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Tumefactive demyelinating disease treated with decompressive craniectomy
Author(s) -
Nilsson P.,
Larsson E.M.,
Kahlon B.,
Nordström C.H.,
Norrving B.
Publication year - 2009
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/j.1468-1331.2009.02547.x
Subject(s) - medicine , plasmapheresis , decompressive craniectomy , demyelinating disease , abnormality , disease , surgery , neurology , neuroimaging , differential diagnosis , radiology , traumatic brain injury , pathology , antibody , psychiatry , immunology
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.