Gutmann Clinical Neurophysiological Testing
Terms
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CMT- 1A
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In 70% of patients.
severe demyelination
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Do ppl have CMT when they're young?
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Yes.
Conduction velocities are still slow, but may appear asymptomatic.
When axons start degenerating, you start seeing the symptoms.
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Genetics of CMT disease
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Autosomal dominant
- What happens with a segmental loss of myelin?
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no saltatory conduction
body increases number of Na channels
Speed of conduction lowers
- What's affected first in CMT disease?
- Distal muscles. (length principle)
- What's Charcot-Marie-Tooth disease?
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Hypertrophic neuropathy.
Prominent slowing of conduction velocities
Involves both Demyelination and axonal degeneration.
This interferes with the metabolic machinery of the nerve.&nbs
- What's demyelination?
- Loss of myelin from internodal segments resulting in a slowing of conduction
- What's that "jump" at Nodes of Ranvier?
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Saltatory conduction.