r-n-jРоссийский неврологический журналRussian neurological journal2658-79472686-7192МИА10.30629/2658-7947-2020-25-4-4-13r-n-j-94Research ArticleОБЗОРЫREVIEWSКальцификация базальных ганглиев. Этиопатогенез, диагностика, клинические проявленияBasal Ganglia Calcification. Aetiopathogenesis, Diagnostics, Clinical Manifestationshttps://orcid.org/0000-0002-9305-8679ХореваМ. А.KhorevaM. A.

Хорева Марина Александровна — канд. мед. наук, доцент кафедры психиатрии, медицинской психологии и неврологии с курсом ДПО, 656038, пр. Ленина, 40, Барнаул, Россия

Khoreva Marina Aleksandrovna — Department of Psychiatry, Medical Psychology and Neurologywith the Course of the DPO, Barnaul

marinakhoreva@mail.ruhttps://orcid.org/0000-0002-7947-4529СмагинаИ. В.SmaginaI. V.

Барнаул

Barnaul

siv7000@yandex.ruФГБОУ ВО «Алтайский государственный медицинский университет» Минздрава РоссииРоссияAltai State Medical UniversityRussian Federation202018102020254413Copyright © Хорева М.А., Смагина И.В., 20202020Хорева М.А., Смагина И.В.Khoreva M.A., Smagina I.V.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/94

Болезнь Фара — это редкое наследственное или спорадическое неврологическое заболевание, характеризующееся билатеральным отложением кальция в области базальных ганглиев, зубчатых ядрах мозжечка и субкортикальном белом веществе головного мозга. Выделяют также синдром Фара, этиология которого связана с нарушением кальциевого обмена, митохондриопатиями, новообразованиями головного мозга, инфекциями, воспалительными заболеваниями нервной системы, травмами. Клиническая манифестация болезни может возникать в разном возрасте, но в основном у взрослых молодого и среднего возраста. Главными клиническими проявлениями болезни являются экстрапирамидные двигательные расстройства, эмоциональные и когнитивные нарушения. При этом соответствие формы и тяжести неврологических расстройства характеру кальциноза базальных ганглиев встречается нечасто. Причем некоторые пациенты остаются асимптомными в течение всей жизни. В настоящее время стратегия лечения болезни Фара основана на симптоматической терапии и коррекции этиологических факторов при синдроме Фара. Имеются сведения об обратимости процесса кальцификации и полного восстановления ментальных функций при ранней диагностике и лечении синдрома Фара.

Fahr disease is a rare hereditary or sporadic neurological condition characterized by bilateral calcium deposition in the basal ganglia, dentate nuclei of cerebellum, and subcortical white matter. We can also distinguish Farh syndrome when its etiology is associated with the disorder of calcium metabolism, mitochondriopathies, cerebrum neoplasms, infections, inflammatory diseases of the nervous system, and injuries. The most common manifestations in patients with calcification of the basal ganglia of cerebrum are neurological and/or psychiatric disorders of varying severity. The clinical manifestation of the disease can occur at different ages, but mainly in young and middle-aged adults. However, some patients remain asymptomatic throughout their lives. The main clinical manifestations of the disease are extrapyramidal and movement disorders, emotional and cognitive impairments. At the same time, the correspondence of the form and severity of neurological conditions and the nature of calcification of the basal ganglia is rare. Currently, the treatment strategy for Fahr disease is based on symptomatic therapy and correction of etiological factors in Fahr syndrome. There is information about the reversibility of the calcification process and the complete restoration of mental functions in the early diagnosis and treatment of Fahr syndrome.

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