Abstract
BACKGROUND: An 81 year old female presented with altered mental status after new onset of severe depression and suicidal ideation with recent psychiatric hospitalization. CASE PRESENTATION: Key clinical features included muscle rigidity, prominent startle reflex, and rapidly progressing cognitive decline. Initial working hypothesis was serotonin syndrome or neuroleptic malignant syndrome but continued deterioration after medication removal prompted evaluation for alternative etiology. Work-up revealed elevated 14-3-3 CSF protein which suggested the prion disorder which was confirmed on post-mortem examination of brain tissue. CONCLUSION: While the degree of depression was unusually severe, the case highlights the behavioral and psychiatric manifestations which frequently accompany Creutzfeldt-Jacob disease.