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Slowly progressive aphemia: a neuropsychological, conventional, and functional MRI study.

Authors
  • Gallassi, R
  • Sambati, L
  • Poda, R
  • Oppi, F
  • Stanzani Maserati, M
  • Cevolani, D
  • Agati, R
  • Lodi, R
Type
Published Article
Journal
Neurological Sciences
Publisher
Springer-Verlag
Publication Date
Dec 01, 2011
Volume
32
Issue
6
Pages
1179–1186
Identifiers
DOI: 10.1007/s10072-011-0625-1
PMID: 21607754
Source
Medline
License
Unknown

Abstract

Slowly progressive aphemia (SPA) is a rare focal degenerative disorder characterized by severe dysarthria, frequent orofacial apraxia, dysprosody, phonetic and phonemic errors without global cognitive deterioration for many years. This condition is caused by a degeneration of anterior frontal lobe regions, mainly of the left frontal operculum. We report a case of SPA with a course of 8 years, evaluated by repeated neuropsychological, conventional, and functional MRI examinations. In our case, neuropsychological examinations showed a progressive impairment of speech articulation including dysprosody, phonetic and phonemic errors, and slight writing errors. No global cognitive deterioration was detected and the patient is still completely autonomous. Morphological and functional investigations showed, respectively, a progressive atrophy and progressive impairment of the left frontal region, confirming the role of the opercular region in determining this rare syndrome. During verbal task generation as the cortical activation of this region gradually decreased, the language articulation worsened.

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