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.

Original publication

DOI

10.1136/practneurol-2015-001146

Type

Journal article

Journal

Pract Neurol

Publication Date

12/2015

Volume

15

Pages

451 - 455

Keywords

demyelination, germ cell cancer, paraneoplastic syndromes, nervous system, seminoma, tumefactive, Antigens, CD, Brain, Brain Neoplasms, Demyelinating Diseases, Glial Fibrillary Acidic Protein, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Neoplasms, Germ Cell and Embryonal, Paraneoplastic Syndromes, Visual Fields