demyelinating/dysmyelinating disease Flashcards Preview

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Flashcards in demyelinating/dysmyelinating disease Deck (11):
1

What increases a patient's risk of progressive multifocal leukoencephalopathy?

natalizumab
remember that this is demylination of CNS d/t destruction of oligos in AIDS pts. It is a reactivation of JC virus infection.

2

What is acute disseminated encephalomyelitis?

multifocal perivenular inflammation and demylination. associated with measles or VZV or rabies/smallpox vaccinations

3

What is metachromatic leukodystrophy?

autosomal recessive lysosomal storage disease, usually d/t arylsulfatase A deficiency. this causes a buildup of sulfatides, leading to impaired production of the myelin sheath. causes central and peripheral demyelination with ataxia and dementia

4

What is Charcot-Marie-Tooth disease?

aka hereditary motor and sensory neuropathy
progressive hereditary disease associated with defective production of peripheral nerves or myelin sheath. usually autosomal dominant and associated with scoliosis and foot deformities

5

What is Krabbe disease?

autosomal recessive lysosomal storage disease that is caused by a galactocerebrosidase defiency. This causes a build-up of galactocerebroside and psychosine, which destroys the myelin sheath. causes peripheral neuropathym developmental delay, optic atrophy and globoid cells

6

What is adenoleukodystrophy?

X-linked genetic disorder that affects mostly males. distrubts the metab of very long chain fatty acids causing excessive build-up in the nervous system, adrenals, and testes. progressive disease that leads to coma/death and adrenal gland crisis

7

Most common site of partial seizures; characteristics

affect 1 area of the brain, though they can spread and secondarily generalize.
most common site: medial temporal lobe with preceding aura

8

What are the types of partial seizures?

simple partial vs. complex. simple: patient remains conscious by has a motor, sensory, autonomic, or psychic disorder
complex partial causes impaired consciousness

9

Absence seizures

3 Hz seizures w/o post-ictal confusion. blank stares. generalized

10

Atonic seizures

drop seizures

11

What is status epilepticus?

medical emergency wherein a pt has continuous seizures for >30 min or recurrent seizures w/o regaining consciousness.

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