title: Clinical features of neuromyelitis optica in children: US Network of Pediatric MS Centers report.
abstract: OBJECTIVE
  To compare clinical features of pediatric neuromyelitis optica (NMO) to other pediatric demyelinating diseases.
  
  
  METHODS
  Review of a prospective multicenter database on children with demyelinating diseases. Case summaries documenting clinical and laboratory features were reviewed by an adjudication panel. Diagnoses were assigned in the following categories: multiple sclerosis (MS), acute disseminated encephalomyelitis, NMO, and recurrent demyelinating disease not otherwise specified.
  
  
  RESULTS
  Thirty-eight cases of NMO were identified by review panel, 97% of which met the revised International Panel on NMO Diagnosis NMO-SD 2014 criteria, but only 49% met 2006 Wingerchuk criteria. Serum or CSF NMO immunoglobulin G (IgG) was positive in 65% of NMO cases that were tested; however, some patients became seropositive more than 3 years after onset despite serial testing. No patient had positive CSF NMO IgG and negative serum NMO IgG in contemporaneous samples. Other than race (p = 0.02) and borderline findings for sex (p = 0.07), NMO IgG seropositive patients did not differ in demographic, clinical, or laboratory features from seronegatives. Visual, motor, and constitutional symptoms (including vomiting, fever, and seizures) were the most common presenting features of NMO. Initiation of disease-modifying treatment was delayed in NMO vs MS. Two years after onset, patients with NMO had higher attack rates, greater disability accrual measured by overall Expanded Disability Status Scale score, and visual scores than did patients with MS.
  
  
  CONCLUSIONS
  The new criteria for NMO spectrum disorders apply well to the pediatric setting, and given significant delay in treatment of NMO compared to pediatric MS and worse short-term outcomes, it is imperative to apply these to improve access to treatment.
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA. tchitnis@rics.bwh.harvard.edu.
authors.forename: Tanuja
authors.initials: T
authors.lastname: Chitnis
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: Jayne
authors.initials: J
authors.lastname: Ness
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: Lauren
authors.initials: L
authors.lastname: Krupp
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Waubant
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: Tyler
authors.initials: T
authors.lastname: Hunt
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: Cody S
authors.initials: CS
authors.lastname: Olsen
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: Moses
authors.initials: M
authors.lastname: Rodriguez
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: Tim
authors.initials: T
authors.lastname: Lotze
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: Mark
authors.initials: M
authors.lastname: Gorman
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: Leslie
authors.initials: L
authors.lastname: Benson
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: Anita
authors.initials: A
authors.lastname: Belman
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: Bianca
authors.initials: B
authors.lastname: Weinstock-Guttman
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: Greg
authors.initials: G
authors.lastname: Aaen
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Graves
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: Marc
authors.initials: M
authors.lastname: Patterson
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: John W
authors.initials: JW
authors.lastname: Rose
authors.affiliation: From Partners Multiple Sclerosis Center (T.C.), Department of Neurology, Brigham and Women's Hospital, Brookline; Partners Pediatric Multiple Sclerosis Center (T.C.), Massachusetts General Hospital for Children, Boston; University of Alabama Center for Pediatric Onset Demyelinating Disease (J.N.), Children's Hospital of Alabama, Birmingham; the Lourie Center for Pediatric MS, Stony Brook Children's Hospital (L.K., A.B.), New York, NY; the Department of Neurology (E.W., J.G.) and the Department of Pediatrics, Benioff Children's Hospital (E.W.), University of California, San Francisco; the Departments of Pediatrics (T.H., C.S.O., T.C.C.) and Neurology (J.W.R.), University of Utah, Salt Lake City; Mayo Clinic's Pediatric MS Center (M.R., M.P.), Rochester, MN; Blue Bird Circle Multiple Sclerosis Center (T.L.), Baylor College of Medicine, Houston, TX; Boston Children's Hospital (M.G., L.B.), MA; the Pediatric Multiple Sclerosis Center (B.W.-G.), Jacobs Neurological Institute, SUNY Buffalo, New York, NY; and Pediatric MS Center at Loma Linda University Children's Hospital (G.A.), CA.
authors.forename: T Charles
authors.initials: TC
authors.lastname: Casper
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D016208:Databases, Factual; D020278:Demyelinating Autoimmune Diseases, CNS / Q000097:blood / Q000134:cerebrospinal fluid / Q000503:physiopathology; D018450:Disease Progression; D004673:Encephalomyelitis, Acute Disseminated / Q000097:blood / Q000134:cerebrospinal fluid / Q000503:physiopathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D007223:Infant; D008297:Male; D009103:Multiple Sclerosis / Q000097:blood / Q000134:cerebrospinal fluid / Q000503:physiopathology; D009471:Neuromyelitis Optica / Q000097:blood / Q000134:cerebrospinal fluid / Q000503:physiopathology; D012720:Severity of Illness Index
chemical_list: D007074:Immunoglobulin G