Atrofi ve proksimal kas güçsüzlüğü olan olgu: seronegatif Lambert Eaton Myastenik Sendrom

Lambert Eaton Myastenik Sendrom (LEMS) proksimal kas güçsüzlüğü, reflekslerde azalma veya kayıp, ılımlı oküler bulgular ve otonom bulgular ile seyreden presinaptik voltaj bağımlı kalsiyum kanallarına antikor gelişimi ile karekterize nadir görülen nöromuskuler kavşak hastalığıdır. Paraneoplastik veya otoimmün olabilir. Paraneoplastik LEMS en sık küçük hücreli akciğer kanseri ile birliktelik gösterir. Primer otoimmun olarak ise diyabet, romatoid artrit, sistemik lupus eritematozus, otoimmun tiroid hastalıklarıyla birliktelik gösterebilir. Tanı klinik bulgular ve elektrofizyolojik olarak ardışık sinir uyarım testi veya tek lif elektromyelografi ile konur. Tedavisinde olguya göre 3,4 diaminopiridin, ıntravenöz immunglobulin, plazmaferez, prednizolon ve azotiopürin yer alır.

Anahtar Kelimeler:

proksimal kas güçsüzlüğü, otonom bulgular, Lambert Eaton Myastenik Sendromu

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.

Keywords:

proximal muscle weakness, autonomic findings, Lambert Eaton Myasthenic Syndrome,

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