Demenz mit argyrophilen Granula

Zusammenfassung

Die Demenz mit argyrophilen Granula (AG) stellt eine erst seit 1987 durch H. und E. Braak beschriebene neuropathologisch definierte Erkrankung dar, die in höherem Alter zu einer Demenz führen kann. Sie tritt häufig mit den Zeichen einer Alzheimer-Pathologie kombiniert auf, scheint aber dennoch eine eigenständige Entität zu sein, da zweifelsfrei belegte reine Fälle mitgeteilt wurden. Sie kann klinisch dem Morbus Alzheimer oder dem Morbus Pick ähneln, war bisher fast nur im Senium anzutreffen und verläuft unaufhaltsam, meist langsam progredient. Wiederholt wurde auf prominente Verhaltensstörungen hingewiesen. Brauchbare Behandlungskonzepte wurden bisher noch nicht entwickelt, in Einzelfällen konnten Acetylcholinesterasehemmer eine vorübergehende günstige Wirkung zeitigen, auch Amantadine und Dopaminagonisten kommen aus theoretischen Erwägungen in Betracht. Die Diagnose ist nur histopathologisch sicher zu stellen. Klinisch ist es wichtig zu wissen, dass die Erkrankung, die zu den so genannten Tauopathien gerechnet wird, eine Zusatz- und Differenzialdiagnose des Morbus Alzheimer wie der frontotemporalen Demenzen darstellt. Die Übersicht wird durch eine eigene Falldarstellung abgerundet.

Abstract

Dementia with argyrophilic grains (AG) is a neuropathologically defined potentially dementing disease of advanced age which was first described by H. and E. Braak in 1987. It is often combined with histopathological signs of Alzheimer's disease but has nevertheless to be considered as a distinct entity since pure bona fide cases have been reported. Clinically it may resemble either Alzheimer's or Pick's disease; it is almost exclusively confined to patients older than 60 years and progresses slowly but relentlessly. Repeatedly prominent behavioral disorders were pointed out. Useful therapeutic regimens are not available at present but acetylcholinesterase inhibitors have proven effective in some cases and amantadines as well as dopamine agonists hold some promise from a theoretical perspective, too. The diagnosis can be ascertained only by histopathological examination. For the clinician it is important to know that this disease is to be considered as an additional and differential diagnosis of both Alzheimer’s disease and the frontotemporal dementias. It must be counted among the tauopathies. This review is supplemented by a detailed case report.

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