复发性痛性眼肌麻痹神经病一例报告并文献复习  

Recurrent painful ophthalmoplegic neuropathy:a case report and literature review

作　　者：吴亦湄 郭虎[1] 卢孝鹏[1] 高修成[2] 李杨 WU Yimei;GUO Hu;LU Xiaopeng;GAO Xiucheng;LI Yang(Departmengt of Neurology,Children's Hospital Affiliated to Nanjing Mediccal University,Nanjing 210008,China;Departmengt of Radiology,Children's Hospital Affiliated to Nanjing Mediccal University,Nanjing 210008,China)

机构地区：[1]南京医科大学附属儿童医院神经内科,江苏南京210008 [2]南京医科大学附属儿童医院放射科,江苏南京210008

出　　处：《东南大学学报（医学版）》2024年第5期782-788,共7页Journal of Southeast University(Medical Science Edition)

摘　　要：目的:探讨复发性痛性眼肌麻痹神经病(RPON)的临床特征,以提高对该病的认识。方法:对南京医科大学附属儿童医院神经内科收治的1例RPON患儿的临床表现及诊治过程进行回顾性分析,并对相关文献进行复习。结果:该患儿反复3次病程,均以头痛及左侧动眼神经麻痹为主诉入院,通过相关检查,排除眼眶、鞍旁及后颅窝的病变,确诊为RPON,予以糖皮质激素等对症治疗后好转出院。文献检索共收集19篇文献,共计26例患儿,其中96.15%患儿有头痛症状,73.08%有伴随症状,所有患儿均表现出眼肌麻痹症状,且均有动眼神经受累,所有病例均接受了增强MRI检查,其中73.08%患儿存在动眼神经增厚、强化。19例接受糖皮质激素治疗的患儿均取得了较好的疗效。结论:RPON是一种以复发性单侧头痛伴同侧眼肌麻痹为特征的罕见病,本病病因未明,典型者幼年开始起病,动眼神经最常受累,部分患者糖皮质激素治疗有效。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 treatment of a child with RPON admitted to the Department of Neurology,Children's Hospital Affiliated to Nanjing Medical 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 orbital,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 headache 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.

关 键 词：复发性痛性眼肌麻痹神经病 头痛 眼睑下垂 动眼神经麻痹 

分 类 号：R725[医药卫生—儿科]