title: Partial recovery after severe immune reconstitution inflammatory syndrome in a multiple sclerosis patient with progressive multifocal leukoencephalopathy.
abstract: Progressive multifocal leukoencephalopathy (PML) is a rare and severe complication of natalizumab therapy in patients with multiple sclerosis and it may be accompanied by immune reconstitution inflammatory syndrome (IRIS). Here, we describe a case of abnormally severe IRIS, which occurred 2 months after natalizumab-associated PML in a 38-year-old woman affected by multiple sclerosis. The patient was John Cunningham virus-positive and was treated for 21 months when she developed PML. The subsequent IRIS diffusely afflicted the brain, producing edema and signs of intracranial hypertension, with a clinically severe form compromising the state of consciousness, requiring intensive care and high-dosage steroid treatment. Nevertheless, she survived and partially recovered. There is still difficulty in differentiating PML progression from IRIS onset and there is not a clear description in the literature about different clinical forms of IRIS, prognostic factors and guidelines to properly treat this complication in order to reduce the residual disability of the patient surviving this treatment complication.
authors.affiliation: Neurology Unit, Department of Pathophysiology & Transplantation, 'Dino Ferrari' Center, University of Milan, Fondazione Cà Granda, IRCCS Ospedale Maggiore Policlinico, Milan, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Calvi
authors.forename: Milena
authors.initials: M
authors.lastname: De Riz
authors.forename: Anna M
authors.initials: AM
authors.lastname: Pietroboni
authors.forename: Laura
authors.initials: L
authors.lastname: Ghezzi
authors.forename: Virginia
authors.initials: V
authors.lastname: Maltese
authors.forename: Andrea
authors.initials: A
authors.lastname: Arighi
authors.forename: Giorgio G
authors.initials: GG
authors.lastname: Fumagalli
authors.forename: Francesca
authors.initials: F
authors.lastname: Jacini
authors.forename: Carlotta
authors.initials: C
authors.lastname: Donelli
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Comi
authors.forename: Daniela
authors.initials: D
authors.lastname: Galimberti
authors.forename: Elio
authors.initials: E
authors.lastname: Scarpini
mesh_terms: D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage / Q000009:adverse effects; D001921:Brain / Q000187:drug effects* / Q000473:pathology / Q000821:virology; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D054019:Immune Reconstitution Inflammatory Syndrome / Q000188:drug therapy* / Q000209:etiology; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects; D007577:JC Virus / Q000502:physiology*; D007806:Language Disorders; D007968:Leukoencephalopathy, Progressive Multifocal / Q000188:drug therapy* / Q000209:etiology; D009103:Multiple Sclerosis / Q000150:complications / Q000188:drug therapy*; D000069442:Natalizumab; D010951:Plasma Exchange; D020127:Recovery of Function; D013256:Steroids / Q000008:administration & dosage; D019562:Viral Load / Q000187:drug effects
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D007166:Immunosuppressive Agents; D000069442:Natalizumab; D013256:Steroids