Table 4

Differential diagnosis of rapidly progressive dementia

  • Reversible processes

    • Non-convulsive status epilepticus

    • Cerebral vasculitis/other inflammatory conditions

    • Non-inflammatory cerebrovascular disease

    • Drug toxicities (especially lithium)

    • Tuberculosis/fungal meningitis

    • Parenchymal Whipple’s disease

    • Chronic subdural haematoma/cerebral tumour

    • Paraneoplastic limbic encephalitis (may improve with treatment of tumour)

    • Antivoltage gated potassium channel antibody syndrome59

    • ?Hashimoto’s encephalopathy

  • Irreversible processes

    • Rapidly progressive neurodegenerative variants:

    • Dementia with Lewy bodies, multiple system atrophy, Alzheimer’s disease

    • Dementia (motor neurone disease) syndromes

    • Neurofilament inclusion body disease23

    • Prion diseases:

      • Sporadic, familial, and new variant Creutzfeldt-Jakob disease

      • Progressive multifocal leukoencephalopathy

      • Subacute sclerosing panencephalitis (sequela of measles)