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Res Quizzes > Demyelinating Diseases > Flashcards

Demyelinating Diseases Flashcards

1
Q

Generally accepted pathologic criteria for multiple sclerosis (3)

A

1 myelin sheath destruction with relative sparing of other elements
2 predominantly perivenous infiltration of inflammatory cells
3 primarily white matter lesions

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2
Q

Define MULTIPLE SCLEROSIS

A

Progessive chronic condition characterized clinically by episodes of focal disorders of the optic nerves, spinal cord, & brain, which remit & recur over many years

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3
Q

Pathologic findings in Multiple Sclerosis

A

sharply delineated periventricular white matter lesions in the brain & spinal cord that do not extend beyond the entry zones of cranial or spinal nerves

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4
Q

Histologic appearance of ACUTE lesions in MS (4)

A
  • loss of myelin in the perivenous distribution
  • slight oligodendroglial degeneration
  • variable astrocytic reaction
  • perivascular & para-adventitial mononuclear & lymphocytic infiltration
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5
Q

Histologic appearance of CHRONIC lesions in MS (4)

A
  • thickly matted, acellular glial tissue
  • occasional perivascular lympthocytes & macrophages
  • walleraian degeneration of long fiber tracts
  • shadow patches : partial remyelination of undamaged fibers
  • cavitation : destruction of supporting tissues & vessels
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6
Q

4 Histologic subgroups or patterns of MS

A

1 inflammatory lesions made up of T cells & macrophages alone
2 an autoaAb lesion mediated by Ig & complement
3 characterized by apoptosis of oligodendrocytes
& no Ig, complement, & + partial remyelination
4 showing only oligodendrocyte dystrophy & no remyelination
*3-4 show a primary oligodendroglial degeneration

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7
Q

True or False.

MS has a bimodal age-specific onset curve

A

False

unimodal

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8
Q

Peak age of developing symptoms of MS

A

30-40 years of age

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9
Q

The strongest genetic factor thought to be associated to developing MS

A

DR locus on chromosome 6

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10
Q

Thought to be markers of MS susceptibility gene, and the presence of any of these increases the risk of developing MS by how much?

A 
HLA-DR2
HLA-DR3
HLA-B7
HLA-A3
3-5x increased risk
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11
Q

Other than the HLA loci, thse too were said to increase the susceptibility to develop MS

A

IL2Ralpha

IL7Ralpha

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12
Q

Most immunologists believe that MS is medidated by

A

T-cell sensitization to some component of myelin

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13
Q

What is the Uhthoff phenomenon & what is its basis?

A

worsening of neurologic symptoms of MS patients upon exposure to heat.

Nerve conduction in demyelinated & remyelinated fibers are sensitive to subtle changes in metabolic & environmental changes

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14
Q

4 MS Syndromes

A

1 optic neuritis
2 transverse myelitis
3 cerebellar ataxia
4 brainstem syndromes

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15
Q

The pain in Lhermitte sign is most probably due to

A

increased sensitivity of demyelinated axons to the stretch or pressure on the spinal cord induced by neck flexion

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16
Q

Prognosis of optic neuritis

A

Good. half recover completely

treatment did not influence outcome

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17
Q

What is Pulfrich effect?

A

psychophysical percept wherein lateral motion of an object in the field of view is interpreted by the visual cortex as having a depth component, due to a relative difference in signal timings between the two eyes

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18
Q

What is Charcot triad?

A

1 scanning speech
2 nystagmus
3 intention tremor

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19
Q

True or False. the presence of bilateral internuclear ophthalmoplegia in a young adult is virtually diagnostic of MS.

A

True

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20
Q

True or False. the occurrence of transient facial hypesthesia or anesthesia or of trigeminal neuralgia in a young adult should always suggest the diagnosis of MS implicating the intramedullary fibers of the fifth cranial nerve

A

True

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21
Q

useful in controlling spontaneous attacks in MS

A

carbamazepine

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22
Q

blocks painful tonic spasms that are elicited by hyperventilation in MS

A

acetazolamide

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23
Q

True or False. Facial palsy along the lines of Bell’s palsy can be a sign of MS

A

false

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24
Q

Most common precipitating factors of MS

A

infection
trauma
pregnancy

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25
Q

Most common precipitating factors of MS (though not firmly established)

A

infection
trauma
pregnancy

26
Q

Variant of MS that is rapidly progressive and highly malignant. A combination of cerebral, brainstem, and spinal manifestations evolves over a few weeks, rendering the patient stuporous, comatose, or decerebrate with prominent cranial nerve and corticospinal abnormalities

A

Marburg type or Tumefactive type

27
Q

Variants of MS consisting of diffuse sclerosis

A

Balo & Schilder Disease

28
Q

CSF oligoclonal bands is positive in what proportion of MS patients?

A

90%

29
Q

Positive test of CSF IgG index in MS is?

