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Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system caused by the John Cunningham (JC) virus typically seen in immuno-compromised patients. Several drugs that suppress that immune system have already been known to cause PML such as natalizumab and rituximab. We present a patient with sarcoidosis who develops PML in the rare setting of minimal immunosuppression with only hydroxychloroquine. There was significant delay in the diagnosis due to negative cerebrospinal fluid testing for JC virus and concern for neuro-sarcoidosis, but eventually a diagnosis of PML was made via brain biopsy.

neuroimmunolgy and neuroinflammation

Progressive multifocal leukoencephalitis in a patient with sarcoidosis on hydroxychloroquine with negative cerebrospinal fluid testing for the John Cunningham virus