Disease course of Chronic Relapsing Inflammatory Optic Neuropathy (CRION) in a single care center

ABSTRACT

Background: Chronic relapsing inflammatory optic neuropathy (CRION) is a recurrent, idiopathic optic neuritis and is considered as a rare disease. Objective: To describe the clinical course during long-term follow-up of patients with a diagnosis of CRION. Methods: From a cohort of 1,735 patients with demyelinating disorders, we selected patients aged over 16 years with CRION according to current criteria. Demographic and clinical data, including initial presentation, symptoms, number of relapses, time delay in diagnosis, diagnostic methods, and treatment were obtained from clinical files. Infections, autoimmune diseases, and multiple sclerosis, among other conditions, were ruled out in all patients. Results: We analyzed 30 patients with CRION: 24 women and six men, with mean age of 42.8±10.2 years, median disease course of 7.9 years (5.29-13.1), and median number of attacks of 2 (IQR 2-4). The initial manifestation was ocular pain in 97% and bilateral and sequential affection in 87%. Visual acuity was recovered in 50%, did not improve in 33%, and recovered incompletely in 17%. Antibodies against aquaporin-4 (AQP4-Abs) were negative in 73.3%. Magnetic resonance imaging of the brain was normal in 76.7%. None of the patients evolved to another demyelinating disease over time. Initial treatment was methylprednisolone in 100%, and plasmapheresis in 20%. Currently, all patients are on maintenance treatment with mycophenolate mofetil or rituximab with a decrease in relapsing rate. Conclusions: Diagnosis of CRION is challenging and should be kept in mind. Prompt diagnosis, adequate treatment and close follow-up are essential to prevent disabling sequelae in these patients.

RESUMEN

Antecedentes: Neuropatía óptica inflamatoria crónica recidivante (CRION) es una neuritis óptica idiopática recurrente, considerada una enfermedad rara. Objetivo: Describir la evolución clínica durante el seguimiento a largo plazo de pacientes con diagnóstico de CRION. Métodos: De una cohorte de 1.735 pacientes con trastornos desmielinizantes, seleccionamos pacientes mayores de 16 años con diagnóstico de CRION según los criterios actuales. Datos demográficos y clínicos, incluyendo presentación inicial, síntomas, recaídas, tiempo de retraso diagnóstico, métodos de diagnóstico y tratamiento se obtuvieron de los archivos clínicos. Se descartaron en todos los pacientes infecciones, enfermedades autoinmunes, esclerosis múltiple, entre otras condiciones. Resultados: Se analizaron 30 pacientes con diagnóstico de CRION: 24 mujeres y 6 hombres, edad media de 42,8±10,2 años, mediana del curso de la enfermedad de 7,9 años (5,2-13,1), mediana del número de recaídas 2 (IQR 2-4). La manifestación inicial fue dolor ocular en el 97% y afección bilateral y secuencial en el 87%. La agudeza visual mejoró en el 50%, sin recuperación en el 33% y con restauración incompleta en el 17%. Los anticuerpos contra acuaporina-4 (AQP4-Abs) fueron negativos en el 73,3%. La resonancia magnética cerebral fue normal en el 76,7%. Ningún paciente evolucionó hacia otra enfermedad desmielinizante en el seguimiento. El tratamiento inicial fue metilprednisolona en el 100%, y plasmaféresis en el 20%. Actualmente, todos los pacientes están en tratamiento de mantenimiento con micofenolato de mofetilo o rituximab con disminución de la tasa de recaídas. Conclusiones: El diagnóstico de CRION representa un desafío y debe tenerse en cuenta. El diagnóstico oportuno, tratamiento adecuado y seguimiento estrecho son fundamentales para evitar secuelas invalidantes.

Keywords:

Optic Neuritis - Recurrence - Therapeutics

Palabras clave:

Neuritis Óptica - Recurrencia - Terapéutica

Authors’ contributions:

LEMC: conceptualization, investigation and supervision; JLT: writing of the original draft, methodology; MPJA: data curation, investigation; MPCD: formal analysis; GM: methodology, writing of the original draft, writing-review and editing.

Publication History

Received: 22 April 2021

Accepted: 19 July 2021

Article published online:
06 February 2023

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