Recurrent painful ophthalmoplegic neuropathy:a case report and literature review
Objective:To investigate the clinical features of recurrent painful ophthalmoplegic neuropathy(RPON),so as to improve the understanding of RPON.Methods:The clinical manifestations,diagnosis and treat-ment of a child with RPON admitted to the Department of Neurology,Children's Hospital Affiliated to Nanjing Medi-cal University were retrospectively analyzed,and the relevant literatures were reviewed.Results:The patient was admitted to the hospital with headache and left oculomotor nerve palsy as the chief complaint.After excluding orbit-al,sellar and posterior fossa lesions,the patient was diagnosed as RPON and discharged with symptomatic treatment such as glucocorticoids.A total of 19 literatures were collected,including 26 children,of whom 96.15%had head-ache symptoms and 73.08%had concomitant symptoms.All of the patients showed ophthalmoplegia symptoms,and the oculomotor nerve was involved.Among the patients who completed enhanced MRI,73.08%showed oculomotor nerve thickening and enhancement.Nineteen patients treated with glucocorticoids achieved good results.Conclusion:RPON is a rare disease characterized by recurrent unilateral headache accompanied by ipsialateral ophthalmoplegia.The etiology of RPON is unknown.The typical patients start to have the disease at an early age,and the oculomotor nerve is the most commonly involved.Glucocorticoid therapy is considered to be effective in clinical practice.