Myastenia Gravis Hastalarının Klinik Özellikleri: 138 Hastanın Değerlendirilmesi

Amaç: Bu çalışmanın amacı, Myastenia Gravis (MG) tanısı ile izlenen hastaların demografik özellikleri, ilk başvuru şikayeti ile klinik seyir, antikor ve elektrofizyolojik özelliklerini değerlendirmektir. Gereç ve Yöntem: 2014-2019 yılları arasında, hastanemiz Nöromusküler ve Kas Hastalıkları Polikliniği’nde takip edilen yüz otuz sekiz MG hastasının demografik özellikleri, ilk başvuru şikayeti ile klinik seyri, antikor, elektrofizyolojik ve radyolojik (toraks tomografi) özellikleri incelendi. Bulgular: Hastaların yaş ortalaması 53.8±16.67 idi. Ortalama hastalık süresi 4.8±5.05 yıl idi. Hastalarımızın en sık, ilk başvuru şikayeti %79 (n=109)’unda ptozis idi. Saf oküler MG olarak takip edilen hasta sayısı %61.6 (n=85) kişiydi. İlk başvurusunda ptozis veya diplopi şikayetiyle gelip, daha sonrasında bulber bulgular ve güçsüzlük eklenerek jeneralize MG düşünülen 26 hasta bulunuyordu. Hastaların %78.3 (n=108)’ünde asetil kolin reseptör antikoru (AntiAChRAb) pozitifliği ve %37.7 (n=52)’sinde toraks tomografide timüs patolojisi bulundu. Sonuç: İlk başvuru şikayeti hastalığın seyri hakkında çok az fikir vermektedir. Asıl önemli olan, klinik yakın takiptir. Her ne kadar MG tedavisi bilinse de MG’in patofizyolojisi halen tam olarak aydınlatılamamıştır ve diğer otoimmun durumlar tedavi seçiminde değişikliğe neden olmaktadır. MG patofizyolojisinin aydınlatılması, daha etkin tanı ve tedavi yöntemlerinin geliştirilmesini sağlayacaktır.

Anahtar Kelimeler:

asetilkolin reseptör antikoru, Myastenia Gravis, pridostigmin, timoma

Clinical Features of Myasthenia Gravis Patients’: The assessment of 138 Patients

Aim: The aim of this study was to evaluate the demographic characteristics, clinical presentation, antibody, and electrophysiological characteristics of patients with the diagnosis of Myasthenia Gravis (MG). Materials and Methods: The demographic characteristics, clinical presentation, antibody, electrophysiological and radiological (thoracic tomography) characteristics of the patients who were followed in the Neuromuscular and Muscular Diseases Polyclinic of the hospital between 2014-2019 were analyzed. Results: The mean age of the patients was 53.8±16.67 years. The mean disease duration was 4.8±5.05 years. Ptosis was the most common complaint in 79% (n=109) of our patients. The number of patients followed as pure ocular MG was 61.6% (n=85). There were 26 patients who presented with ptosis or diplopia at the first presentation and follow-up generalized MG with bulbar findings and weakness. Acetylcholine receptor antibody (AntiAChRAb) positivity was found in 78.3% (n=108) of the patients and thymus pathology was detected in thorax tomography in 37.7% (n=52). Conclusions: The initial complaint provides little insight into the course of the disease. The important factor is clinical follow-up. Although MG treatment is known, the pathophysiology of MG remains to be elucidated, and other autoimmune conditions cause changes in treatment choice. Clarifying the pathophysiology of MG will enable the development of more effective diagnostic and treatment methods.

Keywords:

acetylcholine receptor antibody, Myasthenia Gravis, pyridostigmine, thymoma,

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