title: The evaluation of rapidly progressive dementia.
abstract: BACKGROUND
  Rapidly progressive dementia (RPD) is a unique set of disorders resulting in cognitive, behavioral, and motor decline within 2 years. A variety of etiologies may contribute to RPD including neurodegenerative, inflammatory, infectious, and toxic-metabolic conditions. Jakob-Creutzfeldt disease (CJD) is frequently the most concerning diagnosis on the differential. The challenge for the neurologist is distinguishing prion disease from reversible processes that result in dementia.
  
  
  RESULTS
  This review discusses the clinical aspects and the diagnostic workup of RPD. Particular focus is given to both CJD and the potentially treatable inflammatory conditions that may cause a similar presentation. Furthermore, a standardized stepwise approach is outlined for patients presenting with RPD.
  
  
  CONCLUSIONS
  Neurologists should adopt a standardized approach to the rapidly presenting disease processes that may mimic CJD in their clinical and radiologic features.
authors.affiliation: Bedford VA Hospital, Geriatric Research and Education Clinical Center (GRECC), Bedford. Michael.H.Rosenbloom@HealthPartners.com
authors.forename: Michael Henry
authors.initials: MH
authors.lastname: Rosenbloom
authors.forename: Alireza
authors.initials: A
authors.lastname: Atri
mesh_terms: D001927:Brain Diseases / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D007562:Creutzfeldt-Jakob Syndrome / Q000134:cerebrospinal fluid / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D003704:Dementia / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology*; D018450:Disease Progression; D004569:Electroencephalography; D004660:Encephalitis; D050031:Hashimoto Disease / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D020363:Limbic Encephalitis / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D019636:Neurodegenerative Diseases / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D009460:Neurologic Examination; D017096:Prion Diseases / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D011379:Prognosis