Background

Recurrent painful ophthalmoplegic neuropathy (RPON) is an uncommon disorder characterized by recurrent unilateral headache attacks associated with ipsilateral ophthalmoplegia. We intend to study the clinical picture in our case series along with the published literature to discuss the pathogenesis and propose modified diagnostic criteria for recurrent painful ophthalmoplegic neuropathy.

Methods

We reported five cases diagnosed as ophthalmoplegic migraine/RPON in our medical centers and reviewed the published literature related to RPON from the Pubmed database between 2000 and 2020. In one of these cases, a multiplanar reformation was performed to look at the aberrant cranial nerve.

Results

The mean onset age for RPON was 22.1 years, and the oculomotor nerve was the most commonly involved cranial nerve (53.9%) in 165 reviewed patients. In most patients, ophthalmoplegia started within 1 week of the headache attack (95.7%, 67/70). Additionally, 27.6% (40/145) of patients presented enhancement of the involved nerve(s) from MRI tests. Finally, 78 patients received corticosteroids, out of which 96.2% benefited from them.

Conclusion

This is the first time multiplanar reformation has been performed to reveal the distortion of the oculomotor nerve. Modified diagnostic criteria are proposed. We hope to expand the current knowledge and increase the detection of recurrent painful ophthalmoplegic neuropathy in the future.

中文翻译：

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复发性痛性眼肌麻痹性神经病的改良诊断标准：5 例病例报告和文献复习。

背景

复发性疼痛性眼肌麻痹性神经病 (RPON) 是一种罕见的疾病，其特征是与同侧眼肌麻痹相关的复发性单侧头痛发作。我们打算研究我们病例系列中的临床图片以及已发表的文献，以讨论发病机制并提出复发性痛性眼肌麻痹性神经病的改良诊断标准。

方法

我们报告了我们医疗中心诊断为眼肌麻痹性偏头痛/RPON 的 5 例病例，并回顾了 2000 年至 2020 年 Pubmed 数据库中已发表的与 RPON 相关的文献。在其中一个病例中，进行了多平面重建以观察异常的颅神经。

结果

RPON 的平均发病年龄为 22.1 岁，在 165 名接受审查的患者中，动眼神经是最常受累的颅神经（53.9%）。大多数患者在头痛发作后 1 周内开始出现眼肌麻痹 (95.7%, 67/70)。此外，27.6% (40/145) 的患者在 MRI 测试中表现出受累神经的增强。最后，78 名患者接受了皮质类固醇治疗，其中 96.2% 的患者从中受益。

结论

这是第一次进行多平面重建以揭示动眼神经的扭曲。建议修改诊断标准。我们希望在未来扩展现有知识并增加对复发性眼肌麻痹性神经病变的检测。