Slowly progressive aphemia: A neuropsychological, conventional, and functional MRI study

R. Gallassi, L. Sambati, R. Poda, F. Oppi, M. Stanzani Maserati, D. Cevolani, R. Agati, R. Lodi

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)1179-1186
Number of pages8
JournalNeurological Sciences
Volume32
Issue number6
DOIs
Publication statusPublished - Dec 2011

Keywords

  • Aphemia
  • Apraxia of speech
  • Dysarthria
  • Frontotemporal dementia
  • Primary motor aphasia

ASJC Scopus subject areas

  • Clinical Neurology
  • Psychiatry and Mental health
  • Dermatology

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