Progressive multifocal leukoencephalopathy in the absence of immunosuppression

Abstract

A 69-year-old woman presented with a cortical hand syndrome progressing over several weeks. MRI brain showed characteristic appearances of progressive multifocal leukoencephalopathy (PML), confirmed by detection of the JC virus in CSF, despite the absence of any evidence of immunosuppression. Treatment with mirtazapine, mefloquine and cidofovir did not affect the progression of the disease, which was fatal within 7 months of presentation. This report adds to the small case literature that suggests that PML can occur in immunocompetent people, albeit extremely rarely.

Keywords: JC virus; Neurovirology; Progressive multifocal leukoencephalopathy.

Fig. 1

MR imaging of brain demonstrating multifocal areas of high signal on T2 (a, g) and flair (b, h) with restricted patchy and peripheral diffusion on the axial DWI sequence (c, i) matched on the ADC (d, j). T1 images showed low signal (e, k) and post-contrast (f, l) there was no enhancement. The imaging changes progressed over a one-month interval (a–f followed by g–l)