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Adams 10th Ed > Chap 36: Demyelinating Disease > Flashcards

Chap 36: Demyelinating Disease Flashcards

1
Q

Generally accepted pathologic criteria for demyelinating dz

A

1) destruction of myelin sheaths w/ relative sparing of other elements 2) perivenous infiltration of inflammatory cells 3) primarily white matter lesions

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2
Q

Pattern of MS, where symptoms improve partially or completely then after a variable interval recurrence of same abn or new lesion

A

Relapsing-remitting pattern

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3
Q

Pattern of MS

A

Relapsing-remitting Primary progressive Secondary progressive (initally relpasing then steadily progressive.

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4
Q

Lesion of MS is noteworthy since it is localized

A

perventricular where subependymal veins line

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5
Q

Four histologic subgroups in MS: Pattern I Pattern II Pattern III Pattern IV

A

Pattern I: T-cells and macrophages Pattern II: immunoglobulin and complement Pattern III: apoptosis of oligodendrocytes and absence of Ig, complement w/ partial remyelination Pattern IV: oligodendrocyte dystrophy and no remyelination

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6
Q

Dominant mechanism in demyelination in MS

A

Pattern II: antibody and complement mediated phagocytosis

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7
Q

Age of onset of MS

A

Peak at 3rd-4th decade then low at 6th decade

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8
Q

T/F. Increasing risk of developing MS w/ higher and lower latitude

A

TRUE

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9
Q

Other risk factors for MS aside from latitude

A

Vit D and sun exposure

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10
Q

Number of years needed to become apparent to carry the risk from high risk to low risk zones after migration in MS

A

20 years

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11
Q

Percentage risk of MS px to have relatives

A

15%

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12
Q

Strongest genetic association w/ MS

A

DR locus of Chromosome 6

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13
Q

Approximate animal model of MS, that suggest that MS is mediated by T-cell sensitization

A

experimental allergic encephalomyelitis

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14
Q

Autoantibodies inconsistently found against

A

MOG MBP

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15
Q

Main physiologic effect of demyelination

A

Impede saltatory conduction

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16
Q

Typical symptom of optic neuritis

A

reduction in intensity of color red (desaturation)

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17
Q

Typical features of MS where temporary induction by heat or excercise causes unilateral visual blurring

A

Uhthoff phenomenon

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18
Q

A rise of only ____‘C can block transmission in thinly myelinated or demyelinated fibers;

A

0.5’C

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19
Q

Things that may worsen that may briefly worsen neurologic fxn that may confused w/ relapse

A

Smoking Fatigue Hyperventilation Rise envt temp

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20
Q

A useful finding that a patient w/ MS may present

A

Symptom of one leg but w/ signs in both

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21
Q

Common modes of onset of MS

A

1) Optic neuritis 2) transverse myelitis 3) cerebellar ataxia 4) Brainstem syndrome

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22
Q

If only w/ one feature of MS they are termed as

A

Clinically isolated syndrome

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23
Q

PE where flexion of neck may induce tingling or electric like feeling down shoulder and back, attributed to sensitivity of demyelinated axons to stretch or pressure

A

Lhermitte sign

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24
Q

Typical age of onset for relapsing-remitting pattern

A

<40 y/o

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25
Q

T/F. Optic neuritis, they present as persistent pain in orbit before visual loss

A

FALSE. SHORT LIVED PAIN

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26
Q

Diffrentiate papilledema from papillitis

A

Papillitis presents w/ severe and acute visual loss

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27
Q

Chance of complete recovery from optic neuritis

A

50%

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28
Q

Visual problem that may persist after optic neuritis

A

Dyschromatopsia Pulfrich effect (Pendulum swinging along line of sight appears to move in circular motion

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29
Q

Improvement of optic neuritis begins around

A

2 weeks from onset

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30
Q

T/F. Less than half of adult w/ optic neuritis will eventually develop other signs of MS

A

FALSE, MORE than HALF

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31
Q

T/F. Risk of MS in initial attack of optic neuritis in children is higher than adult

A

FALSE. it is LOWER

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32
Q

T/F. Recurrence of optic neuritis inc risk for MS

A

TRUE

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33
Q

T/F. If cranial MRI is normal, chances of MS relapsing-remitting type is less

A

TRUE

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34
Q

T/F. Spinal cord in MS are symmertical and complete.

