[Sensory nerve involvement in X-linked bulbospinal amyotrophy (Kennedy syndrome). Contributions of electrophysiologic and histologic data].
Two new cases of X-chromosome linked bulbospinal muscular atrophy associated with gynecomastia (described by Kennedy in 1968) are reported. In one patient, an electrophysiological study and a muscle nerve biopsy were performed. Motor nerve conduction studies were normal but sensory action potentials were small or unrecordable in the absence of clinical sensory loss. Superficial peroneal nerve biopsy showed axonal lesions. These findings suggest that this disease is not purely due to degeneration of spinal motor neurons and is also associated with an axonopathy.
Penisson-Besnier
I
I
Service de neurologie A, Laboratoire de neuro-immunologie et neuropathologie, INSERM U 298, Angers, France.
Dubas
F
F
Delestre
F
F
Emile
J
J
D004576:Electromyography; D004594:Electrophysiology; D005073:Evoked Potentials, Somatosensory; D008040:Genetic Linkage; D006177:Gynecomastia / Q000150:complications / Q000235:genetics*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009134:Muscular Atrophy, Spinal / Q000150:complications / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D009431:Neural Conduction; D010523:Peripheral Nervous System Diseases / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D014960:X Chromosome
Atteinte du nerf sensitif dans l'amyotrophie bulbo-spinale liée a l'X (syndrome de Kennedy). Apport des données électrophysiologiques et histologiques.