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[Vol.22 No.4 contents]
Japanese/English
Full Text of this Articlein Japanese PDF (175K) |
| ArticleTitle | Two sibling patients with Alzheimer's disease associated with presenile onset and very slowly progressive clinical course |
| Language | J |
| AuthorList | Yoshimi Kitade1), Atsushi Sato2), Toru Imamura1)3) |
| Affiliation |
1) Department of Speech Therapy, School of Health Sciences, Niigata University of Health and Welfare 2) Division of Speech Therapy, Department of Rehabilitation, Niigata Rehabilitation Hospital 3) Department of Neurology, Niigata Rehabilitation Hospital |
| Publication | Japanese Journal of Neuropsychology: 22 (4), 260-268, 2006 |
| Received | Oct 24, 2005 |
| Accepted | May 8, 2006 |
| Abstract | We reported two sibling patients with Alzheimer's disease (AD) who had 5th decade of the onset age and very slowly progressive clinical course. Patient 1, a 67-year old housewife, presented to us because of a 13-year history of progressive forgetfulness. She was awake but mildly inattentive. She scored 23 on the MMSE and 17 on the Alzheimer's Disease Assessment Scale (ADAS). Amnesia, disorientation and constructional disturbance were apparent. The phenotype of apolipoprotein E (ApoE) was E3/E3. An MR brain imaging revealed a severe atrophy in the medial temporal lobes. Patient 2, a 73-year old housewife, an elder sister of patient 1, presented to us because of a 17-year history of slowly progressive forgetfulness. She was awake but slightly inattentive. She scored 14 on the MMSE and 31 on the ADAS. Amnesia, disorientation and executive dysfunction were observed. The phenotype of ApoE observedwas E3/E3. An MR brain imaging showed a severe atrophy in the medial temporal lobes, and a single photon emission CT (SPECT) of the brain showed a hypoaccumulation in the medial temporal and the parietal lobes. The clinical diagnosis of the two sibling patients was AD based on the history and the neuropsychological and neuroimaging findings. The characteristic of the current patients is an extremely slowly progression when compared with the familial AD with presenile onset and known gene abnormality including amyloid precursor protein, presenilin-1 and presenilin-2. There may be an unknown gone abnormality in the current AD family with presenile onset and extremely slow progression. |
| Keywords | familial Alzheimer's disease, presenile onset, clinical course, gene abnormality |
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