Clinical aspects of argyrophilic grain disease

Argyrophilic grain disease (AGD) is a frequently occurring degenerative ill ness of the aging human brain. It is accompanied by progressive pathologica l alterations of the cytsokeleton which are traceable to an abnormal phosph orylation of the microtubule associated tau protein. Histologically, it is possible with the help of suitable staining techniques to identify pathogno monic spindle-shaped cellular inclusions (argyrophilic grains). These cellu lar inclusions display a typical cortical as well as subcortical distributi on pattern. The goal of the present study is the retrospective evaluation o f the clinical findings from 53 individuals with neuropathologically demons trable AGD-related changes of the brain. Nearly one-half of the cases (49%) was classifiable as demented in accordance with DSM IV-criteria. Moreover, the frequency of the dementia increased significantly in relation to the gr owing severity of the AGD-associated pathological cytoskeletal degeneration . These results confirm the assumption that AGD can cause degenerative chan ges ranging from cognitive impairment all the way to dementia. They also un derscore the necessity of further prospective studies pertaining to the cli nical aspects of this still enigmatic disease.