1 Department of Clinical Medicine, Department of Clinical Medicine, Faculty of Health and Medical Sciences, Københavns Universitet2 unknown3 Department of Clinical Medicine, Department of Clinical Medicine, Faculty of Health and Medical Sciences, Københavns Universitet
BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a rare, demyelinating disease caused by viral infection of glial cells by JC polyomavirus (JCV) in immunocompromised patients. JCV is a member of the Polyomaviridae family. Infection is usually latent and reactivation only occurs in circumstances of extreme immunodeficiency. Development of fulminant PML is rare and treatment options are limited. CASE REPORT: We have presented a case of JCV reactivation resulting in PML 19 years after renal allograft transplantation and after recent conversion of immunosuppressive treatment. One year after conversion of immunosuppressive therapy owing to biopsy-proven acute humoral rejection, our patient presented with symptoms of deteriorating neurologic status. Cerebral computed tomography showed abnormal signals in the frontal lobe suspect for PML. Diagnosis was confirmed by qualitative polymerase chain reaction analysis of the cerebrospinal fluid. Owing to severe renal insufficiency, treatment options were limited to tapering of immunosuppressive treatment in hopes of achieving host clearance of the viral infection. Despite prompt termination of immunosuppressive treatment, the patient suffered rapid progressive neurologic decline and death rapidly ensued. CONCLUSION: Development of PML in transplant recipients remains rare. Despite advances in our understanding of JCV infection and PML, treatment options remain limited and prognosis is often poor.