Decompressive craniectomy for acute disseminated encephalomyelitis.

Background: Acute disseminated encephalomyelitis (ADEM) is a rare, acute demyelinating condition. Although it usually presents in an acute or subacute manner over days, its clinical course may be rapid with symptoms and signs of severe intracerebral mass effect secondary to cerebral oedema. Methods: Case report and literature review.Results: We report a case of a patient presenting with a hyperacute course manifested by rapid loss of consciousness and focal neurological signs. Management with emergency hemicraniectomy and steroids resulted in rapid neurological improvement and minimal long-term deficit. Conclusions: We believe that only surgical decompression is likely to be life saving in similar cases of hyperacute cerebral oedema due to ADEM. The wide decompression performed was concordant with that indicated for traumatic brain swelling. Such aggressive management is vindicated by the rapid recovery shown by our patient within days of surgery and the finding of minimal neurological sequelae at 3 months.