Manifestations of central nervous system (CNS) infection by the polyomavirus, JC virus (JCV), comprise progressive multifocal leukoencephalopathy (PML), its infl ammatory form ( ' encephalitis ' ), JC virus infection of cerebellar granule cell neurons, and JC virus meningitis [1]. Th ese occur mainly in immunocompromised individuals, and are also associated with therapies including rituximab [1 -3].A 51-year-old man with an uneventful medical history complained of dizziness, headache, and mild left-sided weakness, 5 weeks before his admission. Brain magnetic resonance imaging (MRI) scans showed confl uent high signal intensity in the white matter of the right parietal lobe in T2-weighted and fl uid attenuated inversion recovery (FLAIR) images. Serological examination was negative for human immunodefi ciency virus (HIV), Epstein -Barr virus (EBV), cytomegalovirus (CMV), hepatitis C virus (HCV), hepatitis B surface antigen (HBsAg), human herpes virus 8 (HHV8), Borrelia , and human T-lymphotropic virus types I and II (HTLV I and II). Cerebrospinal fl uid (CSF) protein was elevated (75 mg/dL), and 2 white blood cells/ μ L were detected. Th ere were no oligoclonal bands. Screening for autoantibodies (antinuclear antibody [ANA], centromere, proliferating cell nuclear antigen [PCNA], ribosomal, cytoplasmic, cytoskeleton, chromatin, SSA/Ro, SSB/La, Scl-70, Jo-1, Sm/RNP, SM, histone, ds-DNA, nucleosome, reticulin, collagen, mitochondria) and polymerase chain reaction (PCR) examinations of the CSF for HIV, JCV, herpes simplex virus (HSV)-1, -2, and -6, varicella zoster virus (VZV), EBV, CMV, and Toxoplasma were negative. Abdominal and pelvic computed tomography (CT) scans revealed enlarged paraaortic lymph nodes with splenomegaly and mild hepatomegaly. Chest CT showed an enlarged lymph node in the upper mediastinum. Bone marrow histology, immunohistology, cytology, fl ow cytometry (B-cells 6.7%), and cytogenetics were normal, although a slight decrease in the CD4/CD8 ratio was noted (0.72).One month later, left-sided hemiplegia, homonymous hemianopia, and subdominant hemisphere symptoms developed. A control MRI scan showed progression of the white matter lesion [ Figure 1(A)] with peripheral gadolinium enhancement [Figures 1(B) and 1(C)]. In MR spectroscopy, the N -acetylaspartate and creatinine levels were decreased, the choline level was slightly elevated, and the lipid and lactate levels were highly elevated. While the blood cell count was normal, the β 2 -microglobulin level was elevated. Splenectomy was carried out for diagnostic purposes to exclude a lymphoma; however, detailed histological examination revealed only slight follicular hyperplasia. After splenectomy, he was treated with high-dose corticosteroids. Flow cytometry of peripheral blood revealed B-cell loss ( Ͻ 1%) and a further decrease of the CD4/CD8 cell ratio (0.49). Kappa/lambda chains were normal. Genetic analyses of T-cell receptors and heavy-chain immunoglobulins were normal.Five weeks after splenectomy there was a further drop of the CD4/CD8 ratio (...