Clinical features of neuromyelitis optica in children

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.

Child Neurology: Neuromyelitis optica spectrum disorders

“…In one analysis of children with NMOSD, the most common presenting features included visual, motor, and constitutional syndromes. 3 In the largest report of children with NMO, 83% and 78% of children with AQP4-IgG had at least one episode of ON or transverse myelitis, respectively, while 45% had other symptoms such as encephalopathy, seizures, ophthalmoparesis, ataxia, or area postrema syndrome. 4 Children with NMOSD were older than those with acute disseminated encephalomyelitis (ADEM) (mean 10-12 vs 5 years), but approximately the same age as those with MS (13 years).…”

“…4 Children with NMOSD were older than those with acute disseminated encephalomyelitis (ADEM) (mean 10-12 vs 5 years), but approximately the same age as those with MS (13 years). 3,4 Female patients and non-Caucasians are overrepresented in NMOSD. NMOSD is associated with additional autoimmunity, with 42% and 76% of patients with other autoimmune diagnoses or autoantibodies, respectively.…”

“…Roughly 65% of children with NMOSD tested positive for AQP4-IgG, rates similar to those observed in adults with NMOSD, but seropositivity may occur up to 4-5 years after onset. 3 Testing for AQP4-IgG is the most sensitive and cost-effective when performed on serum, which is the source recommended by the Mayo Clinic laboratory. 7 In ON from NMOSD, the CSF may appear bland, while during an episode of myelitis, CSF may be highly inflamed, with pleocytosis .100 cells/mL, commonly with neutrophils or eosinophils.…”

“…6 CSF studies in children with ADEM are variable and often nondiagnostic; however, oligoclonal bands are only rarely present. 3 Children with features of NMOSD but with negative serum AQP4-IgG should have CSF AQP4-IgG testing and may also be tested for myelin-oligodendrocyte glycoprotein antibodies (in the United States, only available through research laboratories). AQP4-IgG may appear up to 4 years after disease onset, and sequential testing should be employed for initially seronegative patients.…”

“…The Wingerchuk 2006 criteria were 49% sensitive for a diagnosis of pediatric NMO, while the 2015 updated international panel for NMO diagnosis criteria, which allowed for diagnosis after one attack and the presence of AQP4-IgG, were 97% sensitive and can be applied to children. 3 This is in part due to the lower lesion accrual in pediatric NMO.…”