Case with atrophy and proximal muscle weakness: seronegative Lambert Eaton Myasthenic Syndrome

Abstract Lambert Eaton Myasthenic Syndrome (LEMS) is a rare neuromuscular junction disease characterized by proximal muscle weakness, decrease or loss of reflexes, moderate ocular and autonomic findings with antibody development to presynaptic voltage-dependent calcium channels. It can be paraneoplastic or autoimmun. Paraneoplastic LEMS is most commonly associated with small-cell lung cancer. Primary autoimmune may be associated with diabetes, rheumatoid arthritis, systemic lupus erythematosus, autoimmune thyroid diseases. Diagnosis is based clinical findings and electrophsyologic studies include repetetif nerve stimulation or single fiber electromyelography. Treatment includes 3,4 diaminopyridine, intravenous immunglobulin, plasmapheresis, prednisolone and azothiopurine according to the case