A

FALSE. Asymmetrical and incomplete w/ long tract signs

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35
Q

Consideration if recurrent myelitis w/ absent cerebral dissemination

A

SLE Sarcoidosis Sjorgen MCTD Dural fistulas / AVM

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36
Q

Triad of nystagmus, scanning speech, intention tremor

A

Charcot triad

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37
Q

The presence of ____ in a young adult is virtually a diagnostic of MS AND occurence of this suggests MS

A

Bilateral INO Transient facial hypesthesia, anesthesia

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38
Q

Most common manifestation of progressive MS

A

Asymmetrical spastic paraparesis w/ degree of impaired posterior column in legs

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39
Q

T/F. Cognitive impairment may occur in long-standing MS

A

TRUE

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40
Q

MS may have euphoria, pathologic cheerfulness seem inappropriate in face of obvious neuro deficit

A

Morbid optimism

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41
Q

Drugs used to alleviate fatigue in MS

A

Modafinil, Amantadine

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42
Q

Most common precipitating factors for acute MS attacks

A

Infection Trauma Pregnancy

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43
Q

T/F. The coincidence of trauma and new or excacerbated MS is incidental

A

TRUE

44
Q

Rapid and highly malignant form of MS w/ acute large plaques and mass effect w/ enhancement that simulate a tumor

A

Tumefactive MS (Marburg variant)

45
Q

Common imaging feature of acute demyelinating plaques that is less typical of tumors or abscess

A

“Open ring” enhancement in T1 MRI

46
Q

MS variant w/ occurrence of alternating bands of destruction and preservation of myelin in a series of concentric rings that represent alternating areas of myelin loss and preservation

A

Concentric sclerosis of Balo

47
Q

MS variant presenting as diffuse sclerosis of usually seen among children and young adults improtant ddx

A

Schilder disease Ddx: Diffuse cerebral neoplasm, Adrenoleukodystrophy, PML

48
Q

1/3 of MS w/ acute onset or exacerbation, may have in CSF and can be the only measure of activity

A

Moderate mononuclear pleocytosis

49
Q

Most widely used CSF test for confirmation of diagnosis of MS, shown in 90% of MS cases

A

Oligoclonal bands

50
Q

Disease entities that may have oligoclonal band

A

Syphillis Lyme SSPE

51
Q

T/F. Positive oligoclonal band is always positive in first attacks or late stages of MS

A

FALSE, not always

52
Q

The presence of oligoclonal bands in first attack of MS is predictive of

A

Chronic relapsing course

53
Q

IgG index refers to: AND a positive test is considered if

A

Proportion of IgG to TP of CSF Positive if >12%

54
Q

MRI sequence particularly sensitive to detecting plaque lesions

A

T2 sequence

55
Q

MRI appearance of MS Acute lesions Chronic lesions

A

Acute: T1 hypo, T2 hyper (tissue expansion due to edema)

Chronic: contracted and T2 hyper

56
Q

T/F. T1 hypointensity is directly proportional to the degree of remyelination

A

FALSE. inversely proportional

57
Q

Radial orientation of lesion in MRI corresponding to course of venules w/in cerebral white matter denoting relapsing-remitting MS

A

Dawson fingers

58
Q

Disruption of BBB due to inflammation is shown in MRI as

A

abn T1 hyper in enhancement

59
Q

MS spinal lesions typically do not extend beyond three vertebral segments, if so it may be

A

NMO

60
Q

Test that are sensitive to detect existence of additional asymptomatic lesion

A

VER BAER Somatosensory evoked response

61
Q

Four typical locations in MS

A

Periventricular Juxtacortical Infratentorial Spinal cord

62
Q

Two features to consider in MS

A

Clinical features (Optic neuritis, transverse myelitis, cerebellar ataxia, Brainstem syndrome) Lab features (MRI, IgG, oligoclonal bands)

63
Q

One clinical feature is diagnostic for MS if

A

2 or more typical attacks of CNS demyelination w/ objective evidence

64
Q

If 2 clinical attacks w/ objective evidence for one lesion

A

MRI lesions in at least two typical locations

65
Q

If 1 clinical attack w/ 2 or more objective evidence of lesion

A

Dissemination over time: simultaneous enhancing and nonenhancing lesions or interval devt of new lesions in MRI

66
Q

If 1 clinical attack w/ objective evidence of one lesion (CIS)

A

Dissemination in space: at least 2 lesions of four typical locations, and dissemination in time

67
Q

If Progressive MS, nonrelapsing, deficits suggestive of MS

A

1 yr dz progression AND dissemination in space by MRI, AND IgG index or oligoclonal bands in CSF

