neurojourНеврологический журнал имени Л.О. БадалянаL.O. Badalyan Neurological Journal2686-89972712-794XФГАУ «НМИЦ здоровья детей» Минздрава России10.46563/2686-8997-2021-2-3-137-145neurojour-48Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSАнти-NMDA-рецепторный энцефалитAnti-NMDA receptor encephalitishttps://orcid.org/0000-0001-9147-8904АзизоваУмида МаликовнаAzizovaUmida M.

Аспирант кафедры неврологии, нейрохирургии и медицинской генетики им. академика Л.О. Бадаляна педиатрического факультета ФГАОУ ВО РНИМУ им. Н.И. Пирогова Минздрава России, 119571, Москва.

e-mail: azizova_umida91@mail.ru 

MD, postgraduate student of the Department of Neurology, neurosurgery and medical genetics named after V.I. academician L.O. Badalyan, Faculty of Paediatrics of the Pirogov Russian National Research Medical University, Moscow, 117997, Russian Federation.

e-mail: azizova_umida91@mail.ru

azizova_umida91@mail.ruhttps://orcid.org/0000-0002-4373-4747БембееваР. Ц.BembeevaRaisa Ts.noemail@neicon.ruhttps://orcid.org/0000-0003-2176-1557КозыреваА. А.KozyrevaAnastasia A.noemail@neicon.ruhttps://orcid.org/0000-0003-0103-7422ЗаваденкоН. Н.ZavadenkoNikolay N.noemail@neicon.ruФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава РоссииРоссияPirogov Russian National Research Medical UniversityRussian Federation20212909202123137145Copyright © Азизова У.М., Бембеева Р.Ц., Козырева А.А., Заваденко Н.Н., 20212021Азизова У.М., Бембеева Р.Ц., Козырева А.А., Заваденко Н.Н.Azizova U.M., Bembeeva R.T., Kozyreva A.A., Zavadenko N.N.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.neuro-journal.ru/jour/article/view/48

Аутоиммунные заболевания центральной нервной системы являются одной из наиболее социально и экономически значимых проблем неврологии. Несмотря на выделение новых нозологических форм аутоиммунных энцефалитов, создание диагностических панелей для верификации аутоантител в биологических жидкостях, применение высокоэффективной патогенетической терапии, число диагностических ошибок остается высоким, что создаёт угрозу жизни пациента и высокий риск развития тяжёлых осложнений. Анти-N-метил-D-аспартат-рецепторный энцефалит (АЭ) - это аутоиммунный энцефалит, обусловленный наличием антител к NR1-субъединице NMDA-рецепторов (NMDAR) и характеризующийся развитием тяжёлого психического и неврологического дефицита у ранее здорового человека. Представленная статья суммирует последние литературные данные об АЭ. Поиск литературы проводился по базам данных Scopus, Web of Science, PubMed, CyberLeninka. В обзоре представлены факты истории изучения заболевания, эпидемиологические данные, современные представления о патогенетических механизмах развития заболевания, спектр клинических проявлений и различные формы течения болезни. Описаны диагностические критерии и методы исследования, применяемые для подтверждения диагноза, кратко изложены подходы к лечению АЭ.

АЭ клинически проявляется сочетанием психических нарушений, эпилептических приступов, речевых и экстрапирамидных расстройств, нарушением ритма сна и бодрствования. Заболевание встречается в любом возрасте. Развитие болезни может быть связано с такими иммунологическими триггерами, как онкологический процесс и герпетический энцефалит, либо носить идиопатический характер. Имеются особенности течения клинической картины в зависимости от возраста пациента, паранеопластической или постгерпетической этиологии заболевания. Диагностический алгоритм наряду с нейровизуализацией, определением специфических антител, электроэнцефалографией, должен включать поиск онкологического процесса. Выздоровление пациентов может длиться от нескольких месяцев до нескольких лет. В ряде случаев развивается стойкий неврологический дефицит. К предикторам благоприятного исхода относят раннее начало и применение комбинированной терапии, выявление и удаление новообразования, низкий титр антител к NMDAR и возраст пациентов старше 12 лет. До 25% случаев возможно ремиттирующее течение заболевания, в связи с чем требуется длительное наблюдение за данными пациентами.

Autoimmune diseases of the central nervous system (CNS) are one of the most socially and economically significant problems of neurology. Despite the identification of new nosological forms of autoimmune encephalitis, the creation of diagnostic panels for the verification of autoantibodies in biological fluids, and the use of highly effective pathogenetic therapy, the number of diagnostic errors remains high, which poses a threat to the patient’s life and a high risk of developing severe complications. Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDAR encephalitis) is autoimmune encephalitis caused by the presence of antibodies (Ab) to the NR1 subunit of NMDA-receptors (NMDAR) characterized by the development of severe mental and neurological deficits in a previously healthy person. This article summarizes the recent literature on anti-NMDAR encephalitis. The literature search was carried out using the Scopus, Web of Science, Pubmed, CyberLeninka databases. The review presents the facts of the history of the study of the disease, epidemiological data, modern ideas about the pathogenetic mechanisms of the development of the disease, the spectrum of clinical manifestations and various forms of the course of the disease. The diagnostic criteria and research methods used to confirm the diagnosis are described, approaches to the treatment of anti-NMDAR encephalitis are outlined.

Anti-NMDAR encephalitis is clinically manifested by a combination of mental disorders, epileptic seizures, speech and extrapyramidal disorders, and disturbances in the rhythm of sleep and wakefulness. The disease occurs at any age. The development of the disease can be associated with such immunological triggers as oncological process and herpetic encephalitis, or be idiopathic in nature. There are features of the course of the clinical picture depending on the age of the patient, paraneoplastic or postherpetic aetiology of the disease. The diagnostic algorithm, along with neuroimaging, determination of specific antibodies, electroencephalography, should also include the search for an oncological process. The recovery of patients can take from several months to years. In some cases, persistent neurological deficits develop. Predictors of a favourable outcome include early initiation and use of combination therapy, detection and removal of neoplasms, a low titer of anti-NMDAR antibodies, and age of patients over 12 years of age. In up to 25% of cases, a relapsing course of the disease is possible, and therefore requires long-term monitoring of these patients.

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