r-n-jРоссийский неврологический журналRussian neurological journal2658-79472686-7192МИА10.30629/2658-7947-2024-29-1-14-18r-n-j-518Research ArticleОБЗОРЫREVIEWSОстрый рассеянный энцефаломиелит как неотложное состояниеAcute disseminated encephalomyelitis as an emergencyhttps://orcid.org/0000-0003-2528-716XЕвтушенкоС. К.YevtushenkoS. K.

Донецк

Donetsk

centerkramatorsk@gmail.comhttps://orcid.org/0000-0002-9185-4073СорокинЮ. Н.SorokinYu. N.

Ростов-на-Дону

Rostov-on-Don

sorokin.yuri@bk.ruДонецкий национальный медицинский университет им. М. ГорькогоРоссияThe Donetsk National Medical UniversityRussian FederationРостовский государственный медицинский университетРоссияThe Rostov State Medical UniversityRussian Federation2024110320242911418Copyright © Евтушенко С.К., Сорокин Ю.Н., 20242024Евтушенко С.К., Сорокин Ю.Н.Yevtushenko S.K., Sorokin Y.N.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/518

Острый рассеянный энцефаломиелит (ОРЭМ) — часто встречающееся острое многоочаговое аутоиммунное воспалительное демиелинизирующее заболевание центральной нервной системы (ЦНС) с преимущественным поражением белого вещества головного мозга, ствола мозга и зрительных нервов, проводников спинного мозга и, реже, серого вещества ЦНС. ОРЭМ обычно является самокупирующимся состоянием со спонтанным улучшением и благоприятным прогнозом. Наряду с возможностью тяжелого течения при классической форме ОРЭМ может возникать развитие сверхострых форм с гораздо более неблагоприятным прогнозом. Такими вариантами течения ОРЭМ являются острый геморрагический лейкоэнцефалит (ОГЛЭ), острый геморрагический лейкоэнцефаломиелит и острый некротизирующий геморрагический лейкоэнцефалит Херста, вызываемые развитием каскада сверхострых неконтролируемых аутоиммунных реакций. Тяжесть этих состояний (объединямых под термином ОГЛЭ) связана с быстрым прогрессированием, вовлечением ствола мозга, большим размером очагов поражения и обусловлена развитием острого отечного некроза и кровоизлияний с высокой вероятностью инвалидизации или смерти. Агрессивное и тяжелое течение ОГЛЭ требует клинической настороженности и незамедлительного начала противоотечной и иммуносупрессивной терапии.

Acute disseminated encephalomyelitis (ADEM) is a common acute multifocal autoimmune infl ammatory demyelinating disease of the central nervous system (CNS) with a predominant lesion of the white matter of the brain, brainstem and optic nerves, the conductors of the spinal cord, less often — gray matter of the CNS. ADEM is usually a self-limiting condition with spontaneous improvement and a favorable prognosis. Along with the possibility of a severe course in the classical form of ADEM, the development of hyperacute forms with a much more unfavorable prognosis is possible. Such variants of the course of ADEM are acute hemorrhagic leukoencephalitis (AHLE), acute hemorrhagic leukoencephalomyelitis and acute necrotizing hemorrhagic leukoencephalitis of Hurst, associated with the development of a cascade of hyperacute uncontrolled autoimmune reactions. The severity of these conditions (grouped under the term OHLE) is associated with rapid progression, brainstem involvement, large lesions is due to the development of acute edematous necrosis and hemorrhage with a high possibility of disability or death. The aggressive and severe course of АHLE requires the immediate initiation of decongestant and immunosuppressive therapy.

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