ssmuБюллетень сибирской медициныBulletin of Siberian Medicine1682-03631819-3684Siberian State Medical University, the Ministry of Healthcare of the Russian Federation10.20538/1682-0363-2019-4-249-255ssmu-2578Research ArticleСЛУЧАЙ ИЗ КЛИНИЧЕСКОЙ ПРАКТИКИCLINICAL CASESТоксическая энцефалопатия при истинной полицитемии (клинический случай)Toxic encephalopathy in a clinicl case of polycythemia verahttps://orcid.org/0000-0002-4626-6520КузнецоваП. И.KuznetsovaP. I.

канд. мед. наук. науч. сотрудник, 1-е неврологическое отделение,

125367, г. Москва, Волоколамское шоссе, 80

PhD, Researcher, 1st Neurological Department, 

80, Volokolamskoe Shosse, Moscow, 125367

angioneurology0@gmail.comhttps://orcid.org/0000-0002-5883-8119ТанашянМ. М.TanashyanM. M.

д-р мед. наук, профессор, зав. 1-м неврологическим отделением, зам. директора по научной работе, 

125367, г. Москва, Волоколамское шоссе, 80

DM, Professor, Head of the 1st Neurological Department, Deputy Director for Scientific Work,

80, Volokolamskoe Shosse, Moscow, 125367

https://orcid.org/0000-0002-7688-3669КорниловаА. А.KornilovaA. A.

аспирант, 1-е неврологическое отделение,

125367, г. Москва, Волоколамское шоссе, 80

Post-Graduate Student, 1st Neurological Department,

80, Volokolamskoe Shosse, Moscow, 125367

Научный центр неврологии (НЦН)РоссияResearch Center of NeurologyRussian Federation201914012020184249255Copyright © Кузнецова П.И., Танашян М.М., Корнилова А.А., 20202020Кузнецова П.И., Танашян М.М., Корнилова А.А.Kuznetsova P.I., Tanashyan M.M., Kornilova A.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://bulletin.ssmu.ru/jour/article/view/2578

В статье представлено описание случая энцефалопатии смешанного (токсического и сосудистого) генеза на фоне Ph-негативного миелопролиферативного заболевания – истинной полицитемии. Описаны симптомы и нейровизуализационная картина энцефалопатии, развившейся на фоне постановки мезентерико-кавального анастомоза после перенесенного тромбоза воротной вены печени. Обсуждаются вопросы этиологии и патогенеза «печеночных» энцефалопатий, принципы терапии, а также особенности клинической картины поражения нервной системы при этом состоянии. Приводятся данные о роли марганца в развитии токсической энцефалопатии, накоплении в базальных ганглиях головного мозга парамагнитного вещества и развитии экстрапирамидной симптоматики. Описаны патогенез токсического повреждения нейронов, повышения их чувствительности к гипоксии и связь с риском нарушения мозгового кровообращения и развития хронической ишемии мозга, вносящей свой вклад в снижение когнитивных функций.

The article deals with a clinical case description of a female patient with toxic encephalopathy against the background of Ph-negative myeloproliferative diseases. The article discusses symptoms and neuroimaging of hepatic encephalopathy developed as a result of a shunt placed after portal vein thrombosis. The issues of etiology and pathogenesis of hepatic encephalopathy, principles of therapy, as well as the unique clinical picture of nervous system damage in this condition are also discussed. Data on the role of manganese in development of toxic encephalopathy, accumulation of paramagnetic substance in the basal ganglia of the brain and development of extrapyramidal symptoms are presented. The pathogenesis of toxic damage to neurons, increase in their sensitivity to hypoxia, and the relationship with the risk of cerebrovascular disorders and development of chronic cerebral ischemia, contributing to reduction of cognitive functions, are described.

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