Case Report Volume 18 Issue 6 - 2026

Psychiatric Predominant Prion Disease: A Case of Rapidly Progressive Creutzfeldt-Jakob Disease

Julia P Negrin*, Dylan Fuchs and Gautam Ullal

American University of the Caribbean School of Medicine, 1 University Drive at, Jordan Dr, Cupecoy, Sint Maarten

*Corresponding Author: Julia P Negrin, American University of the Caribbean School of Medicine, 1 University Drive at, Jordan Dr, Cu- pecoy, Sint Maarten.
Received: June 02, 2026;; Published: July 02, 2026



This case is of particular interest due to its unusually rapid clinical progression from initial symptom onset on March 23rd to death on June 2nd representing one of the fastest documented courses of Creutzfeldt-Jakob disease (CJD). While CJD typically presents with rapidly progressive dementia, ataxia, visual disturbances, and myoclonus, this report underscores the predominance of early psychiatric and behavioral manifestations preceding the classic neurologic findings. The significance of this case lies in emphasizing that initial psychological or psychiatric symptoms may represent the earliest indicators of CJD, warranting prompt neurologic evaluation and advanced diagnostic testing. Recognizing these early psychiatric presentations may facilitate earlier diagnosis, reduce unnecessary investigations, and allow families more time to prepare for the disease’s inevitable progression. Ultimately reinforcing the need for heightened clinical suspicion and multidisciplinary assessment in patients presenting with the rapidly evolving psychiatric and cognitive decline.

Keywords: Creutzfeldt-Jakob Disease (CJD); Prion Disease; Dementia; Transmissible Spongiform Encephalopathies (TSEs)

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Julia P Negrin.,et al. “Psychiatric Predominant Prion Disease: A Case of Rapidly Progressive Creutzfeldt-Jakob Disease”. EC Neurology 18.6 (2026): 01-06.