Pathology of Demyelinating Disease Flashcards

1
Q

Multiple Sclerosis

Pathogenesis:

Genetic component:

Morphology:

Histology:

A

Multiple Sclerosis is an autoimmune demyelinating disorder characterized by distinct episodes of neurologic deficits, separated in time, attributable to white matter lesions that are separated in space.

Pathogenesis: lesions are secondary to an autoimmune response directed against components of the myelin sheath (T-Lymphocyte initiated response)

Genetic component: DR2

Morphology: White Matter disease

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2
Q

What is this?

age at presentation:

histology:

A

Multiple Sclerosis Lesion

age at presentation: usually presents in early adulthood but can be any age. 2X F>M

histology:

active plaques- ongoing breakdown w/ abudnant lymphocytes

inactive plaque- no active breakdown, no inflammatory cells, no myelination of axons, decreased oligodendrocytes but increased astrocytes and gliosis

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3
Q

Multiple Sclerosis

  1. Clinical Features:
  2. CSF examination
  3. what type of imaging can be used to assess disease progression?
A

Multiple Sclerosis

  1. Clinical Features: b/c a lesion can occur anywhere in the CNS, clinical lesions can occur anywhere- however some symptoms occur routinely
    1. Unilateral vision impairment
    2. ataxia, nystagmus, and cranial nerve signs (brainstem involvement)
  2. CSF examination:
    1. Mildly elevated protein and pleocytosis (increased lymphocytes)
    2. increased IgG levels and oligoclonal IgG bands on immunoelectrophoresis
  3. An MRI can be used to assess disease progression?
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4
Q

Neuromyelitis Optica

  1. definition:
  2. characteristics:
A

Neuromyelitis Optica

  1. definition: synchronous bilateral optic neuritis and spinal cord demyelination
    1. synchronus bilateral blurry vision and weakness or paralysis in the legs or arms, loss of sensation, and problems with bladder/bowel function
  2. characteristics:
    1. women>men
    2. less recovery than MS from initial attack
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5
Q

Neuromyelitis Optica

  1. Antibodies against ________
    1. what is the MOA for these antibodies?
  2. CSF examination:
A

Neuromyelitis Optica

  1. Antibodies against aquaporin-4
    1. these antibodies injure astrocytes through complement-dependent mechanisms.
  2. CSF examination: often see neutrophils
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6
Q

Neuromyelitis Optica

  1. Histology:
  2. Therapy:
A

Neuromyelitis Optica

  1. Histology: within damaged areas of white matter:
    1. necrosis, inflammatory cells
    2. vascular deposition of immunoglobulin and complement
  2. Therapy: reduction of Ab burden through:
    1. plasmaphersis
    2. depletion of B-cells via anti- CD20
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7
Q

Acute Disseminated Encephalomyelitis

  1. acute monophasic demyelinating disease that follows a _________.
  2. How long does it take to develop?
    1. what are the symptoms?
  3. Mortality rate?
A

Acute Disseminated Encephalomyelitis

  1. acute monophasic demyelinating disease that follows a viral illness (rarely after immunization).
  2. How long does it take to develop? 7-14 days after illness
    1. symptoms include: lethargy, headache, coma
  3. Mortality rate? 20% die. 80% recover
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8
Q

Acute Disseminated Encephalomyelitis

  1. Morphology
    1. histology
A

Acute Disseminated Encephalomyelitis

  1. Morphology
    1. histology: myelin loss with relative preservation of axons in white matter
    2. all lesions appear similar (this is different than MS)
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9
Q

Acute Necrotizing Hemorrhagic Encephalomyelitis

  1. definition:
  2. almost always preceeded by _______
  3. mortality rate:
  4. histology:
A

Acute Necrotizing Hemorrhagic Encephalomyelitis

  1. definition: Fulminant (sudden/severe) syndrome of CNS demyelination of young adults and children
  2. almost always preceeded by upper respiratory infection
  3. mortality rate: high fatality rate
  4. histology: similar to acute necrotizing hemorrhagic encephalomyelitis but more severe and includes damage to small vessels, with subsequent bleeding
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10
Q

Central Pontine Myelinolysis

  1. definition:
  2. occurs as a result of:
  3. associated with?
    4.
A

Central Pontine Myelinolysis

  1. definition: acute disorder characterized by loss of myelin in the basis pontis and portions of the pontine tegmentum, typically in a roughly symmetric pattern
  2. occurs as a result of: rapid correction of hyponatremia (usually 2-6 days after the correction)
  3. associated with?
    1. other severe electrolyte imbalances
    2. structures other than the pons
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11
Q

What is this?

  1. Pathophysiology:
  2. Clinical features:
  3. take away point:
A

Central Pontine Myelinolysis?

  1. Pathophysiology: rapid increases in osmolality damage oligodendrocytes via uncertain mechanisms
    1. no inflammation and preservation of neurons and axons
  2. Clinical features: rapid evolving quadriplegia
    1. may be fatal
    2. may lead to severe long-term deficits
      1. locked in syndrom
  3. take away point: CORRECT HYPONATREMIA SLOWLY
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