Unusual and Dramatic Presentation of “Adem”: What Could Be Done in Neurosurgical Practice?

Abstract

The authors describe the case of a young patient who presented with new onset of acute disseminated encephalomyelitis responsible for progressive unilateral brain edema causing trans-tentorial herniation. The patient underwent urgent standard right hemispheric decompressive craniectomy; unfortunately this was not successful and the patient had rapid neurological demise.

Fulminant demyelinating disease of the central nervous system effectively treated with a combination of decompressive craniectomy and immunotherapy: A case report and literature review

“…The reports of fulminant demyelinating disease of the CNS requiring decompressive craniectomy are summarized in Table 1. So far, 17 cases have been reported (including the present case), with 8 cases of ADEM, 7–13 4 cases of AHL, 14–17 3 cases of TDLs, 18–20 and 2 cases of the Marburg variant of MS 21,22 . The age of onset ranged widely from 1 to 63 years, but 16 of 17 cases were in their 50s or younger, and 4 were children.…”