Frontotemporale lobäre degenerationen

Frontotemporal lobar degeneration (FTLD) is the second most common cause of presenile dementia. Depending on the main focus of the neurodegenerative process three clinical syndromes within FTLD can be distinguished. Frontotemporal dementia (FTD) is a behavioural syndrome with a selective involvement of the frontal and/or temporal cortices. Semantic dementia (SD) is defined as a disorder of language, semantics, and recognition of visual percept caused by bi-hemispheric anterior temporal pathology. Non-fluent progressive aphasia is a language syndrome associated with an asymmetric degeneration of the homolateral cortex, particularly located in the language dominant hemisphere. The present contribution reviews the symptoms of FTLD, summarizes the current evidence on epidemiology and aetiology, discusses diagnostic issues and shows treatment options.