title: Frontotemporal dementia parkinsonism: Clinical findings in a large Iranian family.
abstract: Frontotemporal dementia (FTD) is a group of neurodegenerative disorders characterized by atrophy of the frontal and temporal lobes. Clinical features suggestive of FTD include pre-senile onset before the age of 65, behavioral changes, social and interpersonal disinhibition, fluent and nonfluent aphasia, and loss of insight. FTD and parkinsonism linked to chromosome 17 (FTDP-17) was defined during the International Consensus Conference in Ann Arbor, Michigan in 1996. FTDP-17 is an autosomally dominant inherited condition. Most genotypic alterations do not correlate with clinical phenotypes. However, mutations affecting exon 10 splicing are associated with parkinsonism. In the present study, a male case with FTDP who presented with insidious onset of speech difficulty at a young age that was associated with signs of parkinsonism and a positive family history of FTD with MAPT gene mutation at exon 13 has been reported.
authors: Department of Neurology, Isfahan University of Medical Sciences, Isfahan, Iran.
authors: Keivan
authors: K
authors: Basiri
authors: Department of Neurology, Isfahan University of Medical Sciences, Isfahan, Iran.
authors: Behnaz
authors: B
authors: Ansari
authors: Isfahan Neurosciences Research Center, Isfahan, Iran.
authors: Rokhsareh
authors: R
authors: Meamar
keywords: Dementia; MAPT gene; frontotemporal dementia parkinsonism