Zusammenfassung
Das Frontotemporale Demenzsyndrom (Frontotemporal Lobe Degeneration, FTLD) ist eine neurodegenerative Erkrankung, die vor allem durch psychopathologische Veränderungen und kognitive Defizite geprägt ist. Drei klinische Subtypen sind unterscheidbar, die eigentliche Frontotemporale Demenz (FTD), in der Wesensänderungen und Störungen des Sozialverhaltens typisch sind; die Semantische Demenz (SD), bei der es zu einem Verlust von semantischem Wissen kommt, und die Primäre Progressive Aphasie (PPA), die durch Defizite bei phonologischen und syntaktischen Sprachleistungen charakterisiert ist. Alle Varianten der FTLD haben einen langsam beginnenden und fortschreitenden Verlauf. Die vorliegende Übersichtsarbeit stellt die klinischen Charakteristika und die wichtigsten Untersuchungsbefunde der FTLD dar und befasst sich mit den Schwierigkeiten der Diagnose und Differenzialdiagnose. Die Diagnose der FTLD basiert vor allem auf der Anwendung der Lund-Manchester-Konsensus-Kriterien [1]
[2], ergänzt durch bildgebende Verfahren und eine neuropsychologische Untersuchung.
Abstract
Frontotemporal Lobar Degeneration (FTDL) is a neurodegenerative disorder which is predominantly characterized by changes in behaviour and loss of cognitive abilities. Three major clinical syndromes have been identified, Frontotemporal dementia (FTD), in which changes in social behaviour predominate, Semantic Dementia (SD) which is characterized by a loss of semantic knowledge, and Primary Progressive Aphasia (PPA), a disorder of phonological and syntactic aspects of language. All subtypes of FTLD have insiduous onset and stepwise progression. The present review lists the clinical symptoms and main findings of the three FTLD-subtypes, and discusses the difficulties of their diagnosis and differential diagnosis. The diagnosis of FTLD is based on the clinical consensus criteria of the Lund and Manchester groups [1]
[2], neuroradiological and neuropsychological investigations.
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Dr. Th. Benke
Klinik für Neurologie
Anichstr. 35
A-6020 Innsbruck, Österreich
Email: thomas.benke@uibk.ac.at
