Neuromuscular DiseasesNeuromuscular DiseasesНервно-мышечные болезни2222-87212413-0443Publishing House ABV Press9910.17650/2222-8721-2012-0-4-53-58CLINICAL CASEКЛИНИЧЕСКИЙ РАЗБОР2 cases transformation myasthenia gravis to amyotrophic lateral sclerosisДва случая трансформации миастении в боковой амиотрофический склерозSanadzeA. G.СанадзеА. Г.
Russian Federation
ag-sanadze@mail.ruKasatkinaL. F.КасаткинаЛ. Ф.
Russian Federation
Moscow Myasthenia CenterМосковский миастенический центр211120122453582102201521022015Copyright ©; 2012, Sanadze A.G., Kasatkina L.F.Copyright ©; 2012, Санадзе А.Г., Касаткина Л.Ф.2012Sanadze A.G., Kasatkina L.F.Санадзе А.Г., Касаткина Л.Ф.https://creativecommons.org/licenses/by/4.0https://nmb.abvpress.ru/jour/article/view/99

A72-year old woman and 38-year old man had clinical sings of myasthenia gravis with ptosis and double vision, speech, swallowing, face and limb weakness. They was diagnosed MG after tensilon test, responding to piridostigmine and prednisolon treatment, neuromusculardisorders and positive anti-AchR and titin antibodies. Four and 7 year ago after debut of myasthenia gravis they had clinical and electrophysiological symptoms of amyotrophic lateral sclerosis.

Описаны 2 случая трансформации миастении в боковой амиотрофический склероз (БАС). У 72-летней женщины и 38-летнего мужчины выявлены достоверные клинические признаки генерализованной миастении, с наличием глазодвигательных расстройств и птоза, нарушением бульбарных функций, слабостью мышц лица и конечностей. Диагноз миастении подтверждался результатами фармакологического теста, электромиографического (ЭМГ) тестирования нервно-мышечной передачи, повышением концентрации аутоантител к ацетилхолиновому рецептору, титин-протеину, а также эффектом глюкокортикоидной терапии и тимэктомии. У обоих пациентов в интервале от 4 до 7 лет от начала болезни появились ЭМГ- и клинические симптомы БАС.

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