Анти-MuSK-миастения как осложнение аллогенной трансплантации

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.

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