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The electrophysiologic profile of Dejerine–Sottas disease (HMSN III)
Author(s) -
Benstead Timothy J.,
Kuntz Nancy L.,
Miller Robert G.,
Daube Jasper R.
Publication year - 1990
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880130705
Subject(s) - hereditary motor and sensory neuropathy , medicine , nerve conduction velocity , disease , anatomy , pathology
Electrophysiologic studies in 11 patients with Dejerine‐Sottas disease (hereditary motor and sensory neuropathy type III, HMSN II) showed median and ulnar motor nerve conduction velocities less than 6 m/sec in all but 1 patient. Marked temporal dispersion without conduction block was present in all patients. Uniform slowing in adjacent motor nerves was consistent with other studies of inherited neuropathies, although marked temporal dispersion may make HMSN III more difficult to distinguish from acquired neuropathies than other hereditary conditions. The electrophysiologic features of HMSN III patients were significantly different from a series of patients with other hereditary neuropathies chosen because of very slow nerve conduction velocity.