TY - JOUR
T1 - Slowly progressive aphemia
T2 - A neuropsychological, conventional, and functional MRI study
AU - Gallassi, R.
AU - Sambati, L.
AU - Poda, R.
AU - Oppi, F.
AU - Maserati, M. Stanzani
AU - Cevolani, D.
AU - Agati, R.
AU - Lodi, R.
PY - 2011/12
Y1 - 2011/12
N2 - 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.
AB - 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.
KW - Aphemia
KW - Apraxia of speech
KW - Dysarthria
KW - Frontotemporal dementia
KW - Primary motor aphasia
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U2 - 10.1007/s10072-011-0625-1
DO - 10.1007/s10072-011-0625-1
M3 - Article
C2 - 21607754
AN - SCOPUS:84856215424
VL - 32
SP - 1179
EP - 1186
JO - Neurological Sciences
JF - Neurological Sciences
SN - 1590-1874
IS - 6
ER -