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.

journal_name

Behav Neurol

journal_title

Behavioural neurology

authors

Ihori N,Araki S,Ishihara K,Kawamura M

doi

10.1155/2006/320638

subject

Has Abstract

pub_date

2006-01-01 00:00:00

pages

97-104

issue

2

eissn

0953-4180

issn

1875-8584

journal_volume

17

pub_type

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