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ADAMS > Multiple Sclerosis and other inflammatory Demyelinating Diseases > Flashcards

Multiple Sclerosis and other inflammatory Demyelinating Diseases Flashcards

1
Q

generally accepted pathologic criteria of a DEMYELINATING DISEASE (3)

A
  1. destruction of myelin sheaths of nerve fibers with relative sparing of other elements 2. infiltration of inflammatory cells, perivenous distribution 3. lesions that are primarily in white matter
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2
Q

topography of lesions in MS

A

periventricular optic nerves and chiasm spinal cord

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3
Q

histologic appearance of MS in the ACUTE phase

A

partial or complete destruction of myelin, perivenous axons are relatively spared slight degeneration of oligodendroglia variable astrocytic reaction perivascular and para-adventitial infiltration of mononuclear cells and lymphocytes

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4
Q

histologic appearance of CHRONIC lesions in MS

A

thickly matter, acellular glial tissue occasional perivascuar lymphocytes and macrophages descending and ascending wallerian degeneration partial remyelination

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5
Q

4 histologic subgroups in MS

A

pattern I inflammatory lesions made up of T cells and macophages pattern II autoantibody lesion mediated by immunoglobulin and complement pattern III apoptosis of oligodendrocytes, absence of IG, complement and partial remyelination pattern IV oligodendrocyte dystrophy, no remyelination

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6
Q

T/F MS has a uni-modal age specific onset curve, similar to that of infectious and connective tissue diseases.

A

True p 917

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7
Q

T/F There is an increased risk of developing MS with higher and lower latitude.

A

True p918

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8
Q

T/F Approximately 15% of MS patients have an affected relative, with the highest risk of concurrence being observed in the patient’s siblings.

A

True p918

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9
Q

T/F In MS, the concordance rate in dizygotic pairs is similar to that of nontwin siblings.

A

True p918

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10
Q

Certain histocompatibility locus antigens (HLAs) are more frequent in patients with MS than in control subjects The strongest association is

A

DR locus on chromosome 6

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11
Q

Presence of markers or HLAs increases the risk of an individual to develop MS by

A

3-5x

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12
Q

other alleles (aside from HLA loci) identified as heritable risk factors

A

IL - 2Ra IL - 7Ra

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13
Q

MS is mediated by ____cell sensitization to some component of _____

A

T cell myelin

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14
Q

typical feature of MS , temporary induction by heat or exercise of symptoms such as unilateral BOV, tinging and weakness of limb

A

Uhtfoff phenomenon

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15
Q

other situations that can briefly worsen neurologic functioning in MS, and may be confused with relapses of disease

A

smoking, fatigue, hyperventilation, rise in environmental temp

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16
Q

syndromes typical of MS (4)

A

optic neuritis transverse myelitis cerebellar ataxia brainstem syndromes

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17
Q

Lhermitte sign

A

flexion of the neck may induce a tingling, electric -lie feeling down the shoulders and back and less commonly down the anterior thighs

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18
Q

T/F In optic neuritis, there is pain within the orbit, worsened by eye movement or palpation of the globe.

A

True p921

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19
Q

T/F In optic neuritis, both optic nerves are sometimes involved either simultaneously or more commonly within a few days or weeks of one another.

A

True p921

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20
Q

percentage of patients with optic neuritis who will develop other signs of MS

A

50% p921

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21
Q

T/F Recurrent optic neuritis increases the chances of developing MS.

A

True p922

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22
Q

Charcot Triad

A

nystagmus scanning speech intention tremor

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23
Q

inernuclear ophthalmoplegia

A

involvement of MLF paresis of MR on lateral gaze, with coarse nystagmus in the abducting eye

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24
Q

True or False The presence of bilateral INO in a young adult is virtually diagnostic of MS.

A

True p924

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25
Q

common precipitating factors in MS but was not really proven

A

infection trauma pregnancy

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26
Q

variant of MS rapidly progressive, highly malignant form combination of cerebral, brainstem and manifestations evolve over a few weeks may render the patient stuporous, comatose, or decerebrate with prominent cranial nerve and CST abnormalities death may end illness CSF shows cellular response but no oligoclonal bands

A

Acute and Tumor-Like (Tumefactive) MS Marburg Variant

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27
Q

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

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28
Q

CSF findings in typical MS

A

slight mononuclear pleocytosis - usually in the range of 6-10, less than 50 cells in cases of severe demyelinating disease of brainstem - up to 100 presence of gamma globulins, seen as oligoclonal bands in electrophoresis increased total protein content more than 100mg/dL is unusual IgG and IgG index positive test >12% concentration of MBP, elevated - not particularly useful in diagnosis, but reflects destruction of myelin

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29
Q

define IgG index

A

proportion of gamma globulin in reference to total protein of CSF

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30
Q

how many percent of MS cases show several bands in determination for oligoclonal IgG

A

90%

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31
Q

other conditions which show demonstrate bands

A

syphilis, Lyme, subacute sclerosing panencephalitis but can be distinguished from MS clinically

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32
Q

True or False The presence of bands in a first attack of MS is predictive of a chronic relapsing course.

