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

Bahar SAY; Ufuk ERGÜN; Gülten KARACA

Article Details

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

Authors:

Bahar SAY | Ufuk ERGÜN | Gülten KARACA

Publisher:

Pamukkale University

Main Topics:

Diseases

Journal:

Pamukkale Medical Journal 2019 Vol.12 Issue 1, pp.181-183

Document Type:

Article

Publication Date:

1/2019

Document Language:

Turkish

Document ID (Org No.):

2000570195

Frequency:

Quarterly

eISSN:

1308-0865

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.




.

 


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. 

.

Article Details

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

Authors: Bahar SAY | Ufuk ERGÜN | Gülten KARACA

Publisher: Pamukkale University

Main Topics: Diseases

Journal: Pamukkale Medical Journal 2019 Vol.12 Issue 1, pp.181-183

Document Type: Article

Publication Date: 1/2019

Document Language: Turkish

Document ID (Org No.): 2000570195

Frequency: Quarterly

eISSN: 1308-0865

Issues

Authors:

Bahar SAY | Ufuk ERGÜN | Gülten KARACA

Publisher:

Pamukkale University

Main Topics:

Diseases

Journal:

Pamukkale Medical Journal 2019 Vol.12 Issue 1, pp.181-183

Document Type:

Article

Publication Date:

1/2019

Document Language:

Turkish

Document ID (Org No.):

2000570195

Frequency:

Quarterly

eISSN:

1308-0865