Uncommon infections of the central nervous system

EBM Guidelines
Jun 27, 2022 • Latest change Jun 27, 2022
Auli Verkkoniemi-Ahola

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  • PML is a rare disease of the white matter of the brain that is caused by the opportunistic JC (John Cunningham) virus (JCV), a polyomavirus that has become pathogenic. Certain potent pharmacotherapies of cancer and autoimmune diseases increase the risk of disease through reactivation of the JCV. PML occurs as a rare adverse effect of these drugs.
    • The harmless primary JCV infection often occurs in childhood already. The opportunistic virus remains in the body. About one in two adults has JCV antibodies, meaning that they carry the virus.
    • JCV causes the severe PML only rarely in cases where the virus is reactivated and modified as a result of a disease impairing the patient's immunity (HIV infection) or medication (several monoclonal antibodies, immunosuppressive and immunomodulatory drugs) and causes a progressive disease of the white matter of the brain.
    • Reactivated JCV is an aggressive pathogen.

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A81.0, A81.1, A81.2, A81.8, A81.9, BSE, CJD, CSF, Cerebrospinal Fluid, Creutzfeldt-Jakob Disease, Creutzfeldt-Jakob Syndrome, Dementia, EEG, Electroencephalography, Encephalitis, Encephalopathy, Bovine Spongiform, F02.1, Infectious diseases, Internal medicine, Leukoencephalopathy, Progressive Multifocal, Neurology, SSPE, Sporadic CJD, Subacute Sclerosing Panencephalitis, Variant CJD, leukoencephalopathy, mad-cow disease, panencephalitis, prion diseases, slow virus infections of central nervous system, vCJD