title: Related or not? Development of spontaneous Creutzfeldt-Jakob disease in a patient with chronic, well-controlled HIV: A case report and review of the literature.
abstract: BACKGROUND
  We report a novel case of a rare disease: spontaneous Creutzfeldt-Jakob disease in a patient with well-controlled HIV. We explore the relationship between spontaneous Creutzfeldt-Jakob disease and HIV.
  
  
  METHODS
  A 66-year-old man with long-standing, well-controlled HIV infection presented with 3 months of progressive, subacute neurocognitive decline. His symptoms included conceptual apraxia, apathy, memory impairment, and gait disturbance, and were initially attributed to depressive "pseudo-dementia." Unfortunately, the patient's symptoms rapidly progressed and he ultimately succumbed to his illness. Autopsy confirmed the clinical diagnosis of spontaneous Creutzfeldt-Jakob disease.
  
  
  CONCLUSIONS
  This case highlights spontaneous Creutzfeldt-Jakob disease as a rare terminal illness in the setting of well-controlled chronic HIV. To our knowledge, this is the first report of a patient with chronic and previously well-controlled HIV infection dying from a prion disease. Despite the very different epidemiology and pathophysiology of HIV and spontaneous Creutzfeldt-Jakob disease, this case does raise questions of whether certain host genetic factors could predispose to both conditions, albeit currently, there is no clear causal link between HIV and spontaneous Creutzfeldt-Jakob disease.
authors: Department of Neurology, Duke University Hospital, Durham, NC, USA.
authors: M-Alain
authors: MA
authors: Babi
authors: Division of Pulmonary and Critical Care Medicine, Department of Medicine, Duke University Medical Center, Durham, NC, USA.
authors: Bryan D
authors: BD
authors: Kraft
authors: Department of Neurology, Duke University Hospital, Durham, NC, USA.
authors: Sweta
authors: S
authors: Sengupta
authors: Department of Medicine, Duke University Medical Center, Durham, NC, USA.
authors: Haley
authors: H
authors: Peterson
authors: Department of Medicine, Duke University Medical Center, Durham, NC, USA.
authors: Ryan
authors: R
authors: Orgel
authors: Department of Medicine, Duke University Medical Center, Durham, NC, USA.
authors: Zachary
authors: Z
authors: Wegermann
authors: Division of Pulmonary and Critical Care Medicine, Department of Medicine, Duke University Medical Center, Durham, NC, USA; Department of Medicine, Duke University Medical Center, Durham, NC, USA.
authors: Njira L
authors: NL
authors: Lugogo
authors: Department of Neurology, Duke University Hospital, Durham, NC, USA.
authors: Matthew W
authors: MW
authors: Luedke
keywords: Creutzfeldt–Jakob disease; HIV; Prion disease; electroencephalography; encephalopathy; neurodegenerative; pathology; spongiform encephalopathy