Abstract
Neuromyelitis Optica Spectrum Disorder (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. 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.
Highlights
Neuromyelitis Optica Spectrum Disorder (NMOSD) is a rare central nervous system (CNS) inflammatory demyelinating disease characterized by recurrent inflammatory events, primarily involving optic nerves and the spinal cord, and affecting other regions of the CNS, including hypothalamus, area postrema and periaqueductal gray matter
At the time of last follow-up, 42 months after Hematopoietic Stem Cell Transplantation (HSCT), Magnetic resonance imaging (MRI) showed a significant reduction of preexisting lesions with no gadolinium enhancement in absence of new lesions
Other rare neurological presentations in NMOSD are represented by cerebral syndromes that are reported in 16–32% of AQP4-IgG positive children [8]
Summary
Neuromyelitis Optica Spectrum Disorder (NMOSD) is a rare central nervous system (CNS) inflammatory demyelinating disease characterized by recurrent inflammatory events, primarily involving optic nerves and the spinal cord, and affecting other regions of the CNS, including hypothalamus, area postrema and periaqueductal gray matter. Eosinophils are involved in the pathogenesis of lesions in NMOSD, and these cells have been shown to contribute to tissue damage [3]. AQP4-IgG seronegativity in 10–25% of NMOSD patients suggests that there are other mechanisms involved in NMOSD pathogenesis [4]. Myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) have been found in a group of AQP4-IgG negative patients, which have a different pathogenesis, younger age at presentation, fewer relapses, and a better outcome [5,6,7]. In this article we present cases of seven pediatric patients with NMOSD and we review the literature related to clinical and neuroimaging characteristics, diagnosis and treatment of pediatric NMOSD
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