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Tumefactive demyelinating disease treated with decompressive craniectomy.

Nilsson, Petra LU ; Larsson, Elna-Marie LU ; Kahlon, Babar LU ; Nordström, Carl-Henrik LU and Norrving, Bo LU (2009) In European Journal of Neurology 16. p.639-642
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.... (More)
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. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
European Journal of Neurology
volume
16
pages
639 - 642
publisher
Wiley-Blackwell
external identifiers
  • wos:000264881400023
  • pmid:19309337
  • scopus:64149125586
ISSN
1351-5101
DOI
10.1111/j.1468-1331.2009.02547.x
language
English
LU publication?
yes
id
bc4d01b5-1b81-4fa1-96d6-daccb4fdff29 (old id 1367482)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/19309337?dopt=Abstract
date added to LUP
2009-04-06 15:23:50
date last changed
2017-08-06 04:44:50
@article{bc4d01b5-1b81-4fa1-96d6-daccb4fdff29,
  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.},
  author       = {Nilsson, Petra and Larsson, Elna-Marie and Kahlon, Babar and Nordström, Carl-Henrik and Norrving, Bo},
  issn         = {1351-5101},
  language     = {eng},
  pages        = {639--642},
  publisher    = {Wiley-Blackwell},
  series       = {European Journal of Neurology},
  title        = {Tumefactive demyelinating disease treated with decompressive craniectomy.},
  url          = {http://dx.doi.org/10.1111/j.1468-1331.2009.02547.x},
  volume       = {16},
  year         = {2009},
}