Progressive multifocal leukoencephalopathy in a kidney transplant recipient - case report and review of the literature

Abstract

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system caused by the JC virus (JCV). Most cases have been reported in severely immunocompromised patients, including transplanted patients. Lesions in the white matter are the cause of neurological dysfunction. Variable neurological and psychiatric symptoms may be observed at the time of presentation. The aim of this paper is to present a case of a 58 year old female recipient of an unrelated living kidney graft, who developed PML 47 months after transplantation and a review of current literature. At the time of transplantation patient received anti-thymocyte globulin induction (ATG, Fresenius) and was then maintained on a triple immunosuppressive regimen (mycophenolate mofetil, tacrolimus and prednisolone). She presented with an abrupt onset of diplopia, vertigo and paraesthesia of the right side. MRI revealed widespread lesions in the white matter. Marked hyperproteinaemia, hypergammaglobulinemia and lymphocytosis was found in cerebrospinal fluid. Patient’s neurological status deteriorated over following two months. Suspicion of PML was raised and the dosages of mycophenolate mofetil and tacrolimus were considerably reduced. Most of the symptoms abated and 12 months later the patient complained only of occasional headaches. Repeated MRI disclosed near complete resolution of white matter lesions. Graft function temporarily worsened but then stabilized. Conclusions: 1. PML should be considered in differential diagnosis of psychiatric and neurological symptoms in transplant recipients. 2. Minimizing of immunosuppressive therapy may lead to regression of this potentially fatal disease