Differential diagnosis checklist
| Common disorders with rare presentation that mimic CJD | Rare but potentially treatable/reversible mimics | Rare and untreatable mimics of CJD |
| Rapidly progressive forms of common neurodegenerative diseases | Limbic and other immune-mediated encephalitis | Unusual infections: subacute sclerosing panencephalitis |
| Delirium and pre-existing dementia | Metabolic and endocrine conditions (hyperammonaemia, electrolyte disturbance, hypoglycaemia/hyperglycaemia, uraemia) | Mitochondrial cytopathy |
| Viral encephalitis | Primary CNS vasculitis | Diffuse neoplastic disease (eg, carcinomatous meningitis, gliomatosis cerebri) |
| Hepatic failure and encephalopathy | Neurosarcoidosis | |
| Cerebrovascular disease (single or multiple strokes) or hypoxic encephalopathy | Primary CNS and intravascular lymphoma | |
| Wernicke’s encephalopathy and related manifestations of alcohol-related dementia (eg, extrapontine myelinolysis) | Unusual infections: progressive multifocal leukoencephalopathy, fungal encephalitis, Lyme disease, Whipple’s disease, neurosyphilis | |
| Thyroid dysfunction | Large dural arteriovenous fistula | |
| HIV-related dementia | ||
| Subacute combined degeneration | ||
| CNS manifestations of autoimmune disorders (eg, lupus, sarcoidosis) | ||
| Heavy metal toxicity (eg, lithium, mercury) | ||
| Toxicity related to medications (eg, lithium, valproate) | ||
| Non-convulsive status epilepticus | ||
| Psychiatric conditions: functional disorders, catatonia, depression |
CJD, Creutzfeldt–Jakob disease; CNS, central nervous system.