medsovetМедицинский СоветMeditsinskiy sovet = Medical Council2079-701X2658-5790REMEDIUM GROUP Ltd.10.21518/2079-701X-2020-19-42-50medsovet-5918Research ArticleНЕЙРОДЕГЕНЕРАТИВНЫЕ ЗАБОЛЕВАНИЯNEURODEGENERATIVE DISEASESДиагностика и лечение лобно-височных дегенерацийDiagnosis and treatment for frontotemporal degenerationshttps://orcid.org/0000-0003-2424-3245ГришинаД. А.GrishinaD. A.

Гришина Динара Александровна - кандидат медицинских наук, ассистент кафедры нервных болезней и нейрохирургии Института клинической медицины, Первый МГМУ имени И.М. Сеченова; врач-невролог, Российский геронтологический научно-клинический центр, РНИМУ им.Н.И. Пирогова.

119991, Москва, ул. Россолимо, д. 11, стр. 1; 129226, Москва, ул. 1-я Леонова, д. 16

Dinara A. Grishina - Cand. of Sci. (Med.), Assistant of the Department of Nervous Diseases and Neurosurgery of the Medical Faculty, Sechenov First MSMU; Neurologist, Russian Gerontology Research and Clinical Centre, Federal State Autonomous Educational Institution of Higher Education “Pirogov RNRMU.

11, Bldg. 1, Rossolimo St., Moscow, 119021; 16, 1st Leonova St., Moscow, 129226

dstepkina@mail.ruhttps://orcid.org/0000-0001-7214-583XШпилюковаЮ. А.ShpilyukovaYu. A.

Шпилюкова Юлия Александровна - врач-невролог, аспирантка, отделение нейродегенеративных и наследственных заболеваний нервной системы.

125367, Москва, Волоколамское шоссе, д. 80

Yuliya A. Shpilyukova - Neurologist, Postgraduate Student, Department of Neurogenetics.

80, Volokolamskoye shosse, Moscow, 125367

jshpilyukova@gmail.comhttps://orcid.org/0000-0001-9467-6244ЛокшинаА. Б.LokshinaA. B.

Локшина Анастасия Борисовна - кандидат медицинских наук, ассистент кафедры нервных болезней и нейрохирургии Института клинической медицины, Первый МГМУ имени И.М. Сеченова; врач-невролог, Российский геронтологический научно-клинический центр, РНИМУ им.Н.И. Пирогова.

119991, Москва, ул. Россолимо, д. 11, стр. 1; 129226, Москва, ул. 1-я Леонова, д. 16

Anastasia B. Lokshina - Cand. of Sci. (Med.), Assistant of the Department of Nervous Diseases and Neurosurgery of the Medical Faculty.

11, Bldg. 1, Rossolimo St., Moscow, 119021

aloksh@mail.ruhttps://orcid.org/0000-0001-8070-7644ФедотоваЕ. Ю.FedotovaE. Yu.

Федотова Екатерина Юрьевна - доктор медицинских наук, заведующая 5-м неврологическим (нейрогенетическим) отделением.

125367, Москва, Волоколамское шоссе, д. 80

Ekaterina Yu. Fedotova - Dr. of Sci. (Med.), Head of the 5th Neurology (Neurogenetics) Department.

80, Volokolamskoye Shosse Moscow, 125367

ekfedotova@gmail.comПервый Московский государственный медицинский университет им. И.М. Сеченова (Сеченовский Университет); Российский геронтологический научно-клинический центрРоссияSechenov First Moscow State Medical University (Sechenov University); Russian Gerontology Research and Clinical CentreRussian FederationНаучный центр неврологииРоссияResearch Center of NeurologyRussian Federation2020101220200194250Copyright © Гришина Д.А., Шпилюкова Ю.А., Локшина А.Б., Федотова Е.Ю., 20202020Гришина Д.А., Шпилюкова Ю.А., Локшина А.Б., Федотова Е.Ю.Grishina D.A., Shpilyukova Y.A., Lokshina A.B., Fedotova E.Y.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.med-sovet.pro/jour/article/view/5918

Лобно-височные дегенерации (ЛВД) - это гетерогенная группа заболеваний, характеризующихся преимущественным поражением лобных и/или передних отделов височных долей головного мозга. ЛВД является второй по распространенности деменцией в пресенильном возрасте (до 65 лет) после болезни Альцгеймера. Обычно ЛВД диагностируется в возрасте 45-65 лет, но возможен более ранний и поздний дебют. До 40% случаев ЛВД имеют положительный семейный анамнез. Согласно современной классификации выделяют следующие клинические формы ЛВД: поведенческая форма ЛВД, аграм-матическая форма первичной прогрессирующей афазии (ППА) и семантическая форма ППА. По мере своего развития эти клинические синдромы широко перекрываются друг с другом и с синдромами атипичного паркинсонизма (прогрессирующий надъядерный паралич, кортикобазальный синдром) и реже с болезнью двигательных нейронов. Вариации клинической картины определяются локализацией дегенеративно-атрофического процесса. Поведенческая форма ЛВД составляет более половины случаев ЛВД и характеризуется сочетанием когнитивных, поведенческих и эмоционально-аффективных нарушений. При ППА речевые расстройства появляются без видимой причины, непрерывно прогрессируют в отсутствие или при минимальной выраженности других когнитивных и поведенческих нарушений в течение двух и более лет. Клинические особенности ППА зависят от локализации патологического процесса. В статье рассмотрен клинический случай аграммати-ческой формы ППА. Показаны современные подходы к диагностике и ведению данной группы пациентов. Представляется целесообразным применение мемантина у пациентов с аграмматической формой ППА.

Frontotemporal degeneration (FTD) is a heterogeneous group of diseases causing neurodegeneration on the frontal and/or anterior temporal lobes. FTD is the second most common dementia in presenile age (up to 65 years) after Alzheimer’s disease. Usually, FTD is diagnosed at the age of 45-65 years, but an earlier and later onset is possible. Up to 40% of FTD cases have a positive family history. According to the current classification, the following clinical variants of FTD are distinguished: behavioral variant of FTD, agrammatic variant of primary progressive aphasia (PPA) and semantic variant of PPA. As they develop, these clinical syndromes overlap widely with each other and with atypical parkinsonism syndromes (progressive supranuclear paralysis, cortico-basal syndrome) and less often with motor neuron disease. Variations in the clinical features are determined by the localization of the degenerative-atrophic process. The behavioral variant of FTD accounts for more than half of the cases of FTD and is characterized by a combination of cognitive, behavioral, and emotional-affective disorders. In PPA, speech disorders appear for no apparent reason and progress continuously in the absence or with minimal severity of other cognitive and behavioral disorders for two or more years. The clinical features of PPA depend on the localization of the pathological process. The article reviews a clinical case of an agrammatic variant of PPA. Modern approaches to the diagnosis and management of this group of patients are shown. It seems appropriate to use memantin in patients with agrammatic variant of PPA.

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