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X‐linked bulbospinal muscular atrophy (Kennedy's syndrome): a report of three cases
Author(s) -
Ertekin C.,
Sirin H.
Publication year - 1993
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1993.tb04076.x
Subject(s) - spinal muscular atrophy , atrophy , electrophysiology , medicine , weakness , peripheral , electromyography , physical medicine and rehabilitation , neuroscience , psychology , pathology , anatomy , disease
Two cases of X‐linked bulbo‐spinal muscular atrophy and one sporadic case with the same clinical features are presented. All three cases were extensively studied by electrophysiological methods. One of the patients showed some transient weakness, which was partly improved by pyridostigmin. In this patient the Decrement test and jitter revealed a mild degree of motor‐end plate dysfunction. Clinical and electrophysiological findings obtained from all three patients suggest that in Kennedy syndrome cell bodies of group IA muscle afferents are also affected selectively, with other peripheral afferents.