Abstract Details

Refining the concept of abrupt onset dementia by clinical characteristics, different etiologies and treatability.

165 patients with RPD were prospectively enrolled from February 2016 to September 2023 in a study of RPD at Mayo Clinic (Jacksonville, Florida) and Washington University (Saint Louis, Missouri). Patients with ≤7 days from first symptom to onset of RPD were labelled as abrupt onset. Patient characteristics, results of investigations, and causes of abrupt-onset dementia were further characterized. We further explored the causes of abrupt-onset dementia in the extant literature to refine the differential diagnosis and approach to evaluation and management of these patients.

Two patients in our study of RPD had abrupt-onset RPD (2/165, 1.2%), attributed to autoimmune and vascular. An additional 63 cases were identified through comprehensive meta-narrative review of the literature (40 publications). Causes of abrupt-onset dementia include vascular, infectious, inflammatory, and toxic. Compromise of structures within the Papez circuit was a common unifying feature common across patients with varying etiologies, informing the localization of abrupt-onset dementia. 20% cases had potentially treatment-responsive causes of abrupt-onset dementia.

Cases of abrupt-onset dementia are rare overall and associated with a limited differential diagnosis. Evaluation of patients with abrupt-onset dementia should prioritize testing for vascular, autoimmune, infectious and toxic causes. Early-recognition of patients with potentially treatment-responsive causes of dementia may promote earlier intervention leading to better outcomes in selected patients.