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- Title
Tumefactive demyelinating disease treated with decompressive craniectomy.
- Authors
Nilsson, P.; Larsson, E.-M.; Kahlon, B.; Nordström, C.-H.; Norrving, B.
- Abstract
Background: Tumefactive demyelinating disease (TDD) is a rare primary demyelinating disease with diagnostic and therapeutic challenges. Methods and results: We report a 50-year old woman with TDD successfully treated with decompressive craniectomy and corticosteroids. The patient presented with seizures, subacute progressive hemispheric syndrome, and a tumourlike abnormality on MRI. Demyelinating disease was initially considered unlikely. Due to a rapidly evolving herniation syndrome hemicraniectomy was performed. Outcome was favourable with only very mild neurological deficits 6 weeks later. Conclusion: TDD should be considered as a differential diagnosis in tumour-like presentations, and appears to have distinctive neuroimaging features. In the advent of treatement failure from high dose corticosteroids and plasmapheresis and development of severe mass effect, decompressive hemicraniectomy is an important treatment option.
- Subjects
DEMYELINATION; DIAGNOSIS; ADRENOCORTICAL hormones; SPASMS; BRAIN imaging; THERAPEUTICS
- Publication
European Journal of Neurology, 2009, Vol 16, Issue 5, p639
- ISSN
1351-5101
- Publication type
Article
- DOI
10.1111/j.1468-1331.2009.02547.x