r-n-jРоссийский неврологический журналRussian neurological journal2658-79472686-7192МИА10.30629/2658-7947-2022-27-4-44-54r-n-j-335Research ArticleИССЛЕДОВАНИЯ И КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCLINICAL RESEARCHES AND CASE REPORTSАнти-MuSK-миастения как осложнение аллогенной трансплантацииAnti-MuSK myasthenia after allogeneic hematopoietic stem cell transplantation in acute myeloid leukemiahttps://orcid.org/0000-0002-3804-6245БардаковС. Н.BardakovS. N.

Санкт-Петербург

St. Petersburg

epistaxis@mail.ruhttps://orcid.org/0000-0001-8699-2482ПолушинА. Ю.PolushinA. Yu.

Полушин Алексей Юрьевич

Санкт-Петербург

Alexey Yu. Polushin

St. Petersburg

alexpolushin@yandex.ruhttps://orcid.org/0000-0002-5459-986XЦаргушВ. А.TsargushV. A.

Санкт-Петербург

St. Petersburg

tsargushvmf@mail.ruКарлиерП.CarlierP.

Орсе

Orsay

pierre.carlier@cea.frhttps://orcid.org/0000-0001-5723-7058ЕмельянцевА. А.EmeliantsevA. A.

Санкт-Петербург

St. Petersburg

igzh@bk.ruhttps://orcid.org/0000-0002-4998-3699ЛапинС. В.LapinS. V.

Санкт-Петербург

St. Petersburg

svlapin@mail.ruhttps://orcid.org/0000-0001-5379-2863МошниковаА. Н.MoshnikovaA. N.

Санкт-Петербург

St. Petersburg

moshnikova-anna@mail.ruhttps://orcid.org/0000-0003-1955-1032СкибаЯ. Б.SkibaIa. B.

Санкт-Петербург

St. Petersburg

yaver-99@mail.ruБельскихА. Н.BelskikhA. N.

Санкт-Петербург

St. Petersburg

belskihAN@mail.ruhttps://orcid.org/0000-0002-9589-4136КулагинА. Д.KulaginA. D.

Санкт-Петербург

St. Petersburg

kulagingem@rambler.ruФГБОУ ВО «Военно-медицинская академия им. С.М. Кирова» Министерства обороны Российской ФедерацииРоссияS.M. Kirov Military medical Academy of the Ministry of defense of the Russian FederationRussian FederationФГБОУ ВО «Первый Санкт-Петербургский государственный медицинский университет имени академика И.П. Павлова» Минздрава РоссииРоссияFirst Pavlov State Medical University of St. PetersburgRussian FederationFrédéric Joliot Institute for Life SciencesФранцияFrédéric Joliot Institute for Life SciencesFrance2022031020222744454Copyright © Бардаков С.Н., Полушин А.Ю., Царгуш В.А., Карлиер П., Емельянцев А.А., Лапин С.В., Мошникова А.Н., Скиба Я.Б., Бельских А.Н., Кулагин А.Д., 20222022Бардаков С.Н., Полушин А.Ю., Царгуш В.А., Карлиер П., Емельянцев А.А., Лапин С.В., Мошникова А.Н., Скиба Я.Б., Бельских А.Н., Кулагин А.Д.Bardakov S.N., Polushin A.Y., Tsargush V.A., Carlier P., Emeliantsev A.A., Lapin S.V., Moshnikova A.N., Skiba I.B., Belskikh A.N., Kulagin A.D.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.r-n-j.com/jour/article/view/335

Анти-MuSK-миастения — это хроническое аутоиммунное заболевание, обусловленное нарушением функции агрин-MuSK-Lrp4-комплекса, сопровождающееся развитием патологической мышечной утомляемости и нередко мышечной атрофии. Заболевание может быть как первичным, так и осложнением иммуносупрессивной терапии и трансплантации гемопоэтических стволовых клеток.Представлен случай анти-MuSK-миастении, развившейся после аллогенной трансплантации гемопоэтических стволовых клеток по поводу острого миелоидного лейкоза у пациентки 44 лет. Особенностью представленного случая являлось генерализованное поражение, включающее не только экстраокулярную, но и паравертебральную мускулатуру, а также dropped head-синдром в вечернее время. Представлены лабораторные, электронейромиографические и визуализационные данные (МРТ мышц, МР-спектроскопия), свидетельствующие о нейрогенном обратимом характере изменений вовлеченной мускулатуры.Результаты. Представлены МРТ- и ЭМГ-признаки нейрогенного поражения аксиальной мускулатуры у пациентки со слабостью паравертебральных мышц в течение 2–4 мес., обусловленной анти-MuSK-миастенией. Клинические проявления, как и отечные нейрогенные изменения паравертебральных мышц регрессировали после проведения патогенетической терапии.Заключение. Рассмотренный клинический случай подтверждает наличие нейрогенных изменений на раннем этапе анти-MuSK-миастении и указывает на необходимость незамедлительного начала патогенетической терапии, позволяющей избежать развития атрофии и жировой инфильтрации мышц.

Anti-MuSK myasthenia is a chronic autoimmune disease caused by violation of the function of the agrin-MuSK-Lrp4 complex, accompanied by the development of pathological muscle fatigue and often muscle atrophy. The disease can be a primary, as well as a complication of immunosuppressive therapy and hematopoietic stem cell transplantation. We present a case of anti-MUSK myasthenia gravis that developed after allogeneic HSCT due to acute myeloid leukemia in a 44-year-old patient (born in 1975). A distinctive feature of the case was a generalized injury, including not only extraocular, but also paravertebral muscles, dropped head syndrome in the evening. In addition, laboratory, electroneuromyographic and neuroimaging (MRI of muscles, MR-spectroscopy) data indicating the neurogenic reversible nature of changes in the involved musculature are presented.Results. This study presents MRI and EMG signs of neurogenic damage of axial musculature in a patient with weakness and swelling of the paravertebral muscles (edematous changes according to STIR, corresponding to MR signs of early functional denervation) for a period of 2–4 months, due to anti-MUSK myasthenia gravis. Clinical manifestations, as well as edematous neurogenic changes in the paravertebral muscles, regressed after pathogenetic therapy. Conclusion. The article presents up-to-date data on the clinical and instrumental characteristics of myasthenia gravis. The considered clinical example confirms the presence of neurogenic changes at an early stage of anti-MUSK myasthenia gravis and indicates the need for immediate initiation of pathogenetic therapy to avoid the development of atrophy and fatty infiltration of muscles.

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The authors declare that there are no conflicts of interest present.