68
Q

The most predictive feature for long term disability is

A

degree of disability at 5 yrs from first symptoms

69
Q

T/F. Pregnancy is associated w/ clinical stability or improvement

A

TRUE

70
Q

Increase risk of excacerbation among women to 2-fold are seen in

A

Few months postpartum

71
Q

Average duration of illness is

A

30 years

72
Q

Recurrent iridocylcitis and meningitis, membrane ulcers, articular symptoms, multifocal cerebral, lung, renal disease among middle eastern origin

A

Bechet disease

73
Q

T/F. Diagnosis of MS should cautious if symptoms and signs can be explained by one single lesion in one region of neuroaxis

A

TRUE

74
Q

T/F. Relapsing remiiting is less responsive to immunomodulatort therapy compared to chronic progressive type

A

FALSE, other way around

75
Q

T/F. in MS, steroids have a significant effect of ultimate course of dz and prevent recurrence

A

FALSE. NO EVIDENCE

76
Q

The use of this drug alone for optic neuritis was cautioned against since inc risk of new episodes

A

Oral Prednisone

77
Q

Drug tx for MS that may alter natural hx of relapsing-remitting type

A

Interferon beta & glatiramer

78
Q

Treatment of relapsing-remitting MS equally effecitve to alter natural hx but given in once a week regimen

A

IFN-beta-1a

79
Q

One issue with long term administration of interferon

A

Antibody devt to drug

80
Q

Side effects seen in interferon

A

flu-like symptoms sweating malaise exacerbation of headaches systemic capillary leak syndrome (RARE)

81
Q

Drug tx of MS that mimic actions of MBP, given daily in SC dosing

A

Glatiramer

82
Q

Advantage of glatiramer over interferon

A

Antibodies do not develop

83
Q

T/F. No advantage w/ Azathioprine in MS

A

TRUE

84
Q

Drugs that can be used for chronic progressive MS

A

Prednisolone plus cyclophosphamide Low dose oral MTX Mitoxantrone

85
Q

Monoclonal antibody directed against alpha-integrin (blocks adhesion and migration to endothelial wall) resulting to reduction of relapse and slows accumulation of lesions in MRI

A

Natalizumab

86
Q

Notorius side effect of Natalizumab in relation to use for MS

A

PML

87
Q

Monoclonal antibodies used for MS

A

Natalizumab (block alpha-integrin) Alemtuzumab (targets CD-52) Rituximab (targets CD 20)

88
Q

Oral immunosuppressive drug that interferes w/ egress of lymphocytes from lymph node, lessen MRI lesions and ralapse rate and comparable or superior to injectables

A

Fingolimod

89
Q

Drugs used for Fatigue in MS

A

Modafinil Amantadine Methlyphenidate

90
Q

Drug used for urinary retention in MS

A

bethanecol

91
Q

Drug used for spastic bladder, used intermittently

A

Propantheline Oxybuntynin

92
Q

Drugs used to reduce paroxysmal symptoms of MS

A

CBZ or gabapentin

93
Q

Simulataenous or successive and severe involvement of optic nerves and spinal cord

A

NMO

94
Q

Antibody seen in NMO

A

Aquaporin-4 antibody

95
Q

Spinal cord lesions in NMO are typically

A

necrotizing

96
Q

The antibodies of NMO are mostly seen in

A

astrocytic end feet adjacent to capillaries, and Virchow0-robin spaces all in periventricular region at central canal

97
Q

Additional criteria on top of NMO antibody that will increase sensi and speci to (99, 90%)

A

Longitudinally extensive myelopathy MRI not characteristic of MS

98
Q

T/F. Tx of NMO is unsucessful despite aggressive tx

A

TRUE

99
Q

Drug that suggest clinical improvement in NMO

A

Methylprednisolone plus Azathioprine

100
Q

T/F. ADEM is indistinguishable on histopathologic grounds from acute MS

A

TRUE

101
Q

T/F. Adult make good recoveries compared to children in ADEM

A

TRUE

102
Q

By what immune mechanism does NMO work

A

humoral response, compared to MS cellular response

103
Q

Vaccine assoc w/ postvaccinal ADEM

A

Rabies vaccine (DEV type)

104
Q

Most fulminant form of demyelinating disease, almost severe end spectrum of ADEM in young adult and children usually preceded by respiratory infection

A

Acute Necrotizing Hemorrhagic Encephalomyeltis (of Weston Hurst)

105
Q

Distinctive pathologic finding of Acute Necrotizing Hemorrhagic Encephalomyeltis

A

Pink or yellow gray tissue and flecked w/ multiple petechial hemorrahages Histopath: perivascular inflammation w/ widespread necrosis and exudation of fibrin

106
Q

White matter cerebral disease associated after transplantation and attributed to PML

A

Graft-vs-Host Disease

Adams 10th Ed (8 decks)

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