Background/Aims: We aimed at characterizing the clinical features of dementia associated with argyrophilic grain disease (AgD). Methods: Relatives or close friends of 24 individuals with autopsy-confirmed AgD and 29 patients with autopsy-confirmed Alzheimer’s disease (AD) were administered a novel Retrospective Dementia Inventory to assess the cognitive, behavioral and affective symptoms of the deceased patients. Results: AgD patients showed less severe impairments in memory, language, attention and executive function than AD patients.Conclusion: Compared to AD patients, individuals suffering from AgD appear to present with comparable deficits in behavior and affect but relatively spared cognitive functioning.