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

Yıl 2019, , 181 - 183, 18.01.2019

Bahar Say , Ufuk Ergün , Gülten Karaca

https://doi.org/10.31362/patd.453646

Öz

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

Öz

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.

Anahtar Kelimeler

proximal muscle weakness, autonomic findings, Lambert Eaton Myasthenic Syndrome

Kaynakça

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