title: Atypical presentation of Creutzfeldt-Jakob disease: a rare but important cause of rapidly progressive dementia.
abstract: We report an atypical presentation of sporadic Creutzfeldt-Jakob disease (CJD) in a 74-year-old woman that illustrates the difficulty in diagnosing this rare, but important, cause of rapidly progressive dementia. Despite well-established criteria, this diagnosis is often missed or substantially delayed (Table 1). In this case, a precipitous cognitive decline associated with a urinary tract infection initiallysuggested delirium. Although atypical CJD was considered as a cause when symptoms persisted, a definitive diagnosis was established postmortem when the cerebrospinal fluid (CSF) prion protein 14-3-3 tested positive. Creutzfeldt-Jakob disease must be considered in the differential diagnosis of rapidly progressive dementia as Connecticut accounts for approximately three of the more than 200 cases diagnosed nationally.
authors: University of Connecticut Center on Aging, MC-5215, 263 Farmington Avenue, Farmington, CT 06030-5215, USA.
authors: Marguerite S
authors: MS
authors: Taillefer
authors: Glendo L
authors: GL
authors: Tangarorang
authors: George A
authors: GA
authors: Kuchel
authors: Daniel L
authors: DL
authors: Menkes
mesh_terms: D048948:14-3-3 Proteins / Q000134:cerebrospinal fluid; D000368:Aged; D015415:Biomarkers / Q000134:cerebrospinal fluid; D003237:Connecticut; D007562:Creutzfeldt-Jakob Syndrome / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000473:pathology / Q000523:psychology; D003704:Dementia / Q000175:diagnosis; D003937:Diagnosis, Differential; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans
chemical_list: D048948:14-3-3 Proteins; D015415:Biomarkers