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CNS Pathology > Demyelinating Disorders > Flashcards

Demyelinating Disorders Flashcards

1
Q

What cells myelinate the CNS?

A

Oligodendrocytes

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2
Q

What cells myelinate the PNS?

A

Schwann cells

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3
Q

What are leukodystrophies?

A

Inherited mutations in enzymes necessary for production or maintenance of myelin

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4
Q

What enzyme is deficient in metachromatic leukodystrophy?

A

Arylsulfatase deficiency; autosomal recessive disorder

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5
Q

What happens due to the arylsulfatase deficiency in metachromatic leukodystrophy?

A

Myelin cannot be degraded and accumulates in the lysosomes of oligodendrocytes

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6
Q

What enzyme is deficient in Krabbe disease?

A

Galactocerebroside-beta-galactosidase is deficient; autosomal recessive

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7
Q

What accumulates in Krabbe disease?

A

Galactocerebroside accumulates in marcophages

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8
Q

What causes adrenoleukodystrophy?

A

Impaired addition of coenzymeA to long chain fatty acids; X-linked defect

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9
Q

What is accumulated in adrenoleukodystrophy?

A

Accumulation of fatty acids damages adrenal glands and white matter of the brain

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10
Q

What is multiple sclerosis?

A

Autoimmune destruction of **CNS myelin and oligodendrocytes **

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11
Q

What gene is associated with multiple sclerosis?

A

HLA-DR2

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12
Q

In what region is multiple sclerosis commonly seenm?

A

Regions away from the equator

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13
Q

How does multiple sclerosis present?

A

Relapsing neurologic deficits with periods of remission

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14
Q

What are the clinical features of multiple sclerosis?

A

Features depend on strcture that is affected

Blurred vision in one eye (optic nerve)

Vertigo and scanning speech mimicking alcohol intoxication (brainstem)

Internuclear ophthalmoplegia (medial longitudinal fasciculus)

Hemiparesis or unilateral loss of sensation (cerebral white matter, usually periventricular)

Lower extremity loss of sensation or weakness (spinal cord)

Bowel, bladder and sexual dysfunction (ANS)

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15
Q

How is the diagnosis for multiple sclerosis made?

A

MRI - reveals plaques (areas of white matter demyelination)

Lumbar puncture - Increased immunoglobulins with oligoclonal IgG bands, increased lmyphocytes and myelin basic protein

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16
Q

What is seen on gross exam of MS?

A

Grey-appearing plaques in the white matter

17
Q

Tx for MS

A

Treat acute attacks with high dose steroids

Long term treatment with interferon-beta to slow progression

18
Q

What is subacute sclerosing panencephalitis?

A

progressive, debilitating encephalitis leading to death

19
Q

What is the cause of subacute sclerosing panencephalitis?

A

Slow progressing, persistent infection of the brain by measles virus

20
Q

What can be found in the neurons and oligodendrocytes in subacute sclerosing panencephalitis?

A

Viral inclusions

21
Q

What is progressive multifocal leukoencephalopathy?

A

JC virus infection of oligodendrocytes (white matter)

22
Q

What causes reactivation of a latent JC virus infection?

A

Immunosupression (ie AIDS or leukemia)

23
Q

What is the clinical presentation of progressive multifocal leukoencephalopathy?

A

Rapidly progressive neurologic signs (visual loss, weakness, dementia) leading to death

24
Q

What is central pontine myelinolysis?

A

Focal demyelination of the pons (anterior brain stem)

25
Q

What causes central pontine myelinolysis?

A

Due to rapid IV correction of hyponatremia in severely malnourished patients

26
Q

What is the classical presentation of central pontine myelinolysis?

A

Acute bilateral paralysis (“Locked in” sundrome)

27
Q

What is Charcot’s classic triad of MS?

A

SIN

Scanning speech

Intention tremor (also incontinence and internuclear ophthalmoplegia)

Nystagmus

28
Q

What is acute inflammatory demyelinating polyradiculopathy?

A

Autoimmune condition that destroys Schwann cells causing inflammation and demyelination of peripheral nerves and motor fibers

29
Q

Clinical features of acute inflammatory demyelinating polyradiculopathy

A

Symmetric ascending muscle weakness/paralysis beginning in lower extremities

Facial paralysis in 50% of cases

Almost all patients survive with full recovery after weeks to months

30
Q

What are the CSF findings in acute inflammatory demyelinating polyradiculopathy?

A

Increased CSf protein with normal cell count

31
Q

Tx for acute inflammatory demyelinating polyradiculopathy

A

Respiratory support until recovery

Plasmapheresis

IV IG

CNS Pathology (11 decks)

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