vspВопросы современной педиатрииCurrent Pediatrics1682-55271682-5535Издательство «ПедиатрЪ»10.15690/vsp.v24i1.2848vsp-3680Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWСиндром опсоклонус-миоклонус у детей: современные данныеOpsoclonus-Myoclonus Syndrome in Children: Modern Datahttps://orcid.org/0000-0003-3692-5673ФедосееваИ. Ф.FedoseevaIrina F.

Федосеева Ирина Фаисовна, кандидат медицинских наук, доцент, доцент кафедры неврологии, нейрохирургии, медицинской генетики и медицинской реабилитации 

650029, Кемерово, ул. Ворошилова, д. 22а  

тел.: +7 (3842) 39-64-30

Kemerovo

irenf1@yandex.ruhttps://orcid.org/0009-0008-8805-6784ГончаренкоА. В.GoncharenkoAlexey V.

Кемерово

Kemerovo

https://orcid.org/0000-0003-2894-3062ПопонниковаТ. В.PoponnikovaTatyana V.

Кемерово

Kemerovo

https://orcid.org/0000-0003-0641-0468ГончаренкоВ. А.GoncharenkoVladimir A.

Кемерово

Kemerovo

КемГМУРоссияKemerovo State Medical UniversityRussian Federation2025250220252416–12Copyright © Федосеева И.Ф., Гончаренко А.В., Попонникова Т.В., Гончаренко В.А., 20252025Федосеева И.Ф., Гончаренко А.В., Попонникова Т.В., Гончаренко В.А.Fedoseeva I.F., Goncharenko A.V., Poponnikova T.V., Goncharenko V.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://vsp.spr-journal.ru/jour/article/view/3680

Синдром опсоклонус-миоклонус (энцефалопатия Кинсбурна) — редкое неврологическое расстройство, для которого характерно сочетание аномальных движений глаз (опсоклонус), миоклонуса и мозжечковой атаксии. Синдром опсоклонус-миоклонус развивается преимущественно у детей в результате патологии иммунной системы, сопровождающей инфекционные или онкологические заболевания. При ассоциации синдрома с нейробластомой своевременная диагностика и комплексное лечение определяют прогноз для жизни пациента и восстановления функций нервной системы. Многообразие факторов, запускающих иммунопатологический процесс, определяет необходимость проведения дифференциальной диагностики с большим спектром заболеваний и повышения осведомленности врачебного сообщества об этом редком синдроме. По мере накопления клинических наблюдений, анализа отдаленных последствий и изучения патогенеза совершенствуются терапевтические подходы. Взаимодействие и преемственность между специалистами неврологического, педиатрического, онкологического профилей, а также онкологическая настороженность на всех этапах ведения пациента являются необходимым условием для диагностики и выбора тактики лечения.

Opsoclonus-myoclonus syndrome (Kinsbourne encephalopathy) is a rare neurological disorder characterized by combination of abnormal eye movements (opsoclonus), myoclonus, and cerebellar ataxia. Opsoclonus-myoclonus syndrome develops primarily in children due to immune system pathology associated with infectious or oncological diseases. If this syndrome is comorbid to neuroblastoma, timely diagnosis and complex management determine prognosis for patient’s life and nervous system functions recovery. The variety of factors triggering immunopathological process determines the need for differential diagnosis with wide range of diseases and raising medical community awareness about this rare syndrome. Therapeutic approaches have been being improved due to clinical data accumulation, long-term consequences analysis, and study of pathogenesis. Interaction and succession between neurologists, pediatricians, oncologists, as well as oncological alertness on all stages of patient management are crucial for diagnosis and choosing treatment tactics.

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