Pediatric Neuromyelitis Optica Spectrum Disorders: Three Case Reports and Review of Literature

Abstract

Introduction: Neuromyelitis optica spectrum disease (NMOSD) is a rare autoimmune inflammatory astrocytopathic demyelinating disease of the CNS. In the majority of patients, an autoantibody to the water channel protein aquaporin-4 (AQP4) would cause humoral inflammatory demyelination and axonal damage. The disease defined as clinical syndromes of optic nerve, area postrema, spinal cord, diencephalon, or cerebrum or MRI finding related to involvement of these regions. According to recent consensus diagnostic criteria, NMOSD categorized as NMOSD with AQP4 Ab, Also NMOSD without AQP4 Ab. Early onset is defined as the disease presentation before 17 years old. According to the pediatric NMOSD working group most clinical, laboratory characteristics and neuroimaging of this disease are the same as adult onset, and same criteria could be used for pediatric NMOSD. Differentiation of NMO from MS and acute disseminated encephalomyelitis (ADEM) are matters of importance in decision making in order to choose an appropriate therapy. It has also been reported that some MS therapies could aggravate NMO exacerbations and lead to permanent disability. Case Presentations: Three patients under the age of 17 years old were present in neurology clinic, due to their recurrent optic neuritis and cervical myelopathy. AQP4 Ab was positive in two patients, and the other patient was seronegative for AQP4 Ab. according to the recent international consensus definition. All of the patients went under treatment with corticosteroid pulse therapy in acute relapses, and two of them were getting azathioprine as a disease modifying therapy, and the other patient went under rituximab, because of new relapses in spite of azathioprine therapy. Azathioprine and rituximab were safe and tolerated well without any significant side effects in all of them. Conclusions: Pediatric NMOSD is a rare but life threatening disease, which pediatrician and pediatric neurologists must be aware of its presentations and treatment.

[Retracted] Correlation of IgH‐CDR3 Immune Repertoire Diversity Test in Peripheral Blood of Neuromyelitis Spectrum Diseases

“…They studied the latest research progress on the role of microglia in the pathogenesis of NMOSD, the mechanisms of microglia activation and microglia-mediated inflammation, and potential research prospects [ 8 ]. Research by Baghbanian et al showed that autoantibodies to the aquaporin 4 (AQP4) caused humoral inflammatory demyelination and axonal damage [ 9 ]. Zhang et al collected data on 40 patients with CTD LETM admitted to the Department of Neurology or Rheumatology, divided into two groups: CTD-LETM-NMOSD and CTD-LETM-non-NMOSD.…”