PMID- 26115897 OWN - NLM STAT- MEDLINE DCOM- 20160513 LR - 20181202 IS - 1532-2653 (Electronic) IS - 0967-5868 (Linking) VI - 22 IP - 10 DP - 2015 Oct TI - Surgical management of malignant cerebral edema secondary to immune reconstitution inflammatory syndrome from natalizumab-associated progressive multifocal encephalopathy. PG - 1669-71 LID - S0967-5868(15)00230-1 [pii] LID - 10.1016/j.jocn.2015.05.003 [doi] AB - We report a rare multiple sclerosis (MS) patient who developed malignant cerebral edema related to progressive multifocal encephalopathy (PML) immune reconstitution inflammatory syndrome (IRIS) after natalizumab discontinuation. The patient subsequently required a decompressive hemicraniectomy to reduce intracranial pressure and to avoid uncal herniation. PML is a demyelinating disease of the central nervous system (CNS) which affects oligodendrocytes and is caused by reactivation of latent John Cunningham virus. Natalizumab is a known risk factor (1 in 1000) for MS patients treated with this drug. Discontinuation of natalizumab treatment decreases the risk of PML progression, but a massive inflammatory response can occur after cell-mediated immune surveillance is reestablished in the CNS, causing immune reconstitution inflammatory syndrome (IRIS). Treatment of IRIS usually consists of steroids and plasma exchange to lessen the immune response, however, mortality has been reported at up to 29.4%, despite aggressive medical treatment. We discuss our management strategy with a review of the pertinent literature. CI - Copyright (c) 2015 Elsevier Ltd. All rights reserved. FAU - Tan, Lee A AU - Tan LA AD - Department of Neurosurgery, Rush University Medical Center, 1725 West Harrison Street, Chicago, IL 60612, USA. Electronic address: lee_tan@rush.edu. FAU - Lopes, Demetrius K AU - Lopes DK AD - Department of Neurosurgery, Rush University Medical Center, 1725 West Harrison Street, Chicago, IL 60612, USA. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20150624 PL - Scotland TA - J Clin Neurosci JT - Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia JID - 9433352 RN - 0 (Antibodies, Monoclonal, Humanized) RN - 0 (Natalizumab) SB - IM MH - Adult MH - Antibodies, Monoclonal, Humanized/adverse effects MH - Brain Edema/*diagnosis/etiology/*surgery MH - Disease Management MH - Female MH - Humans MH - Immune Reconstitution Inflammatory Syndrome/chemically induced/complications/*diagnosis MH - Leukoencephalopathy, Progressive Multifocal/chemically induced/complications/*diagnosis MH - Multiple Sclerosis/diagnosis/drug therapy MH - Natalizumab/*adverse effects OTO - NOTNLM OT - Hemicraniectomy OT - Immune reconstitution inflammatory syndrome OT - Malignant cerebral edema OT - Natalizumab OT - Progressive multifocal leukoencephalopathy EDAT- 2015/06/28 06:00 MHDA- 2016/05/14 06:00 CRDT- 2015/06/28 06:00 PHST- 2015/04/16 00:00 [received] PHST- 2015/05/01 00:00 [accepted] PHST- 2015/06/28 06:00 [entrez] PHST- 2015/06/28 06:00 [pubmed] PHST- 2016/05/14 06:00 [medline] AID - S0967-5868(15)00230-1 [pii] AID - 10.1016/j.jocn.2015.05.003 [doi] PST - ppublish SO - J Clin Neurosci. 2015 Oct;22(10):1669-71. doi: 10.1016/j.jocn.2015.05.003. Epub 2015 Jun 24.