Tom 19, Nr 4 (2023)
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Eksport do Mediów Społecznościowych

Eksport do Mediów Społecznościowych

Zaburzenia zachowania w czasie snu REM — wczesna manifestacja kliniczna α-synukleinopatii

Aneta Marcinkowska1, Andrzej Bogucki1, Agata Gajos1
Pol. Przegl. Neurol 2023;19(4):268-283.

Streszczenie

Zaburzenia zachowania w czasie snu REM (RBD, rapid eye movement sleep behavior disorders) są parasomnią. Dochodzi wtedy do utraty atonii mięśni w czasie snu REM. W RBD w tej fazie snu występują marzenia senne, których treść stanowią sytuacje stwarzające zagrożenie dla pacjenta (np. atak ze strony człowieka lub zwierzęcia). Marzeniom sennym towarzyszy bogata aktywność ruchowa i wokalna pozostająca w ścisłym związku z ich treścią. W czasie incydentu RBD obrażeń może doznać pacjent i osoba towarzysząca. Rozpoznanie RBD ustala się w przypadku występowania charakterystycznych objawów klinicznych po wykonaniu polisomnografii i wykazaniu braku atonii w czasie snu REM. W badaniach skriningowych stosowane są również kwestionariusze diagnostyczne. Lekami najczęściej stosowanymi w leczeniu RBD są klonazepam i malatonina. Zaburzenia zachowania w czasie snu REM stanowią wczesną manifestację chorób neurozwyrodnieniowych z grupy α-synukleinopati: choroby Parkinsona (najczęściej), otępienia z ciałami Lewyego oraz zaniku wieloukładowego. Z danych z metaanalizy wynika, że do fenokonwersji dochodzi u 33,5% pacjentów po pięciu latach obserwacji, u 82,4% po 10,5 roku, a po 14 latach u 96,6%. Podejmowane są próby wskazania markerów prognostycznych konwersji — zwłaszcza wczesnej — RBD do jednej z pełnoobjawowych synukleinopatii. Nieprawidłowy wynik obrazowania układu dopaminergicznego jest sam w sobie czynnikiem ryzyka szybkiej konwersji, a jego wartość wzrasta, gdy zostanie połączony z oceną węchu, funkcji poznawczych i autonomicznych. Badania histopatologiczne umożliwiają stwierdzenie obecności patologicznej, fosforylowanej formy α-synukleiny w wycinkach z jelita grubego, ślinianki i skóry. Przedmiotem dyskusji pozostaje sposób informowania o fenokonwersji pacjentów z RBD w sytuacji, gdy nie wiadomo, kiedy do tego dojdzie, a przede wszystkim, gdy nie można zaoferować pacjentowi leczenia modyfikującego przebieg procesu neurozwyrodnieniowego.

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