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

Abstract

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.

Keywords

• proksimal kas güçsüzlüğü,otonom bulgular,Lambert Eaton Myastenik Sendromu

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

Abstract

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

References

1. Referans1. Schoser B, Eymard B, Datt J, Mantegazza R. Lambert-Eaton myasthenic syndrome (LEMS): a rare autoimmune presynaptic disorder often associated with cancer. J Neurol 2017;264(9):1854-1863.
2. Referans2. Eaton LM, Lambert HT. Electromyography and electrical stimulation of nerves in disease of the motor ünit: observations on a myastenic syndrome associated with malignant tumours. J Amm Med Assoc 1957;163:117-1124.
3. Referans3. Kesner VG, Oh SJ, Dimachkie MM, Barohn RJ. Lambert eaton myasthenic syndrome. Neurol Clin 2018;36: 379–394.
4. Referans4. Crone C, Christiansen I, Vissing J. Myopathic EMG findings and type II muscle fiber atrophy in patients with Lambert-Eaton myasthenic syndrome. Clin Neurophysiol 2013;124(9):1889-1892.
5. Referans5. Komatsu T, Bokuda K, Shimizu T, Komori T, Koide R. Pseudomyopathic changes in needle electromyography in lambert-eaton myasthenic syndrome. Case Rep Neurol Med 2013;369278.
6. Referans6. Gökçal E, Gürsoy AE, Asil T, Ertaş M. Lambert-Eaton Myasthenic Syndrome with a twenty-three-year delay in diagnosis. Noro Psikiyatr Ars 2017;54(2):189-190.
7. Referans7. Oh SJ, Hatanaka Y,Claussen GC, Sher E. Electrophysiological differences in seropositive and seronegative Lambert-Eaton myasthenic syndrome. Muscle Nerve 2007;35(2):178-183.
8. Referans8. Zalewski NL, Lennon VA, Lachance DH, Klein CJ, Pittock SJ, Mckeon A. P/Q- and N-type calcium-channel antibodies: oncological, neurological, and serological accompaniments. Muscle Nerve 2016;54(2):220-227.