[Lambert-Eaton myasthenic syndrome. Physiopathological aspects and therapeutic modalities].
A 68-year old man experienced a progressive proximal tetraparesis with anhidrosis and a single episode of horizontal diplopia before presenting exertional dyspnea; pulmonary investigations revealed a small cell carcinoma of the lung. Clinical and electrophysiological investigations with abnormal SFEMG, repetitive stimulations and autonomic assessment pointed to a pre-synaptic neuromuscular dysfunction compatible with a Lambert-Eaton syndrome. Antibodies to acetylcholine receptors and calcium channels were negative. Thoracic radiotherapy combined with chemotherapy produced marked improvement: repeated electrophysiological evaluations showed a strong correlation between median nerve CMAP amplitude and clinical course. This case prompted us to discuss current concepts of pre-synaptic dysfunction, and paraneoplastic syndrome, and to review therapeutic strategies, in the light of recent studies of Lambert-Eaton syndrome.
Kuntzer
T
T
Service de neurologie, Centre Hospitalier Universitaire Vaudois, Lausanne, Suisse.
Steck
A J
AJ
Fiorini
E
E
Mirimanoff
R O
RO
Regli
F
F
D000368:Aged; D018288:Carcinoma, Small Cell / Q000150:complications; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology*; D008175:Lung Neoplasms / Q000150:complications; D008297:Male
Syndrome myasthénique de Lambert-Eaton. Aspects physiopathologiques et modalités thérapeutiques.