Table 3

Red flags: clinical features that might indicate a CJD mimic

Feature Indicates
FeverInfections, lymphoma
SeizuresImplies less likely to be CJD, infective and immune encephalitis, neoplasia, many other mimics
HyponatraemiaVGKC encephalitis
Facial movement disorderNMDA-R encephalitis, CNS Whipple’s disease
Modest progressionImplies less likely to be CJD, more likely to be a common neurodegenerative disorder
CSF pleocytosisInfections, lymphoma, inflammatory, neoplastic
Contrast-enhancing lesionsInfections, lymphoma, inflammatory, neoplastic
MRI hyperintensities on T2-weighted imaging outside the striatum, thalamus and cortexVascular diseases, lymphoma, progressive multifocal leukoencephalopathy, encephalitis, extrapontine myelinolysis, others
  • CJD, Creutzfeldt–Jakob disease; CNS, central nervous system; CSF, cerebrospinal fluid; NMDA-R, N-methyl-D-aspartate; VGKC, voltage-gated potassium channel.