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Dementia with argyrophilic grains (AG) is a neuropathologically defined potentially dementing disease of advanced age which was first described by H. and E. Braak in 1987. It is often combined with histopathological signs of Alzheimer's disease but has nevertheless to be considered as a distinct entity since pure bona fide cases have been reported. Clinically it may resemble either Alzheimer's or Pick's disease; it is almost exclusively confined to patients older than 60 years and progresses slowly but relentlessly. Repeatedly prominent behavioral disorders were pointed out. Useful therapeutic regimens are not available at present but acetylcholinesterase inhibitors have proven effective in some cases and amantadines as well as dopamine agonists hold some promise from a theoretical perspective, too. The diagnosis can be ascertained only by histopathological examination. For the clinician it is important to know that this disease is to be considered as an additional and differential diagnosis of both Alzheimer's disease and the frontotemporal dementias. It must be counted among the tauopathies. This review is supplemented by a detailed case report.