Lack of relationship with apolipoprotein E4.1 to 5% of AD patients (Togo T. et al, 2002).It is almost impossible to distinguish from late-onset Alzheimer’s disease. in En, Portuguese. Grains are composed of abnormally phosphorylated tau protein with 4 repeats. Argyrophilic grain disease (AGD) was first described as a degenerative disease characterized by argyrophilic grains (AGs) in the entorhinal cortex, hippocampus, amygdala and neighbouring temporal cortex in a subset of patients who had suffered from adult onset dementia (Braak and Braak, 1987, 1989).Although the seminal descriptions emphasized the lack of Alzheimer changes, subsequent studies have show… Second, the presence of AG by itself is insufficient to cause dementia but in the presence of neurofibrillary tangle pathology, even if the second pathology is minimal, can result in dementia. The diagnosis is almost entirely made by post-mortem examination. Moreover, a common genetic background regarding the tau gene haplotype has been suggested for AgD, PSP and CBD. 2003；. Acta Neuropathol. Neuropathological examination showed findings compatible with SCA31 in the cerebellum accompanied by the finding of argyrophilic grains. AgD changes consist of the microtubule-associated protein tau in an abnormally and hyperphosphorylated state and are mainly found in limbic regions, for example, in the hippocampus, the entorhinal and transentorhinal cortices and the amygdala. Copyright © 2013 2002；12（1）：45︲52. Argyrophilic grain disease (AgD) is a late-onset dementia morphologically characterized by the presence of abundant spindle-shaped argyrophilic grains (ArG) in neuronal processes and coiled bodies in oligodendrocytes. The initial reports of argyrophilic grain disease pathology from autopsy series demonstrated an increased frequency with advanced age as well as dementia.49 The prevalence of argyrophilic grain disease pathology in cases of dementia with advanced age can be as high as 42%.4 Argyrophilic grain disease has also been reported to affect 30% of centenarians lacking the signs and symptoms of overt dementia.49,58 The clinical features of argyrophilic grain disease … A sporadic late-onset form of dementia characterised by a neuro-degenerative process, which mainly affects limbic structures (amygdala, hippocampus and mediobasal temporal/entorhinal cortex).It is named after silver-staining (argyrophilic) grains or coiled bodies within the cytoplasm of neurons that consist mainly of tau protein isoforms with four microtubule-binding repeates (4-R tau). Argyrophilic grain disease (AGD) is an under-recognized, distinct, highly frequent sporadic tauopathy, with a prevalence reaching 31.3% in centenarians. Argyrophilic grain disease (AgD) is a late-onset dementia morphologically characterized by the presence of abundant spindle-shaped argyrophilic grains (ArG) in neuronal processes and coiled bodies in oligodendrocytes. AGD lesions are found in about 5% of Alzheimer’s disease (Togo T. et al, 2002).Those related to patients affected by Alzheimer’s disease.Continued research on tau Due to the recent characterisation of this disease, there are no specific available services. AgD shows a significant correlation with advancing age, and it became apparent from recent clinicopathological studies that it might account for approximately 5% of all dementia cases.