| Budget Amount *help |
¥2,800,000 (Direct Cost: ¥2,800,000)
Fiscal Year 2006: ¥500,000 (Direct Cost: ¥500,000)
Fiscal Year 2005: ¥500,000 (Direct Cost: ¥500,000)
Fiscal Year 2004: ¥1,800,000 (Direct Cost: ¥1,800,000)
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| Research Abstract |
Argyrophilic grain dementia (AGD) is a late onset tauopathy characterized by the occurrence of argyrophilic and tau-immunoreactive grains most abundant in the limbic system, especially in the CA1 region of the hippocampus, the entorhinal and transentorhinal cortex, the adjacent temporal isocortex, and the amygdala. These oval, spindle-shaped or comma-like grains can be best detected by the Gallyas-Braak silver technique. Many tau-immunoreactive pretangles with poor neurofibrillary tangles (NFT)-formation are distributed in the limbic system. Tau-immunoreactive glial changes seen in oligodendroglial coiled bodies and astrocytes represent a consistent finding. Ballooned neurons expressing a B-crystallin are constant features in the amygdala of AGD brains. NFT found in AGD patients generally correspond to early (transitional and limbic) Braak stages.
The prevalence of AGD increases with advancing age. Frequency of AGD in 32 centenarians among 3328 autopsied brains was 31.3%. In the demented group, 9 (39.1%) of 23 Cases were found with AGD, while in the non-demented group, AGs were found on only 1 (11.1%) of nine cases. Dementia caused by pure AGD accounted for 13% (3/23) among demented subjects. Frequency of AGD in 110 cases with amyotrophic lateral sclerosis (age 32〜86) was 20%. Clinically 7 cases showed mild mental symptoms. Clinicopathological spectrum of AGD remains to be unclear.
We reported a case with severe frontotemporal atrophy accompanied by numerous AGs. At the age of 49, he exhibited personality change, speech disorder and abnormal behavior. The neuropathological findings of extensive-type AGD would constitute a part of pathological background of frontotemporal dementia.
AGs were immunopositive for 4-repeat tau on immunohistochemical study, using two monoclonal antibodies, RD3, RD4 that effectively distinguish between 3-repeat and 4-repeat tau.
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