Demyelinating Diseases Flashcards by Ela Barcelon

Generally accepted pathologic criteria for multiple sclerosis (3)

1 myelin sheath destruction with relative sparing of other elements
2 predominantly perivenous infiltration of inflammatory cells
3 primarily white matter lesions

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Define MULTIPLE SCLEROSIS

Progessive chronic condition characterized clinically by episodes of focal disorders of the optic nerves, spinal cord, & brain, which remit & recur over many years

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Pathologic findings in Multiple Sclerosis

sharply delineated periventricular white matter lesions in the brain & spinal cord that do not extend beyond the entry zones of cranial or spinal nerves

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Histologic appearance of ACUTE lesions in MS (4)

loss of myelin in the perivenous distribution
slight oligodendroglial degeneration
variable astrocytic reaction
perivascular & para-adventitial mononuclear & lymphocytic infiltration

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Histologic appearance of CHRONIC lesions in MS (4)

thickly matted, acellular glial tissue
occasional perivascular lympthocytes & macrophages
walleraian degeneration of long fiber tracts
shadow patches : partial remyelination of undamaged fibers
cavitation : destruction of supporting tissues & vessels

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4 Histologic subgroups or patterns of MS

1 inflammatory lesions made up of T cells & macrophages alone
2 an autoaAb lesion mediated by Ig & complement
3 characterized by apoptosis of oligodendrocytes
& no Ig, complement, & + partial remyelination
4 showing only oligodendrocyte dystrophy & no remyelination
*3-4 show a primary oligodendroglial degeneration

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True or False.

MS has a bimodal age-specific onset curve

False

unimodal

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Peak age of developing symptoms of MS

30-40 years of age

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The strongest genetic factor thought to be associated to developing MS

DR locus on chromosome 6

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Thought to be markers of MS susceptibility gene, and the presence of any of these increases the risk of developing MS by how much?

HLA-DR2
HLA-DR3
HLA-B7
HLA-A3
3-5x increased risk

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Other than the HLA loci, thse too were said to increase the susceptibility to develop MS

IL2Ralpha

IL7Ralpha

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Most immunologists believe that MS is medidated by

T-cell sensitization to some component of myelin

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What is the Uhthoff phenomenon & what is its basis?

worsening of neurologic symptoms of MS patients upon exposure to heat.

Nerve conduction in demyelinated & remyelinated fibers are sensitive to subtle changes in metabolic & environmental changes

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4 MS Syndromes

1 optic neuritis
2 transverse myelitis
3 cerebellar ataxia
4 brainstem syndromes

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The pain in Lhermitte sign is most probably due to

increased sensitivity of demyelinated axons to the stretch or pressure on the spinal cord induced by neck flexion

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Prognosis of optic neuritis

Good. half recover completely

treatment did not influence outcome

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What is Pulfrich effect?

psychophysical percept wherein lateral motion of an object in the field of view is interpreted by the visual cortex as having a depth component, due to a relative difference in signal timings between the two eyes

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What is Charcot triad?

1 scanning speech
2 nystagmus
3 intention tremor

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True or False. the presence of bilateral internuclear ophthalmoplegia in a young adult is virtually diagnostic of MS.

True

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True or False. the occurrence of transient facial hypesthesia or anesthesia or of trigeminal neuralgia in a young adult should always suggest the diagnosis of MS implicating the intramedullary fibers of the fifth cranial nerve

True

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useful in controlling spontaneous attacks in MS

carbamazepine

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blocks painful tonic spasms that are elicited by hyperventilation in MS

acetazolamide

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True or False. Facial palsy along the lines of Bell’s palsy can be a sign of MS

false

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Most common precipitating factors of MS

infection
trauma
pregnancy

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Most common precipitating factors of MS (though not firmly established)

infection trauma pregnancy

Variant of MS that is rapidly progressive and highly malignant. A combination of cerebral, brainstem, and spinal manifestations evolves over a few weeks, rendering the patient stuporous, comatose, or decerebrate with prominent cranial nerve and corticospinal abnormalities

Marburg type or Tumefactive type

Variants of MS consisting of diffuse sclerosis

Balo & Schilder Disease

CSF oligoclonal bands is positive in what proportion of MS patients?

90%

Positive test of CSF IgG index in MS is?

