Demyelinating Disorders (krafts) Flashcards

0
Q

What is the ratio of MS in genders?

A

F:M 2:1

Autoimmune common ratio

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1
Q

What is the onset of MS?

A

Before 50

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2
Q

What are some characteristics of MS?

A

Episodic symptoms separated in time

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3
Q

Why do you get symptoms with MS?

A

Due to white matter lesions separated in space

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4
Q

What is the pathogenesis of MS?

A

Genetic plus enviornmental
Maybe ebstein-Barr

Linked to HLA-DR2 (DR2 is the variant that increases the likely of getting MS)
Also some link to IL-2 and IL-7 polymorphisms

T-cells against myelin and secrete cytokines
Maybe B cells play a role

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5
Q

What is HLA?

A

Human leukocyte antigen

Encode MHC

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6
Q

How do you diagnosis MS?

A

Oligoclonal bands in CSF

See immunoglobulins in the CSF in different proportions that the blood?

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7
Q

What is the morphology of MS?

A

Plaques adjacent to ventricles

Active plaques - demyelination occurring, responsible for symptoms

  • T cells cuffing vessels
  • axons preserved

Inactive plaque - no myelin

  • decreased Oligodendrocytes
  • gliosis
  • decreased number of axons
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8
Q

How do plaques behave?

A

Follow blood vessels

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9
Q

How can you tell a plaque Histologically?

A

With an H&E stain

  • paler
  • less cellular
  • lymphocytes hanging around vessels (cuffing)
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10
Q

What do myelin stains stain myelin?

A

A dark bluish purple

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11
Q

What is a common symptom of MS early?

A

Unilateral vision problems

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12
Q

What are clinical features of MS?

A

Many different symptoms

  • unilateral visual impairment
  • ataxia
  • nystagmus
  • cranial nerve signs

-motor impairment of trunk

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13
Q

What is acute disseminated encephalomyelitis?

A

ADEM

Diffuse Monophasic demyelination following a viral infection

Rapid - headache, lethargy, coma

USUALLY in children
Rare
Fatal in 10%, rest completely recover
Acute autoimmune against myelin

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14
Q

What is acute necrotizing hemorrhagic encephalomyelitis?

A

Fulminant CNS demyelination

Children and YOUNG ADULTS

Preceded by URI
Very fatal, significant defects in survivors
May be a hyper acute form of ADEM

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15
Q

What is Central Pontine Myelinolysis?

A

Symmetric loss of myelin in basis pontes and part of pontine tegmentum

Rapidly evolving quadriplegia (flaccid to rigid)

Monophasic disease, lesions happen all at once

16
Q

What causes Central Pontine Myelinolysis?

A

Rapid correction of hyponatremia (low sodium)