BMJ Case Reports 2017; doi:10.1136/bcr-2017-220990
  • Unusual presentation of more common disease/injury

Posterior fossa progressive multifocal leukoencephalopathy: first presentation of an unknown autoimmune disease

Open Access
  1. Bram Jacobs3
  1. 1Neurology, Medisch Spectrum Twente, Enschede, Netherlands
  2. 2Radiology, The Rotherham NHS Foundation Trust, Rotherham, UK
  3. 3Neurology, Universitair Medisch Centrum Groningen, Groningen, Netherlands
  1. Correspondence to Paulette Scholten, paulette_scholten{at}
  • Accepted 25 September 2017
  • Published 11 October 2017


We present a case of a 57-year-old man who presented with progressive cerebellar dysarthria and cerebellar ataxia. Additional investigations confirmed the diagnosis of progressive multifocal leukoencephalopathy (PML) in the posterior fossa. This is a demyelinating disease of the central nervous system, caused by an opportunistic infection with John Cunningham virus. PML has previously been considered a lethal condition, but because of careful monitoring of patients with HIV and of patients using immunosuppressive drugs it is discovered in earlier stages and prognosis can be improved. Our patient had no known immune-compromising state, but further work-up revealed that the PML was most likely the first presentation of a previous untreated autoimmune disorder: sarcoidosis.


  • Contributors PS: main contributor, manuscript concept and design. PK: contributor of the radiological data and conclusions, revision of the manuscript. BJ: supervisor, critical revision of the manuscript.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:

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