r-n-jРоссийский неврологический журналRussian neurological journal2658-79472686-7192МИА10.30629/2658-7947-2024-29-1-52-58r-n-j-524Research ArticleИССЛЕДОВАНИЯ И КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCLINICAL RESEARCHES AND CASE REPORTSСиндром Ландау–КлеффнераLandau–Kleff ner syndromehttps://orcid.org/0000-0002-5397-9422МандраЕ. В.MandraE. V.

Москва

Moscow

emandra97@mail.ruhttps://orcid.org/0000-0001-5937-9463ЗиновьеваО. Е.ZinovyevaO. E.

Москва

Moscow

zinovyevaolga@yandex.ruhttps://orcid.org/0000-0001-6729-1875ТоропинаГ. Г.ToropinaG. G.

Москва

Moscow

ggtoropina@mail.ruПервый Московский государственный медицинский университет им. И.М. Сеченова (Сеченовский Университет)РоссияI.M. Sechenov First Moscow State Medical University (Sechenov University)Russian Federation2024120320242915258Copyright © Мандра Е.В., Зиновьева О.Е., Торопина Г.Г., 20242024Мандра Е.В., Зиновьева О.Е., Торопина Г.Г.Mandra E.V., Zinovyeva O.E., Toropina G.G.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/524

Представлено длительное катамнестическое наблюдение (в течение 15 лет) пациента с синдромом Ландау–Клеффнера (СЛК). В дебюте наблюдались эпилептические приступы, речевые нарушения, быстропрогрессирующие до тотальной афазии, когнитивные и поведенческие расстройства, в ЭЭГ разряды пик-волна в височных отведениях. МРТ в режиме эписканирования патологии не обнаружила. Пациент получал леветирацетам и гормональную терапию (глюкокортикостероиды, аналоги АКТГ) с регрессом эпилептических приступов, эпилептических разрядов в ЭЭГ и положительной динамикой речевых и других когнитивных функций. Через 15 лет у пациента наблюдался умеренный когнитивный дефицит. Приведены данные литературы о катамнестическом наблюдении пациентов с СЛК.

The article presents a long-term catamnestic observation (15 years) of a patient with Landau–Kleff ner syndrome (LCS), in its debut manifested by epileptic paroxysms, total aphasia, cognitive and behavioral impairment, on EEG spike-wave complexes. MRI in epileptic mode was without pathology. He received levetiracetam and hormone therapy (glucocorticosteroids, ACTH analogues) with regression of epileptic seizures and epileptic discharges on EEG and pronounced positive dynamics of speech and other cognitive functions. After 15 years, the patient had a confi dent cognitive defi cit, speech disorders were almost absent.

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