Since Mesulam and Weintraub's (1992) original description of six patients with primary progressive aphasia (PPA), there has been considerable debate concerning the quality of the speech and language impairment, the long-term course of the disorder, the existence of syndrome subtypes, and the nature of the underlying pathological process. Me report the case of a 60-year-old woman who presented with a 4-year history of word-finding difficulty, and received a diagnosis of PPA. In contrast to a relentless deterioration of word-finding, comprehension and auditory-verbal memory, all other intellectual skills remained well preserved for a considerable period before the onset of dementia, and death. Regular detailed assessments of language, mnestic, intellectual and psychological functioning were performed over a 6-year period, as well as serial radiology and post-mortem neuropathology. We report the findings and discuss them in the context of the theoretical issues.
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Univ Tokyo, Grad Sch Med, Dept Neurol, Tokyo, JapanTohoku Univ, Grad Sch Med, Dept Behav Neurol & Cognit Neurosci, 2-1 Seiryo, Sendai 9808575, Japan
Hamada, Masashi
Toda, Tatsushi
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Univ Tokyo, Grad Sch Med, Dept Neurol, Tokyo, JapanTohoku Univ, Grad Sch Med, Dept Behav Neurol & Cognit Neurosci, 2-1 Seiryo, Sendai 9808575, Japan
Toda, Tatsushi
Matsuda, Minoru
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Tohoku Univ, Grad Sch Med, Dept Behav Neurol & Cognit Neurosci, 2-1 Seiryo, Sendai 9808575, Japan
Izumi No mori Clin, Dept Neurol, Yamato, JapanTohoku Univ, Grad Sch Med, Dept Behav Neurol & Cognit Neurosci, 2-1 Seiryo, Sendai 9808575, Japan
机构:
Univ Vita Salute San Raffaele, Milan, Italy
Ist Sci San Raffaele, Div Neurosci, In Vivo Human Mol & Struct Neuroimaging Unit, Milan, Italy
Osped San Raffaele, Nucl Med Unit, Milan, ItalyInst Adv Studies, ICoN Cognit Neurosci Ctr, IUSS, Pavia, Italy
Caminiti, Silvia P.
Boccalini, Cecilia
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Univ Vita Salute San Raffaele, Milan, ItalyInst Adv Studies, ICoN Cognit Neurosci Ctr, IUSS, Pavia, Italy
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Univ Florence, Azienda Osped Univ Careggi, Dept Neurosci Psychol Drug Res & Child Hlth, Florence, Italy
IRCCS Fdn Don Carlo Gnocchi, Florence, ItalyInst Adv Studies, ICoN Cognit Neurosci Ctr, IUSS, Pavia, Italy
Sorbi, Sandro
Perani, Daniela
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Univ Vita Salute San Raffaele, Milan, Italy
Ist Sci San Raffaele, Div Neurosci, In Vivo Human Mol & Struct Neuroimaging Unit, Milan, Italy
Osped San Raffaele, Nucl Med Unit, Milan, ItalyInst Adv Studies, ICoN Cognit Neurosci Ctr, IUSS, Pavia, Italy