X-LINKED BULBOSPINAL MUSCULAR-ATROPHY (KENNEDY SYNDROME) - A REPORT OF 3 CASES
Articles > Article · 1993
Authors: CUMHUR ERTEKİN, Hadiye ŞİRİN
ACTA NEUROLOGICA SCANDINAVICA, Journal Indexed in SCI Expanded, International, 1993, vol 87, pp 56-61
Summary
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.