Lambert-Eaton myasthenic syndrome. Clinical and electrophysiological findings in seven cases.
We report the clinical and electrophysiological findings in seven patients with Lambert-Eaton myasthenic syndrome (LEMS). All patients were males aged 40-73 years old. Six presented proximal muscle weakness and one both proximal and distal. The tendon reflexes were absent in four patients, depressed in two and normal in one patient. Three patients presented ophthalmic and four autonomic symptoms. The syndrome was diagnosed 3-12 months after the onset of symptoms in six patients and 4 years later in one. Acetylcholine receptor antibodies were negative in all patients. Voltage-gated calcium channel antibodies (VGCC) were measured in five patients and were positive in four. All patients had low compound muscle action potential (CMAP) at rest, a decrement in CMAP amplitude of 20-47% at 3 Hz repetitive nerve stimulation, and an increment of 200-700% at 40 Hz. In three patients the syndrome was associated with histologically verified small-cell lung cancer (SCLC). In the younger patient (40 years old), a lymph node biopsy performed nine years before the diagnosis of LEMS, had shown an atypical microcellular cancer of undetermined origin, which was treated with chemotherapy. LEMS 9 years after the diagnosis of cancer has not been described previously. The fifth patient had a two years history of bladder cancer (grade II). Three years after the diagnosis of LEMS he presented chronic lymphogenic leukemia. No malignancy was found in the remaining 2 patients.
Zambelis
Th
T
Department of Neurology, University of Athens, Aeginition Hospital, Athens, Greece. tzabelis@med.uoa.gr
Foutsitzi
A
A
Giannakopoulou
A
A
Poulopoulou
K
K
Karandreas
N
N
D000200:Action Potentials / Q000502:physiology*; D000328:Adult; D000368:Aged; D015220:Calcium Channels / Q000097:blood; D018288:Carcinoma, Small Cell / Q000150:complications; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000150:complications* / Q000276:immunology / Q000503:physiopathology*; D007945:Leukemia, Lymphoid / Q000150:complications; D008175:Lung Neoplasms / Q000150:complications; D008297:Male; D008875:Middle Aged; D009119:Muscle Contraction / Q000502:physiology; D018482:Muscle, Skeletal / Q000503:physiopathology*; D001749:Urinary Bladder Neoplasms / Q000150:complications
D015220:Calcium Channels