Table 2.

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.