A

True p928

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33
Q

most widely used CSF test for MS

A

oligoclonal bands

34
Q

most helpful ancillary examination in the diagnosis of MS

A

MRI

35
Q

describe acute lesions of MS in T1 and T2 sequences

A

Acute lesions tend to demonstrate tissue expansion diue to edema that is evident as T1 hypointensity and T2 hyperintensity Chronic lesions - T2 hyperintense

36
Q

relation of T1 hypointensity and remyelination

A

T1 hypointensity is inversely proportional to the degreee of remyelination if there is no or scant remyelination, the center of teh chronic lesion gives the appearance of a “black hole”

37
Q

MRI findings of multifocal, well-demarcated, oval or linear, radially oriented lesions adjacent to the ventricular surface usually denotes the typical RRMS when viewed on sagittal form what patter can be seen?

A

Dawsons fingers the lesions extend from the corpus callosum filliform pattern

38
Q

disruption of BBB in early MS can be seen in T1 as _________ after gadolinium

A

hyperintensity one characteristic pattern: C-shaped partial or open ring of abnormal enhancement

39
Q

True or False Spinal lesions of MS occupy only a portion of the transverse surface. They extend longitudinally beyond three contiguous vertebral segments.

A

False DO NOT extend beyond 3 contiguous vertebral segments

40
Q

Diagnostic Criteria for MS

A
  1. 2 or mote typical attacks with objective evidence for both lesions
  2. 2 clinical attacks, 1 objective evidence, laboratory: lesions located in at least 2 of 4 typical locations
  3. 1 attack with objective evidence of 2 or more lesions, laboratory: dissemination over time: simultaneous enhancing and non-enhancing lesions in the said locations, or interval development of new T2 hyperintense lesions
  4. 1 attack with objective evidence on examination for only 1 lesion (CIS), laboratory: dissemination in space, lesions or T2 hyperintensity located in at least 2 of 4, dissemination time demonstrated by simultaneous enhancing and non-enhancing lesions in the above locations, or interval development of new T2 hyperintense lesions
  5. progressive, nonrelapsing deficits suggestive of MS, one year disease progression and dissemination in space as above an doligoclonal bands or elevated IgG index in teh CSF
41
Q

average relapse rate in MS

A

0.3 - 0.4 attacks per year

in 1 yr 30% of patients

in 2nd yr 20% of patients

42
Q

True or False

In MS, pregnancy is typically associated with clinical relapse.

A

False

It is associated with clinical stability and even with improvement (as it is in a number of autoimmune diseases).

p 930

43
Q

True or False

There is an increased risk of exacerbations up to twofold in MS patients, in the first few months postpartum.

A

True

p930

44
Q

True or False

ADEM is an acute illness with widely scattered small demyelinating lesions that is self-limited and monophasic.

A

True

p931

45
Q

why should the neurologists be cautious in initiating treatment regimens for MS in patients with systemic autoimmune and inflammatory diseases?

A

B-interferon may worsen systemic autoimmune illness

46
Q

True or Fasle

CSF in cervical spondylosis is often elevated.

A

True

p932

but with absent oligoclonal bands

and absent elevated IgG

47
Q

Dosing of IV methylprednisolone and oral prednisone in the treatment of acute exacerbations (aborting or shortening an acute attack) of MS or of optic neuritis

A

500-1000mg of methylprednisolone daily for 3-5 days followed by oral prednisone 60-80mg/day and tapering to a lower dosage over a 12-20 day period

48
Q

If it is impractical to administer parenteral methylprednisolone, one may substitute for oral methylpred with the ffg dosing:

A

48 mg in a single daily dose for 1 week

followed by 24mg daily for 1 week and

finally 12g daily for 1 week

OR the equivalent amount of prednisone

49
Q

Dosing for IV Ig in relapsing-remitting MS

A

IV Ig 0.2g/kg for 2 years

50
Q

results of the ONTT

A

use of IV methylprednisolone followed by orla prednisolone sped the recovery from visual loss although at 6 months, little difference between pts treated vs placebo

those treated with orel pred alone, had slightly increased risk of new episoded of ON

51
Q

What two drugs alterthe natural history of RRMS

A

glatiramer

interferon

52
Q

True or False

Interferon B

Trials have shown that subcutaneous injection of this agent every second day for up to 5 years decreases frequency and severity of relapses by almost one-third and also the number of new or enlarging lesions.

