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Neurology - Physiology > DEMYELINATING DISEASE > Flashcards

DEMYELINATING DISEASE Flashcards

1
Q

Structures affected by MS

A

where pial veins lie next to or within the white matter

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2
Q

In old lesions with interruption of axons, there

may be descending and ascending __________of long fiber tracts in the spinal cord

A

wallerian degeneration

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3
Q

four histologic subgroups of MS

Type 1-4

A
  1. T cells and macrophages
    alone (pattern I);
  2. an autoantibody lesion mediated
    by immunoglobulin and complement (pattern II);
  3. apoptosis of oligodendrocytes
    and absence of immunoglobulin, complement, and with
    partial remyelination (pattern III);
  4. oligodendrocyte dystrophy and no remyelination
    (pattern IV)
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4
Q

Most data suggest that antibody and_________ are the dominant mechanism
of demyelination in MS

A

complement mediated

myelin phagocytosis

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5
Q

Approximately _______percent of MS patients have an affected
relative, with the highest risk of concurrence being
observed in the patient’s siblings

A

15

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6
Q

The strongest association in MS is with

the DR locus on______

A

chromosome 6

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7
Q

infectious agents associated with MS

A

The bacterial agents Chlamydia pneumoniae
and Borrelia burgdorferi (the agent of Lyme disease) and
herpesvirus type 6

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8
Q

Autoantibodies have been found inconsistently that

are directed against _____ and ___

A

myelin oligodendrocyte glycoprotein

(MOG) and MBP

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9
Q

Another typical feature of MS is the temporary ind uction,
by heat or exercise, of symptoms such as unilateral
visual blurring ______

A

(the Uhthoff phenomenon)

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10
Q

A
rise of only ________) can block electrical transmission
in thinly myelinated or demyelinated fibers

A

0.5 C (0.9°F

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11
Q

__________ are all
capable of briefly worsening neurologic functioning and
are easily confused with relapses of disease.

A

Smoking, fatigue, hyperventilation,

and a rise in environmental temperature

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12
Q

These common modes of onset are

A

are: (1) optic
neuritis, (2) transverse myelitis, (3) cerebellar ataxia, and
(4) brainstem syndromes

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13
Q

When these are unaccompanied

by other features of MS, they are termed_______

A

“clinically

isolated syndrome” (CIS)

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14
Q

It is probably attributable to an increased sensitivity
of demyelinated axons to the stretch or pressure
on the spinal cord induced by neck flexion, but it occurs
in other conditions such as cervical spondylosis

A

Lhermitte sign

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15
Q

The typical relapsing-
remitting pattern of disease is more likely to appear
in patients who are younger than ______

A

40 years of age.

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16
Q

In approximately _______ of all MS patients (and
possibly in a larger proportion of children), the initial
manifestation is an episode of optic neuritis .

A

25 percent

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17
Q

ONTT findings

_______ generally taking
the form of a perceived desaturation of colors, frequently
persists as does the

______ wherein an object
such as a pendulum that is swinging perpendicular to
the patient’s line of sight, appears to moving in a threedimensional,
circular motion.

A

Dyschromatopsia,

Pulfrich effect,

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18
Q

More than ______of adult patients who present
with optic neuritis will eventually develop other signs of
MS

A

one-half

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19
Q

Clinically, the illness is characterized by a rapidly
evolving (several hours or days) symmetrical or asymmetrical
paraparesis or paraplegia, ascending paresthesia,
loss of deep sensibility in the feet, a sensory level on
the trunk, sphincteric dysfunction, and bilateral Babinski
signs.

A

TM

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20
Q

Another relatively isolated syndrome,
occurring mainly in _________ is a slowly progressive
cervical myelopathy with weakness and ataxia

A

older women,

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21
Q

The combination of nystagmus, scanning speech, and

intention tremor is known as the______

A

Charcot triad

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22
Q

A predominantly cerebellar or brainstemcerebellar

form occurs in approximately _____ percent of cases

A

5

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23
Q

It has become evident that some degree of cognitive
impairment, and probably a progressive decline,
is present in perhaps _____ of patients with longstanding
MS

A

one-half

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24
Q

Dementia in MS

A

As mentioned above,
the cognitive impairment is in keeping with what has
been ascribed to “subcortical dementia

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25
Q

MC Precipitants of MS

A

The most common are infection,

trauma, and pregnancy.

