neurologic disorders
Progressive Multifocal Leukoencephalopathy (PML)
Treatment of PML
Supportive care Treatment of progressive multifocal leukoencephalopathy is mainly supportive. and other antivirals has failed to provide benefit. Antiretroviral therapy (ART) in AIDS patients has improved outcome in PML, increasing the 1-year survival rate from 10 to 50%. However, patients treated with aggressive antiretroviral therapy may develop Immune reconstitution inflammatory syndrome (IRIS) (IRIS). In IRIS, the recovering immune system produces an intense inflammatory response against the JC virus, thus worsening symptoms. Imaging done after IRIS develops shows greater contrast enhancement of the lesions and may show significant cerebral edema. Corticosteroids may be helpful. Depending on the severity of IRIS and of AIDS, clinicians may decide to withdraw ART. Withdrawal of immunosuppressants may result in clinical improvement. However, patients who stop taking these drugs are also at risk of developing IRIS. If PML develops in patients takingnatalizumab, another immunomodulatory drug, or an immunosuppressant, the drug should be stopped, and plasma exchange should be done to remove residual circulating drug. (immune checkpoint inhibitors) may decrease viral load in the CSF, increase antiviral cellular immune activity, and lead to clinical improvement ( Treatment references , 1.Cortese I, Muranski P, Enose-Akahata Y, et al:Pembrolizumabtreatment for progressive multifocal leukoencephalopathy.N Engl J Med380 (17):1597–1605, 2019. doi: 10.1056/NEJMoa1815039 Epub 2019 Apr 10. 2.Lambert N, El Moussaoui M, Maquet P: Immune checkpoint inhibitors for progressive multifocal leukoencephalopathy: Identifying relevant outcome factors.Eur J Neurol2021 28 (11):3814–3819, 2021. doi: 10.1111/ene.15021 Epub 2021 Jul 26. 