Remission of Progressive Multifocal Leukoencephalopathy Following Splenectomy and Antiretroviral Therapy in a Patient with HIV Infection

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Abstract
Progressive multifocal leukoencephalopathy caused by JC papovavirus infection of glial cells is an increasingly frequent complication of human immunodeficiency virus (HIV) type 1 infection. To date, there is no treatment available for progressive multifocal leukoencephalopathy, and it usually results in death within three to six months of diagnosis.1 We report on a 30-year-old man with hemophilia and AIDS (CD4 count, 64 cells per cubic millimeter) who was being treated with d4T and Septra and who came to our clinic with a two-week history of headache, diplopia, and left-arm ataxia. The patient had a normal mental status (HIV dementia scale, 14 of 16), left-sided gaze-evoked nystagmus and right beating nystagmus in the primary gaze position, and a marked intention tremor with slowed rapid alternating movements on the left side. A magnetic resonance imaging (MRI) scan revealed a nonenhancing lesion in the left cerebellar hemisphere without mass effect ( Figure 1A ). Brain biopsy of the lesion showed oligodendrocytes with enlarged nuclei ( Figure 2 ), infiltrating macrophages ( Figure 2 ), and lymphocytes on histologic analysis; JC virus inclusions were identified by electron microscopy. Because of previous reports suggesting that splenectomy increased CD4 levels in HIV infection2,3 and reduced viral load in simian immunodeficiency virus infection,4 the patient underwent a complete splenectomy three weeks after presentation. The patient's initial HIV viral load was 9.6×105 copies per milliliter. After splenectomy, the patient was also treated with saquinavir, zidovudine, and lamivudine. Three weeks after the splenectomy, the HIV viral load decreased to 1.1×105 copies per milliliter. The patient's tremor and ataxia in the left arm improved markedly without evidence of nystagmus. The patient continued to do well, and at the seven-month follow-up, his HIV viral load was 6×103 copies per milliliter. A repeated MRI scan revealed only scarring from the biopsy and marked reduction in the left cerebellar lesion ( Figure 1B ).