Progressive multifocal leukoencephalopathy successfully treated with Cidofovir in a patient with pulmonary sarcoidosis

We report the clinical course of a 68 year-old patient who developed symptoms of the parieto-occipital cortex. Homonymous hemianopia and brachiofacial paresis on the right, mnestic aphasia and disturbances in reading, writing, and calculating were slowly progressive over 4 months. MRI scans of the brain were consistent with progressive multifocal leukoencephalopathy (PML), the diagnosis of which could be ensured by DNA of JC virus (JCV) in the cerebrospinal fluid (CSF) by polymerase chain reaction.

In the medical history mild pulmonary sarcoidosis was known, but there was no need for treatment with immunosuppressive drugs. HIV screenig was negativ.

Cidofovir therapy was given according to the producers recommendation for one year and probenecid was added for nephroprotection. Frequency of intravenous application was once a week at the beginning for two weeks, three cycles once every two weeks, four cycles monthly and the last application after two month period. Beside of monocular pupil deformation following iritis no serious side effects could be observed, particularly no nephrotoxicity. Clinical symptoms and neuroradiological findings did stop progression and ameliorated over the follow-up period of 4 months. After 4 weeks JC virus DNA could not be detected anymore in CSF.

This is the first case of PML in untreated mild sarcoidosis and without other underlying immunopathology. Treatment with cidofovir for one year was successful regarding clinical symptoms, JCV in CSF, and neuroradiological findings in MRI.