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Clinical Neuroscience > Path- 7: Demyelinating Diseases > Flashcards

Path- 7: Demyelinating Diseases Flashcards

1
Q

What happens in leukodystrophies?

A

inherited disturbances in the formation and prresenvation of myelin

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2
Q

What is the most common leukodystrophy?

A

Metachromatic leukodystrophy

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3
Q

What is the inheritance of metachomatic leukodystrophy?

A

AR

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4
Q

What is the main problem in metachromatic leukodystrophy?

A

myelin metabolism

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5
Q

There is an increase in what substance in the white matter of the brain and PNS for metachomatic leukodystrophy?

A

Sulfatide (cerebroside sulfate)

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6
Q

What are the epidemiology of metachomatic leukodystrophy?

A

predominates in infancy –> letal within several years

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7
Q

What is the epidemiology of Krabbe disease?

A

early months of life –> death within 2 yrs

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8
Q

What is the deficiency in Krabbe?

A

galactocerbroside ß-galactosidase

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9
Q

There a subsequent increase in what cytotoxic sunstance in Krabbe?

A

galactosylsphingosine

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10
Q

What is the inheritance of Krabbe?

A

AR

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11
Q

What is the defect in metachomatic leukodystrophy?

A

arylsulfatase-A

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12
Q

What accumulates in metachomatic leukodystrophy?

A

Sulfatides –> myelin breakdown

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13
Q

What is the inheritance of metachomatic leukodystrophy?

A

AR

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14
Q

What are the clinical findings of Krabbe?

A

mose myelin and oligodendrocytes, reactive asterogliosis, multineucleared macrophages called GLOBOID cells that accumulate around blood vessels.

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15
Q

What is adrenoleukodystrophy (ALD)?

A

XR disorder of the adrenal Cx and demyelination of the nervous system

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16
Q

Who gets ALD?

A

kids ages 4-8

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17
Q

What increases in tissues and body fluids in ALD?

A

very long chain FA’s because u cant degrade them in peroxisomes

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18
Q

What happens in the cortical white matter in ALD pt’s?

A

severe demyelination, esp in parieto-occipital regions

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19
Q

What happens in Alexander disease?

A

in infants/kids, lose myelin in brain, accumulation of irregular extracellular fibers (rosenthal fibers)

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20
Q

What causes Alexander disease?

A

a mutation in GFAP

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21
Q

What are the clinical features of Alexander disease?

A

psychomotor retardation, dementia, paralysis –> death

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22
Q

What is the most common chronic CNS disease of young adults in the US?

A

MS

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23
Q

What is the epidemiology of MS?

A

women 2:1, genetics, colder climates increase risk

24
Q

What do Th1 cells secrete in chronic MS?

A

IFNg

25
Q

What does INFg activate in MS?

A

Macrophages

26
Q

What do the activated macrophages do in MS?

A

Demyelination

27
Q

What are the hallamrks of MS on histological exams?

A

Demyelinated plaques in white matter (esp in optic nerves)

28
Q

Once the plaque demyelinates the neuron, what does it leave behind?

A

gliosis (scar), which impairs structural integrity of axons

that bitch.

29
Q

What are the clinical findings to MS?

A

onset 30-40 yrs, PNS is usually spared, begin with eye problems, lesions in spinal cord cause leg weakness, ataxia or numbeness

30
Q

What is seen on lumbar puncture for MS?

A

mild increase in CSF protein, increase in CSF IgG with oligoclonal bands

31
Q

What is the characteristic pathogenesis of MS?

A

chronic relapsing and remitting course

32
Q

What causes death in MS?

A

respiratory paralysis or UTI

33
Q

What is the survival rate for MS?

A

20-30 years following initial Sx

34
Q

True or False: there is a presence of a rimmed-pattern lesions in MS

A

True

35
Q

True or False: the rimmed-pattern lesions in MS have surrounding vasogenic edema

A

False.

This is how u can distinguish MS from abscesses and neoplasms!

36
Q

Who has neuromyelitis optica (devic disease)?

A

Asians (lel)

37
Q

What is the clinical presentation of devic disease?

A

bilateral optic neuritis and spinal cord involvement, lesions are similiar to MS but more destructive.

38
Q

What causes acute disseminated encephalomyelitis?

A

follows either viral infeciton or immunization

39
Q

What are the Sx of acute disseminated encephalomyelitis?

A

headache, lethargy or coma. typically 1-2 weeks after causitive agents.rapidly progressing

40
Q

What causes acute necrotizing hemorrhagic encephalomyelitis?

A

usually preceded by upper resp infection by mycoplasma pneumoniae

41
Q

What is the prognosis of acute necrotizing hemorrhagic encephalomyelitis?

A

NOT GOOD

42
Q

What happens in central pontine myelinolysis?

A

lose myelin, preserve neurons and axons, caused from rapid correction of hyponatremia

43
Q

What are the Sx in central pontine myelinolysis?

A

rapidly developing quadriplegia from lesion in basis pontis. non-inflammatory. no Tx.

44
Q

Where is the dysfxn in Marchiafava-Bignami disease?

A

disorder of the myelin of the corpus callosum and anterior commisure.

45
Q

Who gets Marchiafava-Bignami disease?

A

Alcoholics

46
Q

What is the cardiac problem in B1 deficiency?

A

Berberi

47
Q

What is Wernicke encephalopathy from b1 deficiency?

A

u get psychoses and opthaloplegia

48
Q

What happens if Wenickie encephalopathy isnt treated?

A

u get korsakoff syndrome (memory disturbances) too

49
Q

What are the Sx to Wenicke-Korsakoff syndrome?

A 
Inability to form new memories 
  Loss of memory, can be severe 
  Loss of muscle coordination (ataxia) 
  Unsteady, uncoordinated walking 
  Making up stories (confabulation) 
  Seeing or hearing things that aren't
   really there (hallucinations) 
  Vision changes 
  Abnormal eye movements 
  Double vision 
  Eyelid drooping
50
Q

What are the 3 big Sx to delirium tremens?

A

Agitation
Fever
Convulsions

51
Q

What are the CNS problems with alcohol withdrawl?

A

focal hemorrhages/necrosis in mamillary bodies from macrophages containing hemosiderin

52
Q

Why does B12 deficiency cause numbness, tingling and slight ataxia in the lower extremities?

A

cuz it affects lateral and posterior columns, may progress to spastic weakness and complete paraplegia

53
Q

What is the SCDSC of B12 deficiency?

A

Subacute Combined Degeneration of the Spinal Cord, it’s the swelling of the myelin producing vacuoles –> paraparesis and marked paresthesias

54
Q

Which cells are targeted selectively by mercury compounds in vivo?

A

Cerebellar granule cells

55
Q

What is the main compound in mercury poisonings, whcih isnt cleared by the kidneys?

A

methylmercury

56
Q

What are the Sx of industrial Hg poisoning?

A

renal failure, tremors

Clinical Neuroscience (12 decks)

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