SummaryRapidly progressive dementias are conditions that typically cause dementia over weeks or months. They are a particular challenge to neurologists as the differential diagnosis often is different from the more typical, slowly progressive dementias. Early and accurate diagnosis is essential, as many of the etiologies are treatable. The information in this review is in part based on experience through our rapidly progressive dementia program at the University of California San Francisco, Memory and Aging Center. As treatment of a rapidly progressive dementia is entirely dependent on the diagnosis, we present a comprehensive, structured, but pragmatic approach to diagnosis, including key clinical, laboratory, and radiologic features. For the 2 most common causes of rapid dementia, treatment algorithms for the autoimmune encephalopathies and symptomatic management for the neurodegenerative causes are discussed.A lthough no formal definition exists for what constitutes a rapidly progressive dementia (RPD), generally we use the term when dementia occurs in less than 1-2 years from illness onset, but more commonly over weeks to months.1 Because these conditions are relatively uncommon, the appropriate diagnostic workup and treatments often are unfamiliar to many neurologists. Accurate, thorough, and prompt diagnosis is important as many RPDs are treatable, and even curable. In this article, we present a practical, systematic approach for RPD diagnosis as well as treatment algorithms for the management of immunotherapy-responsive and other dementias.
Etiology of RPDsThe breakdown of etiologies of RPDs varies among dementia centers. At our center (University of California, San Francisco, Memory and Aging Center), the diagnostic breakdown of RPDs *These authors contributed equally to this work.