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Medical Response

There are no known interventions that can reliably prevent PML or adequately treat PML if it occurs. It is believed that the best prognosis for PML is afforded by reconstitution of the immune system leading to viral clearance.1 Where possible, this may be achieved through the discontinuation or removal of immunosuppressant agents, or in the case of patients with HIV, initiation of highly active anti-retroviral therapy (HAART).2 For some immunosuppressants, plasma exchange (PLEX) may be an effective means of rapidly removing drug from the circulation.3 There are, however, currently no proven effective anti-viral therapies, or any other therapies. Anti-infective agents such as cidofovir and mefloquine have been used in an attempt to treat PML; however, despite limited case reports of efficacy, small clinical trials have not demonstrated efficacy.4

 

In patients exhibiting successful immune reconstitution, reduction of viral load in CSF is often accompanied by immune reconstitution inflammatory syndrome (IRIS), leading to a significant decline in a patient’s condition. Features of IRIS include significant clinical worsening and gadolinium enhancement or edema on MRI and can lead to death or severe disability. Clinical vigilance, including repeated brain MRI, is therefore critical in patients exhibiting immune reconstitution. Recent observations suggest that corticosteroid use may be beneficial for the treatment of IRIS.

 

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1 Bruce Brew, Nicholas Davies, et. al. Progressive multifocal leukoencephalopathy and other forms of JC virus disease. Nat Rev Neurol. 2010 Dec;6(12): 674.

2 Thomas Weber. Progressive multifocal leukoencephalopathy. Neurol Clin. 2008 Aug;26(3):833-54, x-xi and Brew, 668. For information on specific therapeutics, please refer to the manufacturers' websites.

3 Khatri BO, Man S, Giovannoni G, Koo AP, Lee JC, Tucky B, Lynn F, Jurgensen S, Woodworth J, Goelz S, Duda PW, Panzara MA, Ransohoff RM, Fox RJ. Effect of plasma exchange in accelerating natalizumab clearance and restoring leukocyte function. Neurology. 2009;72:402-9.

4 Brew, 675.