A

True

p933

53
Q

role of interfero in progressive MS

there was delay in progression by how many months

A

9-12 months

54
Q

side effects of interferon agents

A

flu-like symptoms, sweating, malaise, beginningseveral hrs after injection up yo 14 hrs

(reduced by pre and post treatment with NSAIDs

prednisone 10mg may be taken few hrs before and after)

exacerbation of headaches in migraineurs

55
Q

mimic actions of MBP

given subcutaneous dily 20mg

A

glatiramer

56
Q

side effects of glatiramer

A

flushing

chest tightness

dyspnea

palpitations

severe anxiety

57
Q

role of azathioprine in MS

A

British and Dutch MS Azathioprine Study Group

no significant advantage to treatment with azathioprine

58
Q

role of oral methotrexate in MS

A

one trial showed that in patients with progressive MS, weekly low-dose of methotrexate resulted in slight improvement difference and produced some reduction in the volume of cerebral lesions on the MRI compared with control cases

59
Q

MOA Natalimumab

A

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

shown to reduce the number of relapses and slowing of the accumulation of MRI lesions

60
Q

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

A

Alemruzumb

61
Q

side effect of Natalimumab

A

appearance of progressive multifocal leukoencephalopathy (PML)

62
Q

the risk of developing PML in patients receiving Natalimumab is 11 per 1000 in the presence of these factors

A

postive anti-JC virus antibodies

long duration of matalimumab use over 24 months

63
Q

side effecs of alemtuzumab

A

appearace of Idiopathic Thrombocytopenic Purpura

Autoimmune thyroiditis

64
Q

B-cell depleting monoclonal antibody that targets CD20 lymphocytes

effective in reducing relapses and accumulation of MRi lesions in RRMS,

long term safety is still being established

A

Rituximab

65
Q

oral immunossuppressive drug for MS that interferes with egress of lymphocytes form lymph nodes

has a short-term effect on MRI lesion burden and relapse rate that is comparable or slightly superior to injectable agents in a randomized trial

A

fingolimod

66
Q

side effects of fingolimod

A

lymphophenia

bardycardia, AV block, macular edema, herpes infections, elevations of LFTs

67
Q

what drug do we give for patients with MS experiencing the ffg symptoms:

fatigue

urinary retention

spastic bladder

sexual dysfunction

A

fatigue: amantadine, modafinil, pemoine, methylphenidate or dextroamphetamine

urinary retention: bethanechol

spastic bladder: propantheline, oxybutynin

sexual dysfunction: sildenafil

68
Q

What do we give for the ffg symptoms in MS patients

spasticity

tremors

A

spasticity: botox, baclofen
tremors: isoniazid with pyridoxine, carbamazepine or clonazepam

69
Q

monophasic, demyelinating disease with simultaneous or successive and usually sever involvement of optic nerves and spinal cord

A

Neuromyelitis optica

Devic Disease

70
Q

Indicate if MS or NMO

  1. presence of oligoclonal bands in CSF
  2. higher tendency to pleocytosis
  3. necrotizing and cavitary nature of teh spinal cord lesion affecting both white and gray matter, centrally located
  4. involves several contiguous longitudinal segments of spinal cord
  5. involves less than 3 contiguous vertebral segments of SC
  6. spinal lesions occupy only a portion of the transverse surface of SC
  7. clinical effects are likely to be permanent
A
  1. MS
  2. NMO
  3. NMO
  4. NMO
  5. MS
  6. MS
  7. NMO
71
Q

antibody found in serum of patients with Dveic disease

A

NMO antibody

IgG antineural antibody directed against aquaporin-4 that binds complement

72
Q

True or False

Devic disease involves humorla process in contrast to the cellular mechanism in MS.

A

True

p936

73
Q

acute encephalitix, myelitic, encephalomyelitic process usually following a febrile illness up to 2 weeks, more commonly seen in children

originally, occurred within a few days of onset of the exanthem of measle, rubella, smallpox or chickenpox

A

Acute disseminated encephalomyelitis

74
Q

True or False

In ADEM, teh encephalitic form is expressed more fully in children than in adults.

A

True

p938

as an acute illness is resolving there is an abrupt onset or over a day or two, of confusion, somnolence, convulsions, headache, fever, neck stiffness.

ataxia is common

75
Q

True or False

The myelitic form of ADEM may simulate a spinal artery occlusion syndrome.

A

True

p939

76
Q

CSF findings in ADEM

A

slight increase in lymphocytes and protein content

77
Q

MRI in ADEM

A

bilateral confluent white matter lesions in both cerebral hemispheres early in the course

78
Q

A postinfectious encephalomeylitis that involves predominantly the cerebellum presents as mild ataxia with variable CST signs

seen in patients with childhood exanthems such as:

A

EBV

CMV

Mycoplasma

Legionella

79
Q

most fulminant form of demyelinating disease almost certainly the sever end of the spectrum of ADEM, affects mainly young adults and children

usually preceded by a respiratory infection of variable duration, sometimes caused by M. pneumoniae

headache, fever, stiff neck, confusion

followed by seizures, hemiplegia/quadriplegia, pseudobulbar paralysis, progressive deepening coma

A

Acute Necrotizing Hemorrhagic Encephalomyelitis

(Acute Hemorrhagic Leukoencephalitis of Weston Hurst)

80
Q

imaging findings in Acute Necrotizing Hemorrhagic Encephalomyelitis

A

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

81
Q

treatment

ADEM and Acute Necrotizing Hemorrhagic Encephalomyelitis

A

high dose steroids

82
Q
A

ADAMS (29 decks)

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