Progressive multifocal encephalopathy unmasked by ...

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Progressive multifocal encephalopathy unmasked by HAART in a ... case with diagnosis of progressive multifocal leukoencephalopathy (PML), a subacute.

Progressive multifocal encephalopathy unmasked by HAART in a HIV-infected patient 在免疫缺陷病毒感染病患使用高效抗逆轉錄病毒治療後表現進行性多發性腦白質病變 Han-Tao Li, Chung-Yang  Sung, Kuo-Hsuan Chang Department of Neurology, Chang Gung Memorial Hospital, Linkou, Taiwan 李翰道 宋崇元 張國軒 林口長庚紀念醫院 神經內科 Background For patients with HIV/AIDS, highly active antiretroviral therapy (HAART) is currently the only effective therapy [1]. Here we report a case with diagnosis of progressive multifocal leukoencephalopathy (PML), a subacute demyelinating disease caused by infection of oligodendrocytes by the polyomavirus JC [2], occurs after HAART.

Case Report A 52-year-old HAART-treated HIV-1-infected male patient began to develop progressive cognitive impairment, disorientation, and right limb weakness for about two months before admission. His neurological examinations showed motor aphasia, acalculia, and ideomotor apraxia. Hemiplegia, hyperreflexia and present Babinski sign were noted on his right limbs.

His brain magnetic resonance imaging (MRI) showed T2-weighted hyperintense lesions in left frontal white matter and callososeptal area (Fig.1). Laboratory tests found low CD4 T-cell counts of 433 cells/µL and high HIV-RNA viral load of 43,292 copies/mL. The stereotatic brain biopsy showed inflammatory infiltrations, gliosis with focal demyelination, and intranuclear inclusion positive for anti-JC virus antibody (Fig.2), which was compatible with PML. His neurological deficits became stable following a short-term treatment with dexamethasone and mannitol. And then his HAART with combivir and efavirenz was restarted.

Conclusion PML could occur in HIV-infected patients following HARRT. The mechanism for the reactivation of JC virus by immune reconstitution remains elusive.

Figure 2. Immunohistochemistry study showed JC-virus positive Intranuclear inclusions.

Figure 1. Brian MRI of the patient. A mass-like lesion with rim enhancement and central necrosis is located over left frontal lobe, measuring 5.5 x 2.9 x 4.6 cm in size. Multifocal perpendicular calloso-septal enhancing lesions are revealed after contrast injection.

References 1. Optimal time for initiation of antiretroviral therapy in asymptomatic, HIV-infected, treatment-naive adults (Review). Cochrane Database of Systematic Reviews 2010;3. 2. Progressive multifocal leukoencephalopathy and other disorders caused by JC virus: clinical features and pathogenesis. Lancet Neurol 2010;9:425-37.

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