Peripheral motor neuropathy associated with autonomic dysfunction in two sisters: New hereditary syndrome?
✍ Scribed by Lisker, Rubén ;García-Ramos, Guillermo ;de la Rosa-Laris, Carlos ;Díaz-Mitoma, Francisco ;Opitz, John M.
- Publisher
- John Wiley and Sons
- Year
- 1981
- Tongue
- English
- Weight
- 404 KB
- Volume
- 9
- Category
- Article
- ISSN
- 0148-7299
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✦ Synopsis
Abstract
We describe two sisters with distal, slowly progressive muscular weakness and hypotrophy since childhood, autonomic dysfunction characterized by profuse sweating, distal cyanosis related to cold weather, orthostatic hypotension, and esophageal achalasia. Nerve conduction velocity of several motor nerves was slow, and although no sensory abnormalities were present, sural nerve biopsy revealed severe nonspecific demyelination. No similar patients could be found in the literature and we therefore suggest the possibility that these individuals have a newly recognized hereditary syndrome.