Neuromyelitis optica in childhood: a case report

Abstract

Introduction: Neuromyelitis optica (NMO) is a relatively rare inflammatory disease that primarily affects the spinal cord and optic nerves. Objectives: To report an atypical case of NMO in the child, with brainstem syndrome. Case report: A 12-year-old patient, 20 days after Pfizer vaccination for severe acute respiratory syndrome coronavirus-19 (SARS-COV-19), started with asymmetrical ascending flaccid paraparesis, with global arreflexia, ataxia, dysphagia and dysarthria. On admission, after 2 months, thought of acute polyradiculoneuritis, immunoglobulin was administered without improvement. However, the patient evolved with signs of pyramidal release, methylprednisolone was administered with a good outcome. Cerebrospinal fluid: cytology: 0/mm³, glucose: 59/mm³, protein: 34/mm³. electroneuromyography: upper and lower limbs show signs of polyradiculoneuritis in the hyperacute phase, the nature cannot be classified as myelin or axonal; Cranial magnetic resonance imaging (MRI): signal alteration compromising supra and infratentorial structures, T2/ FLAIR (T2-weighted-Fluid-Attenuated Inversion Recovery) symmetrical hypersignal in the bulbopontine transition suspicious for an demyelinating process. Cervical/thoracic spine MRI: no alterations. Anti Aquaporin-4 IgG: reagent. Discussion: Clinical features are frequently more diverse than just optic neuritis and transverse myelitis in children, making a diagnosis can be challenging. The case series of pediatric NMO have shown a female preponderance and almost all NMO-IgG positive, which had relapsing course predicted by seropositivity. The median age of children diagnosed with NMO ranges from 10 to 14 years. Coexisting autoimmune disorders were reported in 42%. Longer term immunotherapy reduce the chance of further potentially disabling attacks. Conclusion: Diagnosis of NMO isn’t easy, especially when patients are children, the disease is potentially severe and needs to be better understood and managed.