A

> 12%

30
Q

True or False. The finding of oligoclonal bands in a suspected MS patients connotes that the disease is already chronic

A

True

31
Q

True or False. Oligoclonal bands in blood of MS patients is also diagnostic along with the clinical features consistent with MS

A

False

32
Q

Most commonly used diagnostic test in MS

A

CSF oligoclonal bands

33
Q

Most helpful diagnostic test in MS

A

MRI

34
Q

In MS. T1 hypointensity is ___________ proportional to the degree of remyelination

A

inversely

35
Q

filiform pattern in the corpus callosum in the MRI of MSI patients

A

Dawson’s fingers

36
Q

Cranial MRI findings in MS

A
  • T2 hyperintense, T1 hypointense
  • multifocal, well-demarcated, oval or linear, radially oriented lesionsadjacent to the ventricular surface
  • C-shaped partial or medially open ring of abnormal enhancement
37
Q

___% of definite MS & ___% of probable or possible MS have abnormal VER.

A

70

60

38
Q

___% of definite MS & ___% of probable or possible MS have abnormal SSEP

A

60

40

39
Q

___% of definite MS & ___% of probable or possible MS have abnormal BAER

A

40

20

40
Q

diagnostic study to dedmonstrate axonal loss or thinning of the retina

A

optical coherence tomography

41
Q

4 typical locations of T2 hyperintesities in MS

A

1 periventricular
2 juxtacortical
3 infratentoria
4 spinal cord

42
Q

The most predictive of long term disability in MS

A

degree of disability at 5 years from the onset of the first symptoms

43
Q

True or False. IV methylprednisolone administered at 1 g/d for 5 days per month over 5 years, was shown to reduce disability and brain atrophy and total volume of hypodense lesions on Tl-weighted MRI

A

True

study by Zivadinov

44
Q

MPPT dose for MS

A

1) 0.5-1g/day x 3-5days then oral prednisone 60-80mg/day x taper 12-20 days

2) oral methylprednisolone 48mg/day x 1 week
24mg/day x 1 week
12mg/day x 1 week

45
Q

IV Ig dose for MS

A

0.2g/kg monthly x 2 years

46
Q

The main problem in MS patients treated with interferon

A

development of antibodies against it

47
Q

Monoclonal antibodies used in MS and their targets (4)

A

Natalizumab - anti alpha integrins to block lymphocyte, monocyte adhesion to endolium, migration
Alemtuzumab - anti CD52 antigen to reduce B, T cells
Rituximab - anti CD20 lymphocytes, depleting Bcell monoclonal Ab
Ocrelizumab - anti CD20

48
Q

Monoclonal antibodies used in MS and their side effects (4)

A

Natalizumab - PML
Alemtuzumab -ITP, thyroiditis
Rituximab - infection, lymohoma
Ocrelizumab - infection, lymohoma

49
Q

Mode of action of glatiramer acetate

A

mimics myelin basic protein

50
Q

Side effects of fingolimod

A

lymphopenia, adenopathy, bradycardiam AV block, macular edema, herpes infection, increased LFTs

51
Q

Medications that may be used to address fatigue in MS

A 
amantadine
modafinil
permoline
methylphenidate
dextroamphetamine
52
Q

Isoniazid can be used in MS to address what?

A

severe postural tremor

53
Q

Distinctive characteristics of Devic’s Disease from MS (6)

A

1 failure to develop cerebral demyelinating lesions even after years of illness
2 absence of CSF oligoclonal bands
3 tendency to CSF pleocytosis more so than in MS
4 necrotizing cavitary nature of the spinal cord lesion, affecting white and gray matter alike with prominent thickening of vessels
5 but with minimal inflammatory infiltrates
6 involve several contiguous longitudinal segments of the spinal cord

54
Q

Antibody specific to Devic’s Disease and its sensitivity and specificity?

A

NMO antibody
anti aquaporin 4

76% sensitive
94% specific

55
Q

Criteria for the diagnosis of NMO which is 99% senstivie and 90% specific

A

2 of the following:
1 longitudinally extensive myelopathy
2 positive antibodies
3 nitial MRI that is not characteristic for MS

56
Q

The only histopathologic characteristic of ADEM that distinguishes it from MS

A

multifocal meningeal infiltration

57
Q

Most fulminant form of demyelinating disease

A

Weston Hurst Disease

acute hemorrhagic leukoencephalitis

58
Q

Microorganism associated with Weston Hurst Disease

A

none. most cases has indeterminate cause.

Mycoplasma pneumoniae

59
Q

Neuroimaging findings that distinguish Weston Hurst Disease from ADEM

A

1 size of the lesions
2 hemorrhagic character
3 extent of the surrounding edema

60
Q

Neuroimaging findings in Weston Hurst Disease

A

bilateral asymmetrical large, confluent, edematous lesions white matter with a myriad of punctate
hemorrhages in gray and white matter

61
Q

Distinctive pathologic characteristic of Weston Hurst Disease

A

destruction of white matter to the point of liquefaction

62
Q

Mcdonald Criteria for MS

A 
≥2 attack, ≥ 2 lesion
≥2 attack, 1 lesion = DIS
1 attack, ≥2 lesion = DIT
1 attack, 1 lesion = DITS
0 attack = 1 year of disease progression and 2 out of 3 criteria: DIS brain, DIS cord, +CSF

Res Quizzes (18 decks)

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