Neurología (English Edition) (May 2021)
Optic neuritis in paediatric patients: experience over 27 years and a management protocol
Abstract
Introduction: In this article, we present our experience on optic neuritis (ON) and provide a diagnostic/therapeutic protocol, intended to rule out other aetiologies (particularly infection), and a fact sheet for parents. Methods: We conducted a descriptive, retrospective study of patients with ON over a 27-year period (1990–2017). A review of the available scientific evidence was performed in order to draft the protocol and fact sheet. Results: Our neuropaediatrics department has assessed 20 744 patients in the last 27 years, of whom 14 were diagnosed with ON: 8 had isolated ON, 1 had multiple sclerosis (MS), 1 had clinically isolated syndrome (CIS), 3 had acute disseminated encephalomyelitis (ADEM), and 1 had isolated ON and a history of ADEM one year previously. Patients’ age range was 4–13 years; 50% were boys. Eight patients were aged over 10: 7 had isolated ON and 1 had MS. Nine patients had bilateral ON, and 3 had retrobulbar ON. MRI results were normal in 7 patients and showed involvement of the optic nerve only in 2 patients and optic nerve involvement + central nervous system demyelination in 5. Thirteen patients received corticosteroids. One patient had been vaccinated against Meningococcus-C the previous month. Progression was favourable, except in the patient with MS. A management protocol and fact sheet are provided. Conclusions: ON usually has a favourable clinical course. In children aged older than 10 years with risk factors for MS or optic neuromyelitis (hyperintensity on brain MRI, oligoclonal bands, anti-NMO antibody positivity, ON recurrence), the initiation of immunomodulatory treatment should be agreed with the neurology department. The protocol is useful for diagnostic decision-making, follow-up, and treatment of this rare disease with potentially major repercussions. The use of protocols and fact sheets is important. Resumen: Introducción y objetivos: Se presenta nuestra experiencia en neuritis óptica (ON) y se elabora un protocolo diagnóstico-terapéutico, que contempla descartar otras causas, principalmente infecciosas y se elabora una hoja informativa para padres. Material y método: Estudio descriptivo retrospectivo de los pacientes con ON en 27 años (1990–2017). Revisión de evidencia científica para elaboración del protocolo y hoja informativa. Resultados: En nuestra sección de neuropediatría se valoraron 20.744 niños en 27 años, 14 con ON: 8 ON aisladas, 1 esclerosis múltiple (EM), 1 episodio clínicamente aislado (CIS), 3 encefalomielitis agudas diseminadas (EMAD) y 1 paciente con ON aislada que el año anterior había sufrido una EMAD. Edades entre 4-13 años, 50% varones. Mayores de 10 años, 8 pacientes: 7 ON aisladas y 1 EM. Bilaterales 9, retrobulbares 3. Resonancia magnética (RM) cerebral normal en 7, sólo afectación del nervio óptico en 2 y con desmielinización del SNC en 5 casos. Recibieron corticoterapia 13/14. Un caso vacunado de Meningococo-C el mes anterior. Todos evolucionaron favorablemente, salvo la EM. Se presentan el protocolo y la hoja de información. Conclusiones: Habitual curso favorable. En niños a partir de 10 años, con factores de riesgo de desarrollar EM o neuromielitis óptica (presencia de hiperseñales en RM cerebral, bandas oligoclonales, anti-NMO, recurrencia de ON), se consensúa con Neurología el inicio de tratamiento inmunomodulador. Utilidad del protocolo para la toma de decisiones diagnósticas, de seguimiento y tratamiento, de una patología poco frecuente pero con posibles repercusiones importantes. Importancia de la protocolización y hojas informativas.
