Chap 36: Demyelinating Disease Flashcards by Josh Abejero

Generally accepted pathologic criteria for demyelinating dz

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

Relapsing-remitting pattern

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Pattern of MS

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

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Lesion of MS is noteworthy since it is localized

perventricular where subependymal veins line

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

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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Dominant mechanism in demyelination in MS

Pattern II: antibody and complement mediated phagocytosis

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Age of onset of MS

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

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

TRUE

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Other risk factors for MS aside from latitude

Vit D and sun exposure

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

20 years

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Percentage risk of MS px to have relatives

15%

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Strongest genetic association w/ MS

DR locus of Chromosome 6

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

experimental allergic encephalomyelitis

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Autoantibodies inconsistently found against

MOG MBP

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Main physiologic effect of demyelination

Impede saltatory conduction

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Typical symptom of optic neuritis

reduction in intensity of color red (desaturation)

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

Uhthoff phenomenon

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

0.5’C

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

Smoking Fatigue Hyperventilation Rise envt temp

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A useful finding that a patient w/ MS may present

Symptom of one leg but w/ signs in both

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Common modes of onset of MS

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

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

Clinically isolated syndrome

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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

Lhermitte sign

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Typical age of onset for relapsing-remitting pattern

<40 y/o

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

FALSE. SHORT LIVED PAIN

Diffrentiate papilledema from papillitis

Papillitis presents w/ severe and acute visual loss

Chance of complete recovery from optic neuritis

50%

Visual problem that may persist after optic neuritis

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

Improvement of optic neuritis begins around

2 weeks from onset

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

FALSE, MORE than HALF

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

FALSE. it is LOWER

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

TRUE

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

TRUE

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

FALSE. Asymmetrical and incomplete w/ long tract signs

Consideration if recurrent myelitis w/ absent cerebral dissemination

SLE Sarcoidosis Sjorgen MCTD Dural fistulas / AVM

Triad of nystagmus, scanning speech, intention tremor

Charcot triad

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

Bilateral INO Transient facial hypesthesia, anesthesia

Most common manifestation of progressive MS

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

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

TRUE

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

Morbid optimism

Drugs used to alleviate fatigue in MS

Modafinil, Amantadine

Most common precipitating factors for acute MS attacks

Infection Trauma Pregnancy

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

TRUE

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

Tumefactive MS (Marburg variant)

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

"Open ring" enhancement in T1 MRI

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

Concentric sclerosis of Balo

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

Schilder disease Ddx: Diffuse cerebral neoplasm, Adrenoleukodystrophy, PML

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

Moderate mononuclear pleocytosis

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

Oligoclonal bands

Disease entities that may have oligoclonal band

Syphillis Lyme SSPE

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

FALSE, not always

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

Chronic relapsing course

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

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

MRI sequence particularly sensitive to detecting plaque lesions

T2 sequence

MRI appearance of MS Acute lesions Chronic lesions

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

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

FALSE. inversely proportional

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

Dawson fingers

Disruption of BBB due to inflammation is shown in MRI as

abn T1 hyper in enhancement

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

NMO

Test that are sensitive to detect existence of additional asymptomatic lesion

VER BAER Somatosensory evoked response

Four typical locations in MS

Periventricular Juxtacortical Infratentorial Spinal cord

Two features to consider in MS

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

One clinical feature is diagnostic for MS if

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

If 2 clinical attacks w/ objective evidence for one lesion

MRI lesions in at least two typical locations

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

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

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

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

If Progressive MS, nonrelapsing, deficits suggestive of MS

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

The most predictive feature for long term disability is

degree of disability at 5 yrs from first symptoms

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

TRUE

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

Few months postpartum

Average duration of illness is

30 years

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

Bechet disease

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

TRUE

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

FALSE, other way around

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

FALSE. NO EVIDENCE

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

Oral Prednisone

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

Interferon beta & glatiramer

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

IFN-beta-1a

One issue with long term administration of interferon

Antibody devt to drug

Side effects seen in interferon

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

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

Glatiramer

Advantage of glatiramer over interferon

Antibodies do not develop

T/F. No advantage w/ Azathioprine in MS

TRUE

Drugs that can be used for chronic progressive MS

Prednisolone plus cyclophosphamide Low dose oral MTX Mitoxantrone

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

Natalizumab

Notorius side effect of Natalizumab in relation to use for MS

PML

Monoclonal antibodies used for MS

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

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

Fingolimod

Drugs used for Fatigue in MS

Modafinil Amantadine Methlyphenidate

Drug used for urinary retention in MS

bethanecol

Drug used for spastic bladder, used intermittently

Propantheline Oxybuntynin

Drugs used to reduce paroxysmal symptoms of MS

CBZ or gabapentin

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

NMO

Antibody seen in NMO

Aquaporin-4 antibody

Spinal cord lesions in NMO are typically

necrotizing

The antibodies of NMO are mostly seen in

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

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

Longitudinally extensive myelopathy MRI not characteristic of MS

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

TRUE

Drug that suggest clinical improvement in NMO

Methylprednisolone plus Azathioprine

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

TRUE

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

TRUE

By what immune mechanism does NMO work

humoral response, compared to MS cellular response

Vaccine assoc w/ postvaccinal ADEM

Rabies vaccine (DEV type)

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

Acute Necrotizing Hemorrhagic Encephalomyeltis (of Weston Hurst)

Distinctive pathologic finding of Acute Necrotizing Hemorrhagic Encephalomyeltis

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

White matter cerebral disease associated after transplantation and attributed to PML

Graft-vs-Host Disease

Chap 36: Demyelinating Disease Flashcards

(106 cards)