Pediatric Neuromyelitis Optica Spectrum Disorder: Case Series and Literature Review

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.

Area Postrema Syndrome as the Onset of Neuromyelitis Optica in a Pediatric Patient: Case Report

“…If the initial response to steroids is insufficient, plasmapheresis or immunoglobulin therapy should be considered. 16 In our case, a stepped therapy approach using immunoglobulin and steroids was performed, resulting in the complete resolution of symptoms. For NMOSD relapse prevention, immunomodulators like rituximab, mycophenolate mofetil, and azathioprine are key, effectively lowering relapse frequency and severity.…”

Longitudinally extensive transverse myelitis, a disabling disorder with a good prognosis: a case series from Nepal

“…Noninflammatory causes have specific treatments like antimicrobial therapy for syphilis, neuroborreliosis, parasitic infections, surgery for dural fistula, and treatment of vitamin and mineral deficiency whereas, for inflammatory etiologies, management can be divided into acute and prophylactic treatment where acute treatment mainly involves immunosuppressive agents or plasma exchange therapy depending upon the severity of disease and risk of relapse 15 . In NMOSD, at least 5 years of maintenance therapy is required 17 . In our cases all three acute cases were treated with intravenous methyl prednisone and all four cases were given maintenance therapy with mycophenolate mofetil.…”

Acute gastroenteritis and unilateral vision loss leading to a diagnosis of aquaporin-4-IgG seropositive neuromyelitis optica spectrum of disorders in a child: A case of atypical optic neuritis in the era of biomarkers

“…[2] Paediatric optic neuritis accounts for 25% of acute demyelinating syndromes in this age group. [3,4] Post-infectious (usually viral) or postimmunisation aetiologies usually predominate. [5] Less commonly, it may be the first manifestation of multiple sclerosis (MS) or other diffuse demyelinating disorders such as acute demyelinating encephalomyelitis (ADEM), NMOSD and myelin oligodendrocyte glycoprotein-associated disease (MOGAD).…”