r-n-jРоссийский неврологический журналRussian neurological journal2658-79472686-7192МИА10.30629/2658-7947-2023-28-4-43-53r-n-j-454Research ArticleКЛИНИЧЕСКИЕ ИССЛЕДОВАНИЯ И НАБЛЮДЕНИЯCLINICAL RESEARCH AND SURVEILLANCEПаранеопластическая мозжечковая дегенерацияParaneoplastic cerebellar degenerationНужныйЕ. П.NuzhnyiE. P.

  Нужный Евгений Петрович

Москва

Moscow

enuzhny@mail.ruhttps://orcid.org/0000-0002-5321-3451КрасновМ. Ю.KrasnovM. Yu.

 Москва

 Moscow

https://orcid.org/0000-0003-3843-6435МоскаленкоА. Н.MoskalenkoA. N.

 Москва

Moscow

https://orcid.org/0000-0001-8070-7644ФедотоваЕ. Ю.FedotovaE. Yu.

 Москва

Moscow

https://orcid.org/0000-0001-5442-0877ЧекановаЕ. О.ChekanovaE. O.

Москва

 Moscow

https://orcid.org/0000-0002-2704-6282ИллариошкинС. Н.IllarioshkinS. N.

 Москва

 Moscow

Научный центр неврологии РАНРоссияResearch Center of NeurologyRussian Federation2023021020232844353Copyright © Нужный Е.П., Краснов М.Ю., Москаленко А.Н., Федотова Е.Ю., Чеканова Е.О., Иллариошкин С.Н., 20232023Нужный Е.П., Краснов М.Ю., Москаленко А.Н., Федотова Е.Ю., Чеканова Е.О., Иллариошкин С.Н.Nuzhnyi E.P., Krasnov M.Y., Moskalenko A.N., Fedotova E.Y., Chekanova E.O., Illarioshkin S.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/454Введение

Введение. Паранеопластическая мозжечковая дегенерация (ПМД) — иммуноопосредованный быстропрогрессирующий мозжечковый синдром, развивающийся в результате перекрестного иммунного ответа на общие для опухоли и клеток мозжечка антигены. Своевременная диагностика и лечение ПМД способствует улучшению функционального статуса и выживаемости данных пациентов.

Цель работы — описание клинико-лабораторных и нейровизуализационных характеристик серии случаев пациентов с ПМДв сопоставлении с данными литературы.

Материал и методы

Материал и методы. Обследованы 16 пациентов с ПМД (13 женщин, 3 мужчины). Проведены оценка клинической картины, МРТ головного мозга, лабораторные исследования крови и цереброспинальной жидкости, проанализированы данные онкопоиска и катамнез пациентов.

Результаты

Результаты. Медиана возраста пациентов составила 55 лет, длительность заболевания — 8,5 мес. (от 4 до 16 мес.). У 12 пациентов ПМД являлась первым проявлением онкологического заболевания. Клиническая картина была представлена быстропрогрессирующей мозжечковой атаксией, часто в сочетании с глазодвигательными нарушениями, пирамидным и бульбарным синдромами, тремором рук и фокальной мышечной дистонией.

Ассоциированная опухоль выявлена 13 больных (81%). Антинейрональные антитела обнаружены у 14 пациентов (88%): анти-Yo-1, антитела к амфифизину, анти-Hu, анти-CV2 и анти-GAD. Легкие атрофические изменения мозжечка по данным МРТобнаружены у 6 пациентов, в 2 случаях — атрофия одного из полушарий мозжечка.

Заключение

Заключение. ПМД является редким инвалидизирующим, но потенциально курабельным заболеванием. Основу диагностики составляет анализ клинической картины заболевания и даннных нейровизуализации, выявление антинейрональных антител и воспалительных изменений в цереброспинальной жидкости, а также проведение тщательного онкопоиска.

Introduction. Paraneoplastic cerebellar degeneration (PCD) is an immune-mediated and rapidly progressive cerebellar syndrome that develops as a result of a cross-immune response to the common antigens for the tumor and cerebellar cells. Timely diagnosis and treatment of PCD improves the functional status and survival of these patients.Objective. To analyze the clinical, laboratory and neuroimaging characteristics of PCD case series in comparison with literature data.Material and methods. 16 patients with PCD (13 women, 3 men) were examined. An assessment of the clinical presentation, brain MRI study, blood and cerebrospinal fl uid laboratory tests were carried out, the data of cancer search and patients follow-up were analyzed.Results. The median age of PCD patients was 55 years, the duration of the disease was 8.5 months (range 4 to 16 months). In 12 patients, PCD was the fi rst manifestation of cancer. The clinical prentation was presented by rapidly progressive cerebellar ataxia, often in combination with oculomotor disturbances, pyramidal and bulbar syndrome, hand tremor and dystonia. An associated cancers were detected in 13 patients (81%). Antineuronal antibodies were found in 14 patients (88%): anti-Yo-1, antibodies to amphiphysin, anti-Hu, anti-CV2 and anti-GAD. Mild atrophic changes of the cerebellum were found in 6 patients, and in 2 cases cerebellar hemiatrophy was observed.Conclusion. PCD is a rare disabling but potentially curable disease. The basis of diagnosis is the analysis of the clinical presentation and neuroimaging data, the detection of antineuronal antibodies and in fl ammatory changes in the cerebrospinal fl uid, as well as a thorough cancer search.

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