Autosomal dominant transmission of Dejerine-Sottas disease (HMSN III).
case_report · Level V
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- Record sourced from PubMed, PMID 9270606.
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Abstract
Hereditary motor-sensory neuropathy type III (HMSN III) (Dejerine-Sottas disease) is a severe demyelinating neuropathy that is traditionally considered autosomal recessive. We report a father and daughter diagnosed with HMSN III by clinical, electrophysiologic, and pathologic criteria, thus showing that it may be transmitted in an autosomal dominant fashion in selected families.
Medical subject headings
- Genes, Dominant
- Hereditary Sensory and Motor Neuropathy