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Yıl:2021; Cilt: 58; Sayı: 2>> Özet
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Nöropsikiyatri Arşivi; 2021;58(2):154-162
Miyastenia Gravis Tarihçesine Yeniden Bir Bakış
F Deymeer
İstanbul Üniversitesi, İstanbul
Miyastenia gravis’i (MG) ilk olarak Thomas Willis’in 1672 yılında tarif ettiği düşünülür. MG’ye esas ilgi 19. yüzyılın ortalarından sonra başlamış ve 150 yıllık sürede MG hakkında geniş bilgi edinilmiştir. Bu derlemenin amacı, MG tarihçesini belli bir sistematik içinde toparlamak ve İstanbul Üniversitesi deneyimini kısaca aktarmaktır. MG tarihçesi dört döneme ayırarak incelenmiştir: 1868–1930, 1930–1960, 1960–1990 ve 1990–2020. İlk dönemde (1868–1930), MG’nin bütün klinik özellikleri tanımlanmıştır. Sinir-kas arasındaki iletimin fizyolojik/ farmakolojik özellikleri anlaşılmaya başlanmış, ardışık sinir uyarımının temelleri atılmıştır. Kürar zehirlenmesine benzetilen MG’ye toksik bir ajanın neden olduğu düşüncesi hakim olmuş, MG ile timus ilişkisi dikkati çekmeye başlanmıştır. Tedavi ile ilgili kayda değer bir gelişme olmamıştır. İkinci dönemde (1930–1960), nöromüsküler kavşakta iletimin asetilkolin aracılığıyla olduğu anlaşılmıştır. Ardışık sinir uyarımı MG tanısında kullanılmaya başlanmıştır. MG’nin otoimmun bir hastalık olabileceği fikri doğmuş, bu amaçla yapılan deneyler sonuç vermeye başlamıştır. Bu döneme damgasını vuran antikolinesterazların ve timektominin tedavideki yerini almasıdır. Üçüncü dönemi (1960–1990) MG açısından devrimsel bir dönem olarak kabul etmek mümkündür. Önemli immunolojik mekanizmalar aydınlatılmış (asetilkolin reseptör izolasyonu, anti-asetilkolin reseptör antikoru bulunması) ve MG’nin otoimmun bir hastalık olduğu gösterilmiştir. Tedavinin çehresini tümüyle değiştiren pozitif basınçlı mekanik ventilasyon ve kortikosteroidlerin yanı sıra plazma değişimi/İVİg ve azatioprin kullanıma girmiştir. Dördüncü dönemde (1990–2020), anti-MuSK antikoru bulunmuş ve immunolojide yeni gelişmeler kaydedilmiştir. Videotorakoskopik yöntemle yapılan timektomi, ameliyatla ilişkili morbidite ve mortalitenin azalmasını sağlamıştır. Yeni ilaçlar kullanıma girmiş ve klinik çalışmalar yapılmaya başlanmıştır. Değerli kılavuzlar hazırlanmıştır. Derlemenin son bölümünde, MG konusunda Türkiye’de öncü olan İstanbul Üniversitesi’nin deneyimi anlatılmıştır.
History of Myasthenia Gravis Revisited
The first description of myasthenia gravis (MG) was given by Thomas Willis in 1672. MG was the focus of attention after mid-nineteenth century and a great amount of information has been accumulated in a span of 150 years. The aim of this review is to convey this information according to a particular systematic and to briefly relate the experience of Istanbul University. MG history was examined in four periods: 1868- 1930, 1930-1960, 1960-1990, and 1990-2020. In the first period (1868- 1930), all the clinical characteristics of MG were defined. Physiological / pharmacological studies on the transmission at the neuromuscular junction were initiated, and the concept of repetitive nerve stimulation emerged. A toxic agent was believed to be the cause of MG which appeared to resemble curare intoxication. Association of MG with thymus was noticed. No noteworthy progress was made in its treatment. In the second period (1930-1960), acetylcholine was discovered to be the transmitter at the neuromuscular junction. Repetitive nerve stimulation was used as a diagnostic test. The autoimmune nature of MG was suspected and experiments to this end started to give results. The hallmark of this period was the use of anticholinesterases and thymectomy in the treatment of MG. The third period (1960-1990) can probably be considered a revolutionary era for MG. Important immunological mechanisms (acetylcholine receptor isolation, discovery of anti-acetylcholine receptor antibodies) were clarified and the autoimmune nature of MG was demonstrated. Treatment modalities which completely changed the prognosis of MG, including positive pressure mechanic ventilation and corticosteroids as well as plasma exchange/IVIg and azathioprine, were put to use. In the fourth period (1990-2020), more immunological progress, including the discovery of anti-MuSK antibodies, was achieved. Videothoracoscopic thymectomy reduced the morbidity and mortality rate associated with surgery. New drugs emerged and clinical trials were performed. Valuable guidelines were published. In the last part of the review, the experience in MG of Istanbul University, a pioneer in Turkey, is related.

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