Journal de la maladie d'Alzheimer et du parkinsonisme

Abstrait

Heterogeneity of Parkinsonism in Familial Frontotemporal Dementia: Insights from Genetic Clues on the Pathogenesis

Chin-Hsien Lin

Frontotemporal dementia (FTD) is a clinically, genetically and pathologically heterogeneous disorder characterized by personality changes, language impairment, and deficits of executive functions, often overlapped with Parkinsonism syndrome and motor neuron disorder. Owing to the advancement in the field of molecular genetics in the familiar forms of FTD, several recent breakthroughs have provided notable insights into the pathogenesis of this neurodegenerative disorder. Nevertheless, Parkinsonism in FTD has received relatively less attention. Given that Parkinsonism feature is found in approximately 15-20% of patients with FTD, there is a need to understand Parkinsonism in FTD in order to obtain a better landscape of this disease spectrum. Recent advances have revealed mechanistic links between Parkinsonism and frontotemporal dementia, as well as between other neurodegenerative diseases, such as amyotrophic lateral sclerosis. Here, we summarize recent genetic advances and molecular findings in familial FTD with Parkinsonism and delineate the mechanisms underlying this overlapped neurodegenerative disorder.