Affiliations: Department of Rehabilitation, Kawasaki Cooperative
Hospital, Kawasaki, Japan | Department of Neurology, Kawasaki Cooperative
Hospital, Kawasaki, Japan | Department of Neurology, Showa University School of
Medicine, Tokyo, Japan
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.
