Pediatric Neuromyelitis Optica (NMO). Demographic and Clinical Features of NMO in Children

Introduction: Neuromyelitis optica (NMO) is a severe, inflammatory CNS demyelinating disorder. NMO is characterized by sever optic neuritis (ON) and transverse myelitis (TM), occurring either simultaneously or sequentially. Incidence of NMO in children is unknown, criteria for pediatric neuromyelitis are: 1- MRI evidence of a contiguous spinal lesion at least three spinal segments length, 2- Brain MRI not meating diagnostic criteria for MS, 3- NMO- IgG seropositivity. Pediatric neuromyelitis optica has a diverse clinical presentation. The international panel suggested that adult criteria of neuromyelitis optica spectrum disorders (NMOSD) also are appropriate in pediatric NMO.Methods: Review of prospective multicenter database on children with demyelinating disease by US network pediatric MS canters 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 (ADEM), NMO and recurrent demyelinating disease not otherwise specified. Results: Thirty- eight cases of NMO were identify by review panel, 97% of which met the revised international panel on NMO diagnosis criteria, serum or CSF NMO immunoglobulin G (IgG) was positive in 65% of NMO cases. No patient had positive CSF NMO IgG and negative serum NMO IgG in contemporaneous samples. NMO IgG seropositive patients did not differ in demographic, clinical or laboratory features from seronegetives. 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 with NMO had higher attack rates, greater disability accrual measured by overall expanded disability status scale score (EDSS), and visual scores than did patients with MS. Conclusion: The new criteria for NMO spectrum disorders apply well to the pediatric setting and giver 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. Early diagnosis and immune suppressive treatment help to the slow the accumulation of several disability.

Keywords:

Neuromyelitis optica , Pediatric