Journal article
Acquired versus familial demyelinative neuropathies in children
Muscle & nerve, Vol.8(3), pp.205-210
03/1985
DOI: 10.1002/mus.880080305
PMID: 4058465
Abstract
The electrophysiologic differences between chronic acquired demyelinative neuropathy and the demyelinative form of Charcot‐Marie‐Tooth disease have recently been reported. The present report extends these observations to include the genetically determined demyelinating neuropathies seen in metachromatic leukodystrophy, Krabbe's leukodystrophy, and Cockayne's syndrome. The electrophysiologic features of metachromatic leukodystrophy (five patients), Krabbe's (four patients), and Cockayne's syndrome (three patients) were all similar. There was uniform slowing of conduction (both in different nerves and in different nerve segments), and conduction block was not seen. These findings are consistent with a uniform degree of demyelination in multiple nerves and throughout the entire length of individual axons. Thus, uniform slowing of nerve conduction constitutes strong evidence for a familial demyelinative neuropathy, as opposed to the multifocal slowing seen in acute and chronic acquired demyelinative neuropathy.
Details
- Title: Subtitle
- Acquired versus familial demyelinative neuropathies in children
- Creators
- Robert G MillerLudwig GutmannRichard A LewisAustin J Sumner
- Resource Type
- Journal article
- Publication Details
- Muscle & nerve, Vol.8(3), pp.205-210
- Publisher
- Wiley Subscription Services, Inc., A Wiley Company; Hoboken
- DOI
- 10.1002/mus.880080305
- PMID
- 4058465
- ISSN
- 0148-639X
- eISSN
- 1097-4598
- Number of pages
- 6
- Language
- English
- Date published
- 03/1985
- Academic Unit
- Neurology
- Record Identifier
- 9984020647202771
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