Téma: Original Works

Lambert-eaton myasthenic syndrome – pathogenesis, diagnosis and treatment

Peter Špalek

Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune neuromuscular junction disease caused by autoantibodies against presynaptic voltage-gated P/Q calcium channels (VGCCs). This decreases calcium entry into the presynaptic terminal, which prevents binding of vesicles to presynaptic membrane and inhibits acetylcholine release. LEMS occurs in 60 % patients as a paraneoplastic disorder most often associated with small cell lung cancer (SCLC). Idiopathic nontumour LEMS presents 40 % of the cases. The most common initial complaint is proximal muscle weakness involving the lower extremities more than the upper extremities. Absent or depressed tendon reflexes and autonomic dysfunction are frequently present. Involvement of bulbar and respiratory muscles is rare. Diagnosis is confirmed by electrophysiological testing, which demonstrates a marked (greater than 100 %) increment in compound motor action potential amplitude with 30 Hz repetitive stimulation, and by serological test for VGCCs autoantibodies. In paraneoplastic LEMS is essential the treatment of underlying cancer. In idiopathic nontumour LEMS prednisone and immunosuppressant drugs have a key role in the treatment.

Ročník 2009  Témy časopisu Neurology 3 / 2009

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