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Paraneoplastic tumefactive demyelination with underlying combined germ cell cancer
  1. Jack R Broadfoot1,
  2. Hilary A Archer2,
  3. Elizabeth Coulthard2,
  4. Auke P A Appelman3,
  5. Judit Sutak4,
  6. Jeremy P Braybrooke5,
  7. Seth Love6
  1. 1Movement Disorder Service, Southmead Hospital, Bristol, UK
  2. 2Neurology Department, Southmead Hospital, Bristol, UK
  3. 3Neuroradiology Department, Southmead Hospital, Bristol, UK
  4. 4Pathology Department, Southmead Hospital, Bristol, UK
  5. 5Bristol Cancer Institute, University Hospitals, Bristol, UK
  6. 6Neuropathology Department, Southmead Hospital, Bristol, UK
  1. Correspondence to Dr Jack Richard Broadfoot, Movement Disorder Service, Southmead Hospital, Southmead Road, Bristol BS10 5NB, UK; jackbroadfoot1{at}doctors.org.uk

Abstract

Paraneoplastic demyelination is a rare disorder of the central nervous system. We describe a 60-year-old man with tumefactive demyelination who had an underlying retroperitoneal germ cell cancer. He presented with visuospatial problems and memory loss and had a visual field defect. His MRI was interpreted as a glioma but stereotactic biopsy showed active demyelination. Investigation for multiple sclerosis was negative but CT imaging showed retroperitoneal lymphadenopathy, and nodal biopsy confirmed a combined germ cell cancer. He responded poorly to corticosteroid treatment, and his visual field defect progressed. However, 6 months after plasma exchange and successful chemotherapy, he has partially improved clinically and radiographically. Tumefactive demyelination is typically associated with multiple sclerosis but may be paraneoplastic. It is important to recognise paraneoplastic tumefactive demyelination early, as the neurological outcome relies on treating the associated malignancy.

  • germ cell cancer
  • seminoma
  • paraneoplastic syndromes, nervous system
  • demyelination
  • tumefactive

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