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.
Highlights
Since Mesulam [19,20] proposed primary progressive aphasia (PPA), several cases of degenerative dementia with isolated progressive deterioration in language function have been reported, and most of these might be classified as “Pick-lobar atrophy” complex or frontotemporal lobar degeneration (FTLD) [16,21]
We apply the definitions by Darley et al [7]: dysarthria refers to speech disorders attributable to motor deficits, such as paralysis or ataxia of the speech organs, and apraxia of speech refers to those thought to be caused by deficits at a higher programming level of articulatory movements
We report a case of progressive dysarthria as a symptom of anterior operculum syndrome. This patient showed personality and behavioral changes from the onset of the disease and her magnetic resonance image (MRI) showed progressive atrophy in the frontotemporal lobes; she was diagnosed with frontotemporal lobar degeneration (FTLD)
Summary
Since Mesulam [19,20] proposed primary progressive aphasia (PPA), several cases of degenerative dementia with isolated progressive deterioration in language function have been reported, and most of these might be classified as “Pick-lobar atrophy” complex or frontotemporal lobar degeneration (FTLD) [16,21]. The types of progressive speech disorders reported include apraxia of speech We report a case of progressive dysarthria as a symptom of anterior operculum syndrome This patient showed personality and behavioral changes from the onset of the disease and her magnetic resonance image (MRI) showed progressive atrophy in the frontotemporal lobes; she was diagnosed with frontotemporal lobar degeneration (FTLD).
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