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A 68-year-old woman presented with 3 weeks of progressive left-sided weakness. She had a history of Sjögren’s syndrome, complicated by mononeuritis multiplex and arteritic anterior ischaemic optic neuropathy, and had taken azathioprine for 14 months. On examination, she was disorientated, inattentive, with a left hemiparesis and extensor left plantar response. MR scan of brain showed asymmetrical bilateral frontoparietal leukoencephalopathy, involving U-shaped fibres and deep white matter, with foci of diffusion restriction in the right frontoparietal and left parietal lobes, and a thin, linear, gyriform susceptibility weighted imaging (SWI)-hypointense rim in the periphery of the lesion (figure 1). Cerebrospinal fluid (CSF) examination showed 2 white cells/µL (≤5), glucose 5.6 mmol/L (2.5–4.4) and protein 0.44 g/L (0.15–0.45); Gram stain was negative. Quantitative DNA polymerase chain reaction (PCR) in CSF for JC (John Cunningham) polyomavirus was positive, supporting the diagnosis of progressive multifocal leukoencephalopathy (PML), an infective and life-threatening demyelinating disease of …
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Contributors VLB conceived of the pathway and project, wrote the first draft and submmited. PF and FPS executed and wrote the first draft. DBB and HBF provided expert advice and reviewed the manuscript. EMLdO supervised the project and reviewed the manuscript and gave expert opinions. All authors approved the final version of this manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests PF is an editorial board member of the neurology resident and fellow section. VLB, FPS, DBB, HBF and EMLdO declare that there are no conflicts of interest relevant to this work.
Provenance and peer review Not commissioned; externally peer reviewed by Aaron Berkowitz, San Francisco, USA.