Diagnosis of dementia with grains (argyrophilic grain disease)

Tadashi Adachi¹⁾, Yuko Saito²⁾, Kenji Nakashima¹⁾, Shigeo Murayama¹⁾

¹⁾Division of Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University
²⁾Department of Pathology and Laboratory Medicine, National Center for Neurology and Psychiatry Hospital
³⁾Department of Neurology & Bioresource Center, Tokyo Metropolitan Geriatric Hospital & Institute of Gerontology

Argyrophilic grain disease is a sporadic four repeat tauopathy. We propose the following clinical diagnostic criteria for this disease:late onset slowly progressive dementia, often presenting as initially amnesia (similar to Alzheimer's disease or dementia with Lewy bodies), and well-preserved executive function with less involvement of the neocortex. In addition, irritability or agitation, similar to that seen with frontotemporal dementia, is supported by imaging studies that showed atrophy and hypometabolism of the anterior temporal lobe with frequent asymmetry. Lastly, mild elevation of phosphorylated tau levels, but not so high compared with Alzheimer's disease by cerebrospinal fluid examination.

Address correspondence to Dr. Tadashi Adachi, Division of Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University(36-1 Nishi-cho, Yonago-shi, Tottori 683-8504, Japan)