Creutzfeldt Jakob disease (CJD): differential diagnosis

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

Metabolic

Autoimmune

Inflammatory

Infections

Malignancy

Features favouring CJD mimics

Features favouring CJD

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. 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.
4. 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.
5. Xu Z, Zhao Y. A Creutzfeldt-Jakob disease case misdiagnosed with acute cerebral infarction and review of the literature. Clin Case Rep 2020; 8:3311-3315.
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Related checklists

• Creutzfeldt Jakob disease (CJD): clinical features
• Creutzfeldt Jakob disease (CJD): investigations
• Creutzfeldt Jakob disease (CJD): treatment
• New variant Creutzfeldt Jakob disease (nvCJD)