AUTOSOMAL-DOMINANT TRANSMISSION OF DEJERINE-SOTTAS-DISEASE (HMSN-III)

Authors
LYNCH DR HARA H YUM SW CHANCE PF SCHERER SS BIRD SJ FISCHBECK KH
Citation
Dr. Lynch et al., AUTOSOMAL-DOMINANT TRANSMISSION OF DEJERINE-SOTTAS-DISEASE (HMSN-III), Neurology, 49(2), 1997, pp. 601-603
Citations number
10
Categorie Soggetti
Clinical Neurology
Journal title
Neurology → ACNP
ISSN journal
00283878
Volume
49
Issue
2
Year of publication
1997
Pages
601 - 603
Database
ISI
SICI code
0028-3878(1997)49:2<601:ATOD(>2.0.ZU;2-3
Abstract
Hereditary motor-sensory neuropathy type III (HMSN III) (Dejerine-Sott as disease) is a severe demyelinating neuropathy that is traditionally considered autosomal recessive. We report a father and daughter diagn osed with HMSN III by clinical, electrophysiologic, and pathologic cri teria, thus showing that it may be transmitted in an autosomal dominan t fashion in selected families.