Creutzfeldt Jakob disease (CJD): differential diagnosis

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Creutzfeldt Jakob disease (CJD): differential diagnosis. Alzheimer disease, Frontotemporal dementia (FTD). Fully referenced neurology checklist.

Neurodegenerative

  • Alzheimer disease
  • Frontotemporal dementia (FTD)
  • Progressive supranuclear palsy (PSP)
  • Corticobasal degeneration (CBD)
  • Dementia with Lewy bodies (DLB)
  • Adult polyglucosan body disease
  • Huntington’s disease (HD)

Vascular

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Metabolic

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Autoimmune

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Inflammatory

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Infections

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Malignancy

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Features favouring CJD mimics

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Features favouring CJD

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References

  1. Chitravas N, Jung RS, Kofskey DM, et al. Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease. Ann Neurol 2011; 70:437-444.
  2. Dorey A, Tholance Y, Vighetto A, et al. Association of cerebrospinal fluid prion protein levels and the distinction between Alzheimer disease and Creutzfeldt-Jakob disease. JAMA Neurol 2015; 72:267-275.
  3. Elgenidi A, Shir D, Piura YD, et al. Prion disease mimicking rapidly progressive Alzheimer disease: case series and systematic review. J Neurol 2026; 273:565.
  4. Tsivgoulis G, Bonakis A, Papathanasiou MA, et al. Magnetic resonance imaging reveals Creutzfeldt-Jakob disease in a patient with apparent dementia with Lewy bodies. J Neurol Sci 2014; 340:130-132.
  5. Dirzius E, Balnyte R, Steibliene V, et al. Sporadic Creutzfeldt-Jakob disease with unusual initial presentation as posterior reversible encephalopathy syndrome: a case report. BMC Neurol 2016; 16:234.
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