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Neurology. 2016 Jan 19;86(3):245-52. doi: 10.1212/WNL.0000000000002283. Epub 2015 Dec 18.

Clinical features of neuromyelitis optica in children: US Network of Pediatric MS Centers report.

Author information

1
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.
2
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.

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.

CONCLUSION:

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.

PMID:
26683648
PMCID:
PMC4733158
DOI:
10.1212/WNL.0000000000002283
[Indexed for MEDLINE]
Free PMC Article

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