>12%

True or False. The finding of oligoclonal bands in a suspected MS patients connotes that the disease is already chronic

True

True or False. Oligoclonal bands in blood of MS patients is also diagnostic along with the clinical features consistent with MS

False

Most commonly used diagnostic test in MS

CSF oligoclonal bands

Most helpful diagnostic test in MS

MRI

In MS. T1 hypointensity is ___________ proportional to the degree of remyelination

inversely

filiform pattern in the corpus callosum in the MRI of MSI patients

Dawson's fingers

Cranial MRI findings in MS

- T2 hyperintense, T1 hypointense - multifocal, well-demarcated, oval or linear, radially oriented lesionsadjacent to the ventricular surface - C-shaped partial or medially open ring of abnormal enhancement

___% of definite MS & ___% of probable or possible MS have abnormal VER.

70 | 60

___% of definite MS & ___% of probable or possible MS have abnormal SSEP

60 | 40

___% of definite MS & ___% of probable or possible MS have abnormal BAER

40 | 20

diagnostic study to dedmonstrate axonal loss or thinning of the retina

optical coherence tomography

4 typical locations of T2 hyperintesities in MS

1 periventricular 2 juxtacortical 3 infratentoria 4 spinal cord

The most predictive of long term disability in MS

degree of disability at 5 years from the onset of the first symptoms

True or False. IV methylprednisolone administered at 1 g/d for 5 days per month over 5 years, was shown to reduce disability and brain atrophy and total volume of hypodense lesions on Tl-weighted MRI

True | study by Zivadinov

MPPT dose for MS

1) 0.5-1g/day x 3-5days then oral prednisone 60-80mg/day x taper 12-20 days 2) oral methylprednisolone 48mg/day x 1 week 24mg/day x 1 week 12mg/day x 1 week

IV Ig dose for MS

0.2g/kg monthly x 2 years

The main problem in MS patients treated with interferon

development of antibodies against it

Monoclonal antibodies used in MS and their targets (4)

Natalizumab - anti alpha integrins to block lymphocyte, monocyte adhesion to endolium, migration Alemtuzumab - anti CD52 antigen to reduce B, T cells Rituximab - anti CD20 lymphocytes, depleting Bcell monoclonal Ab Ocrelizumab - anti CD20

Monoclonal antibodies used in MS and their side effects (4)

Natalizumab - PML Alemtuzumab -ITP, thyroiditis Rituximab - infection, lymohoma Ocrelizumab - infection, lymohoma

Mode of action of glatiramer acetate

mimics myelin basic protein

Side effects of fingolimod

lymphopenia, adenopathy, bradycardiam AV block, macular edema, herpes infection, increased LFTs

Medications that may be used to address fatigue in MS

``` amantadine modafinil permoline methylphenidate dextroamphetamine ```

Isoniazid can be used in MS to address what?

severe postural tremor

Distinctive characteristics of Devic's Disease from MS (6)

1 failure to develop cerebral demyelinating lesions even after years of illness 2 absence of CSF oligoclonal bands 3 tendency to CSF pleocytosis more so than in MS 4 necrotizing cavitary nature of the spinal cord lesion, affecting white and gray matter alike with prominent thickening of vessels 5 but with minimal inflammatory infiltrates 6 involve several contiguous longitudinal segments of the spinal cord

Antibody specific to Devic's Disease and its sensitivity and specificity?

NMO antibody anti aquaporin 4 76% sensitive 94% specific

Criteria for the diagnosis of NMO which is 99% senstivie and 90% specific

2 of the following: 1 longitudinally extensive myelopathy 2 positive antibodies 3 nitial MRI that is not characteristic for MS

The only histopathologic characteristic of ADEM that distinguishes it from MS

multifocal meningeal infiltration

Most fulminant form of demyelinating disease

Weston Hurst Disease | acute hemorrhagic leukoencephalitis

Microorganism associated with Weston Hurst Disease

none. most cases has indeterminate cause. | Mycoplasma pneumoniae

Neuroimaging findings that distinguish Weston Hurst Disease from ADEM

1 size of the lesions 2 hemorrhagic character 3 extent of the surrounding edema

Neuroimaging findings in Weston Hurst Disease

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

Distinctive pathologic characteristic of Weston Hurst Disease

destruction of white matter to the point of liquefaction

Mcdonald Criteria for MS

``` ≥2 attack, ≥ 2 lesion ≥2 attack, 1 lesion = DIS 1 attack, ≥2 lesion = DIT 1 attack, 1 lesion = DITS 0 attack = 1 year of disease progression and 2 out of 3 criteria: DIS brain, DIS cord, +CSF ```

Demyelinating Diseases Flashcards

(62 cards)