Pediatric Neuromyelitis Optica Spectrum Disorder: Case Series and Literature Review

Objective: NMOSD is a central nervous system (CNS) inflammatory demyelinating disease characterized by recurrent inflammatory events that primarily involve optic nerves and the spinal cord, but also affect other regions of the CNS, including hypothalamus, area postrema and periaqueductal gray matter. The aquaporin-4 antibody (AQP4-IgG) is specific for NMOSD. Recently, myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) have been found in a group of AQP4-IgG negative patients. NMOSD is rare among children and adolescents, but early diagnosis is important to start adequate therapy. Methods: In this report, we present cases of seven pediatric patients with NMOSD and we review the clinical and neuroimaging characteristics, diagnosis, and treatment of NMOSD in children. Results: This is a narrative review in which we have included original studies and case reports exclusively on pediatric patients with NMOSD. After the initial identification of 757 papers, we selected for the final analysis 41 manuscripts. Conclusions: We have reviewed recent literature to provide a tool for diagnosing and treating children with NMOSD. These case reports are an example of the diagnostic and therapeutic complexity of pediatric NMOSD. Our cases, although limited in number, offer a wide range of personalized therapeutic strategies for the individual patient. Furthermore, our series also discusses AQP4-negative patients, unlike the other reviews that focus on AQP4-positive pediatric patients.