title: Surgical management of malignant cerebral edema secondary to immune reconstitution inflammatory syndrome from natalizumab-associated progressive multifocal encephalopathy.
abstract: 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.
authors.affiliation: Department of Neurosurgery, Rush University Medical Center, 1725 West Harrison Street, Chicago, IL 60612, USA. Electronic address: lee_tan@rush.edu.
authors.forename: Lee A
authors.initials: LA
authors.lastname: Tan
authors.affiliation: Department of Neurosurgery, Rush University Medical Center, 1725 West Harrison Street, Chicago, IL 60612, USA.
authors.forename: Demetrius K
authors.initials: DK
authors.lastname: Lopes
mesh_terms: D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000009:adverse effects; D001929:Brain Edema / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery*; D019468:Disease Management; D005260:Female; D006801:Humans; D054019:Immune Reconstitution Inflammatory Syndrome / Q000139:chemically induced / Q000150:complications / Q000175:diagnosis*; D007968:Leukoencephalopathy, Progressive Multifocal / Q000139:chemically induced / Q000150:complications / Q000175:diagnosis*; D009103:Multiple Sclerosis / Q000175:diagnosis / Q000188:drug therapy; D000069442:Natalizumab / Q000009:adverse effects*
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D000069442:Natalizumab
keywords: Hemicraniectomy; Immune reconstitution inflammatory syndrome; Malignant cerebral edema; Natalizumab; Progressive multifocal leukoencephalopathy