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
MC Precipitants of MS
The most common are infection, | trauma, and pregnancy.
26
many plaques are of the same age and the confluence of
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)
27
Usually the CSF shows a cellular response but no | oligoclonal bands.
True
28
_____ 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.
The concentric sclerosis of Balo
29
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.
diffuse sclerosis
30
CSF test_____ may in fact be the only measure | of activity of the disease.
pleocytosis
31
The presence of _____in a first attack of MS is predictive of a chronic relapsing course,
bands
32
IgG index considered positive
a positive test is considered to be greater than 12 percent of the total protein.
33
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
MRI
34
The ______sequence is particularly sensitive in detecting | lesions in the brainstem, cerebellum, and spinal cord.
T2
35
The presence of T1 hypointensity depends on the extent | of _____ of the lesion
remyelination
36
T1 hypointensity | was inversely proportional to the degree of ______
remyelination
37
MRI pattern that differentiates MS from abscess
C-shaped partial or open ring of abnormal | enhancement;
38
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
cortical atrophy
39
Abnormalities of visual evoked responses have been found in approximately ____ percent of patients with the clinical features of definite MS
70
40
____ is a technique for creating two- and three-dimensional images of the optic nerve and retina.
Optical coherence tomography (OCT)
41
Measures degree of disability inMS
Kurtzke Disability Status Scale
42
in approximately _______ percent of cases, the clinical course lacks periodic relapses and is almost evenly progressive from the beginning (primary progressive MS;
10
43
Highest rate of relapse in pregnancy
first trim, 1.2 attacks
44
effect of methylprednisolone administered at 1 g/ d | for 5 days per month over 5 years
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
T or F A randomized trial comparing oral and intravenous methylprednisolone in acute relapses of MS demonstrated no clear advantage of the intravenous regimen
t
46
Oral methylpredinsolone loading
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
methylprednisolone 500 mg orally for | 5 days had a beneficial effect on visual function at ________ However, at 8 weeks, no effect could be shown
1 and | 3 weeks.
48
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
T
49
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.
IFNB
50
Dose of IFN B
The dose | currently used is 30 meg, or 6.6 million units
51
longer term administration of | interferon is the development of _____ to the drug.
antibodies
52
After a period of years, _____ of patients demonstrate antibodies with daily administration,
30 percent
53
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;
flu-like symptoms,
54
Complication in patients with a monoclonal gammopathy who receive interferon
"systemic capillary leak syndrome
55
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
Controlled High Risk Subjects Avonex Multiple Sclerosis Prevention Study (CHAMPS
56
synthesized to mimic the actions of myelin basic protein, a putative autoantigen in MS, is given daily in subcutaneous doses of 20 mg
Copolymer I (glatiramer acetate),
57
_______ 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
mitoxantrone,
58
_____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
Natalizumab
59
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
AFFIRM
60
Anaphylaxis in Natalizumab
2%
61
side effect of Nataluzimab
PML
62
CX of plasma exchange to rapidly clear natalizumab has | reversed PML
IRIS
63
____ 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.
Alemtuzumab
64
SE of Alemtuzumab
The drug can produce idiopathic | thrombocytopenic purpura and autoimmune thyroiditi
65
__________ 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
Rituximab,
66
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.
fingolimod,
67
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 ______
amantadine modafinil methylphenidate dextroamphetamine.
68
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
DEVIC
69
Receptor affected in devic
autoantibody to the aquaporin-4 water channel | protein
70
In certain parts of the world, this form of aggressive and usually monophasic demyelinating disease is more common than is typical MS
"Asian optic-spinal MS"
71
Difference of MS and Devic
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
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)
humoral cellular
73
Distribution of antibodies in DEVIC
astrocytic end | feet adjacent to capillaries, pia, and Virchow-Robin spaces
74
acute inflammatory and demyelinating disease, distinguished pathologically by numerous foci of demyelination scattered throughout the brain and spinal cord
ADEM
75
viruses associated with ADEM
measles, rubella, smallpox, or chickenpox
76
ADEM in adults
most adults make good recoveries
77
T or F ADEM is a humoral disease
T