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Updated: Nov 28 2021

Creutzfeldt-Jakob disease

  • Snapshot
    • A 55-year-old man is brought to the emergency department by his wife after falling down and having a generalized tonic-clonic seizure. Over the course of the past few months, she felt he has been having abnormal behavior. He was previously admitted to the inpatient psychiatric service after having delusions and violent outbursts. On physical exam, the patient was nonparticipatory due to being postictal. A noncontrast CT head was unremarkable. He was admitted to the neurology service for evaluation of his seizures. He was placed on video electroencephalogram and it was remarkable for biphasic synchronous sharp wave complexes. An MRI brain demonstrated cortical ribboning with caudate head signal changes. Cerebrospinal fluid analysis was remarkable for the presence of 14-3-3 protein.
  • Introduction
    • Definition
      • human prion disease that leads to a rapid progressively dementia
    • Types
      • spontaneous Creutzfeldt-Jakob disease (CJD)
        • most common form
          • results in the spontaneous formation of prions for unknown reasons
      • familial CJD
        • spotaneous formation of prions secondary to autosomal dominant mutations
      • acquired CJD
        • iatrogenic transmission (e.g., neurosurgical transmission)
        • dietary exposure
  • Pathogenesis
    • Abnormal prion isoform leads to prion misfolding within neurons
  • Presentation
    • Symptoms/physical exam
      • rapidly progressive mental deterioration
        • poor concentration, memory, and executive function
      • myoclonus
        • provoked by startle (thus "startle myoclonus')
  • Imaging
    • MRI brain
      • findings
        • cortical ribboning
        • signal intensity in the putamen and head of the caudate on T2 and FLAIR
  • Studies
    • Electroencephalogram
      • synchronous sharp wave complexes (has a very high specificity)
    • Lumbar puncture
      • presence of 14-3-3 protein
        • a negative study does not exclude CJD
    • Neuropathology
      • spongiform vacuolation
      • abnormal accumulation of abnormal prion protein
  • Differential
    • Alzheimer disease (AD)
      • differentiating factors
        • AD does not rapidly progress like CJD
  • Treatment
    • There is no effective treatment for this fatal disease
  • Complications
    • Aspiration and pneumonia secondary to dysphagia (most common cause of death)
  • Prognosis
    • Typically death occurs within 1-2 years of symptom onset
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