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26
Q

many plaques are of the same age and the confluence of

A

Acute a n d Tu m o r- L i ke (Tu m efactive) M u l t i p l e

S c l e ro s i s ( M a r b u rg Va r i a nt)

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27
Q

Usually the CSF shows a cellular response but no

oligoclonal bands.

A

True

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28
Q

_____ has as its distinguishing
feature the 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.

Some
studies have found a high incidence in the Philippines.

A

The concentric sclerosis of Balo

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29
Q

large, well demarcated
areas of demyelination in the white matter of
both cerebral hemispheres, as well as a number of smaller
demyelinating foci, resembling the common lesions of
MS.

A

diffuse sclerosis

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30
Q

CSF test_____ may in fact be the only measure

of activity of the disease.

A

pleocytosis

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31
Q

The presence
of _____in a first attack of MS is predictive of a chronic
relapsing course,

A

bands

32
Q

IgG index considered positive

A

a
positive test is considered to be greater than 12 percent of
the total protein.

33
Q

I t i s now widely appreciated that ____ is the most helpful
ancillary examination in the diagnosis of MS, by virtue
of its ability to reveal symptomatic and asymptomatic
plaques in the cerebrum, brainstem, optic nerves, and
spinal cord

A

MRI

34
Q

The ______sequence is particularly sensitive in detecting

lesions in the brainstem, cerebellum, and spinal cord.

A

T2

35
Q

The presence of T1 hypointensity depends on the extent

of _____ of the lesion

A

remyelination

36
Q

T1 hypointensity

was inversely proportional to the degree of ______

A

remyelination

37
Q

MRI pattern that differentiates MS from abscess

A

C-shaped partial or open ring of abnormal

enhancement;

38
Q

This change probably reflects both the loss of glial
cells and, importantly, wallerian degeneration and loss
of axons triggered acutely by inflammation and more
chronically by other neurodegenerative stimuli

A

cortical atrophy

39
Q

Abnormalities of visual
evoked responses have been found in approximately
____ percent of patients with the clinical features of
definite MS

A

70

40
Q

____ is a technique for
creating two- and three-dimensional images of the optic
nerve and retina.

A

Optical coherence tomography (OCT)

41
Q

Measures degree of disability inMS

A

Kurtzke Disability Status Scale

42
Q

in approximately _______ percent of cases,
the clinical course lacks periodic relapses and is almost
evenly progressive from the beginning (primary progressive
MS;

A

10

43
Q

Highest rate of relapse in pregnancy

A

first trim, 1.2 attacks

44
Q

effect of methylprednisolone administered at 1 g/ d

for 5 days per month over 5 years

A

there was a reduction
in disability as well as in the

degree of brain atrophy and

total volume of hypodense lesions on Tl-weighted MRI

45
Q

T or F

A randomized
trial comparing oral and intravenous methylprednisolone
in acute relapses of MS demonstrated no clear
advantage of the intravenous regimen

A

t

46
Q

Oral methylpredinsolone loading

A

48 mg in a single daily
dose for 1 week, followed by 24 mg daily for 1 week, and
finally 12 mg daily for 1 week)

47
Q

methylprednisolone 500 mg orally for

5 days had a beneficial effect on visual function at ________ However, at 8 weeks, no effect could be shown

A

1 and

3 weeks.

48
Q

True or False

In
this study, it was found that the use of intravenous methylprednisolone
followed by oral prednisone did, indeed,
speed the recovery from visual loss,

although at 6 months
there was little difference between patients treated in this
way and those treated with placebo

A

T

49
Q

subcutaneous injection
of this agent every second day for up to 5 years decreases
the frequency and severity of relapses by almost one-third
and also the number of new or enlarging lesions (“lesion
burden”) in serial MRis.

A

IFNB

50
Q

Dose of IFN B

A

The dose

currently used is 30 meg, or 6.6 million units

51
Q

longer term administration of

interferon is the development of _____ to the drug.

A

antibodies

52
Q

After a period of years,
_____ of patients demonstrate antibodies with daily
administration,

A

30 percent

53
Q

Overall, the side effects of these interferon agents are
modest, consisting mainly of _____ sweating,
and malaise beginning several hours after the injection
and persisting for up to 14 h;

A

flu-like symptoms,

54
Q

Complication in patients with a monoclonal gammopathy who receive
interferon

A

“systemic capillary leak syndrome

55
Q

in patients with a first episode of optic neuritis
and at least two lesions on MRI that were compatible with MS.

