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  • Title: Autosomal dominant transmission of Dejerine-Sottas disease (HMSN III).
    Author: Lynch DR, Hara H, Yum SW, Chance PF, Scherer SS, Bird SJ, Fischbeck KH.
    Journal: Neurology; 1997 Aug; 49(2):601-3. PubMed ID: 9270606.
    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.
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