Clinical aspects of argyrophilic grain disease

Argyrophilic grain disease (AGD) is a frequently occurring degenerative illness of the aging human brain. It is accompanied by progressive pathological alterations of the cytsokeleton which are traceable to an abnormal phosphorylation of the microtubule associated tau protein. Histologically, it is possible with the help of suitable staining techniques to identify pathognomonic spindle-shaped cellular inclusions (argyrophilic grains). These cellular inclusions display a typical cortical as well as subcortical distribution pattern. The goal of the present study is the retrospective evaluation of the clinical findings from 53 individuals with neuropathologically demonstrable 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 growing severity of the AGD-associated pathological cytoskeletal degeneration. These results confirm the assumption that AGD can cause degenerative changes ranging from cognitive impairment all the way to dementia. They also underscore the necessity of further prospective studies pertaining to the clinical aspects of this still enigmatic disease.