Over 3 years, there was a modest reduction in clinical
progression or relapse from 37 percent to 28 percen

A

Controlled
High Risk Subjects Avonex Multiple Sclerosis Prevention
Study (CHAMPS

56
Q

synthesized to mimic the actions of myelin basic
protein, a putative autoantigen in MS, is given daily in
subcutaneous doses of 20 mg

A

Copolymer I (glatiramer acetate),

57
Q

_______
a drug with broad immunosuppressant and cytotoxic
activity, has attracted interest because one study has
shown a slight beneficial effect on the progressive form
of the disease

A

mitoxantrone,

58
Q

_____is directed against alpha-integrin in order to
block lymphocyte and monocyte adhesion to endothelial
cells and their migration through the vessel

were able to demonstrate a reduction in
the number of relapses and a slowing of the accumulation of MRI lesions

A

Natalizumab

59
Q

Nataluzimab study

showed a 68 percent reduction
in relapses, an 80 percent reduction in new or enlarging
T2 cerebral lesions and a 96 percent reduction in
gadolinium-enhancing lesions on MRI after a year

A

AFFIRM

60
Q

Anaphylaxis in Natalizumab

A

2%

61
Q

side effect of Nataluzimab

A

PML

62
Q

CX of plasma exchange to rapidly clear natalizumab has

reversed PML

A

IRIS

63
Q

____ is a monoclonal antibody that targets
CD-52 antigen expressed on T and B lymphocytes,
reduces the number of circulating B cells and, for a
longer period, T cells.

A

Alemtuzumab

64
Q

SE of Alemtuzumab

A

The drug can produce idiopathic

thrombocytopenic purpura and autoimmune thyroiditi

65
Q

__________ a B-cell-depleting monoclonal antibody
that targets CD20 lymphocytes, has been tested in several
trials and found to be effective in reducing relapses and
the accumulation of MRI lesions in a trial of relapsingremitting
cases over 4 years, but long-term safety is still
being established

A

Rituximab,

66
Q

One immunosuppressive drug that interferes with
egress of lymphocytes from lymph nodes,_______
has had a short-term effect on MRI lesion burden and
relapse rate that is comparable or slightly superior to
inject able agents

It causes a lymphopenia by restricting
lymphocytes to the lymph nodes and causes adenopathy.

A

fingolimod,

67
Q

Fatigue, a common complaint
of MS patients, particularly in relation to acute attacks,
responds to some extent to ____ (100 mg morning
and noon), ______ (200 to 400 mg/ d), or pemoline
(20 to 75 mg each morning), ________, or ______

A

amantadine

modafinil

methylphenidate

dextroamphetamine.

68
Q

acute to
subacute onset of blindness in one or both eyes, preceded
or followed within days or weeks by a severe transverse
or ascending myelitis

A

DEVIC

69
Q

Receptor affected in devic

A

autoantibody to the aquaporin-4 water channel

protein

70
Q

In certain parts of the world, this form of aggressive
and usually monophasic demyelinating disease is
more common than is typical MS

A

“Asian optic-spinal MS”

71
Q

Difference of MS and Devic

A

a failure to develop cerebral demyelinating
lesions typical of MS even after years of illness;

theabsence of oligoclonal bands in the CSF;

a tendency toCSF pleocytosis more so than in MS,

and the necrotizingand cavitary nature of the spinal cord lesion, affecting white and gray matter alike with prominent thickening
of vessels but with minimal inflammatory infiltrates

72
Q

This has led to the conclusion that the Devic process
is a _____ disease in contrast to the ______ mechanism
that is proposed for MS

immunoglobulin (Ig) G antineural antibody directed
against aquaporin-4, (NMO antibody)

A

humoral

cellular

73
Q

Distribution of antibodies in DEVIC

A

astrocytic end

feet adjacent to capillaries, pia, and Virchow-Robin spaces

74
Q

acute inflammatory
and demyelinating disease, distinguished pathologically
by numerous foci of demyelination scattered throughout
the brain and spinal cord

A

ADEM

75
Q

viruses associated with ADEM

A

measles, rubella, smallpox, or chickenpox

76
Q

ADEM in adults

A

most adults make good recoveries

77
Q

T or F

ADEM is a humoral disease

A

T

Neurology - Physiology (44 decks)

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