Özet:

We report a case of 40-year-old female who is known to have systemic lupus erythematosus (SLE) presenting with severe cognitive impairment with lymphopenia, proteinuria, and evidence of SLE serological activity. Initially, she was managed as a case of neuropsychiatric systemic lupus erythematosus (NPSLE) with Cyclophosphamide and pulse steroids. However, she has been deteriorating clinically in forms of right sided hemiparesis, blindness, and aphasia despite normalization of complements and anti-double stranded DNA antibodies levels. Diagnosis of progressive multifocal leukoencephalopathy (PML) was made based on her clinical manifestations with brain MRI findings of subcortical white matter lesions and detection of John Cunningham virus (JCV) in cerebrospinal fluid analysis. Immunosuppressive agents were discontinued aiming for immune system restoration. 

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