title: A case of frontotemporal lobar degeneration with progressive dysarthria.
abstract: We investigated the evolution of the neurological and neuropsychological characteristics in a right-handed woman who was 53-years-old at the onset and who showed personality changes and behavioral disorders accompanied by progressive dysarthria. She had hypernasality and a slow rate of speech with distorted consonants and vowels, which progressed as motor disturbances affecting her speech apparatus increased; finally, she became mute two years post onset. Her dysarthria due to bilateral voluntary facio-velo-linguo-pharyngeal paralysis accompanied with automatic-voluntary dissociation fit the description of anterior opercular syndrome. She showed personality changes and behavioral abnormalities from the initial stage of the disease, as is generally observed in frontotemporal degeneration (FTD), and her magnetic resonance image showed progressive atrophy in the frontotemporal lobes; thus, she was clinically diagnosed with FTLD. This patient's symptoms suggest that FTLD, including bilateral anterior operculum degeneration, causes progressive pseudobulbar paretic dysarthria accompanied by clinical symptoms of FTD, which raises the possibility of a new clinical subtype in the FTLD spectrum.
authors.affiliation: Department of Rehabilitation, Kawasaki Cooperative Hospital, Kawasaki, Japan. ihorinami@aol.com
authors.forename: Nami
authors.initials: N
authors.lastname: Ihori
authors.forename: Shigeo
authors.initials: S
authors.lastname: Araki
authors.forename: Kenji
authors.initials: K
authors.lastname: Ishihara
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Kawamura
mesh_terms: D001284:Atrophy / Q000473:pathology; D018450:Disease Progression; D004401:Dysarthria / Q000150:complications / Q000473:pathology* / Q000503:physiopathology*; D005260:Female; D005625:Frontal Lobe / Q000473:pathology* / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009410:Nerve Degeneration / Q000473:pathology* / Q000503:physiopathology*; D010700:Phonetics; D013702:Temporal Lobe / Q000473:pathology* / Q000503:physiopathology*; D014832:Voice Disorders / Q000150:complications / Q000175:diagnosis