLD?

Answer 53

dementia and ataxia

Question 54

what is another name for GLD?

Answer 54

Krabbe disease

Question 55

what is inheritance of GLD?

Answer 55

AR

Question 56

what causes demyleination in GLD?

Answer 56

accumulation of galactocerebriside

Question 57

what are symptoms of GLD?

Answer 57

DD, peripheral neuropathy | optic atrophy

Question 58

what can happen post radiation for brain tumor?

Answer 58

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