The Japanese journal of neuropsychology

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ArticleTitle	A case initially presenting as rapidly progressive aphasia without dementia -The concept and pathogenesis of rapidly progressive aphasia-
Language	J
AuthorList	Yoko Warabi¹⁾, Maki Ishikawa²⁾, Eiji Isozaki¹⁾, Mitsuaki Bandoh¹⁾, Hideaki Hayashi¹⁾
Affiliation	Departments of ¹⁾Neurology and ²⁾Rehabilitation, Tokyo Metropolitan Neurological Hospital
Publication	Japanese Journal of Neuropsychology: 19 (4), 200-207, 2003
Received	Dec 5, 2002
Accepted	Sep 19, 2003
Abstract	A presenile-onset case of dementia initially presenting with rapidly progressive aphasia without dementia (RPA) is reported. A 50-year-old, right-handed man presented with difficulty in finding words. During the next seven months Wernicke's aphasia developed. Neuropsychological evaluation demonstrated aphasia without dementia, e.g., VIQ 52 and PIQ 91 in WAIS-R, RCPM 33. Three months later, dementia appeared and he died in a state of akinetic mutism 26 months after the onset of neurological symptoms. At the time RPA developed, MRI showed brain atrophy, more severe in the left temporal lobe, and hyperintense cortical areas in the same region on FLAIR and diffusion-weighted images. ECD-SPECT disclosed diminished perfusion of the left temporal and parietal lobes. These abnormalities on MRI and SPECT gradually progressed. There have been few reports evaluating the relation of RPA and the results of standard neuropsychological and language tests and there have been no papers presenting serial MRI and SPECT of RPA patients. Five reports describing autopsy cases showing similar antemortem manifestations of RPA were reviewed. Difficulty in finding words rapidly progressed to complete aphasia within less than one year, while the duration of aphasia without dementia was less than nine months. Compared with SPA, typical Broca or Wernicke's aphasia is observed in RPA. There had been brain atrophy resembling that of SPA at the time of aphasia without dementia in RPA. Neuropathologically, a diagnosis of Creutzfeldt-Jakob disease (CJD) was made in four of five RPA patients and Alzheimer's disease (AD) was diagnosed in one patient. We suppose that CJD is a likely diagnosis in our patient, although a pathological diagnosis was not made. RPA may appear as an exceptional form of rapidly progressive neurological disorders, including CJD and AD, when these diseases start with focal brain damage in language-related areas. Furthermore, the speed and type of aphasia deterioration, as well as the duration of aphasia without dementia, should provide a new informative perspective on progressive aphasia.
Keywords	rapidly progressive aphasia without dementia, slowly progressive aphasia without dementia, Creutzfeldt-Jakob disease, MRI, SPECT