J Oral Facial Pain Headache. 2020;34(4):374-378. doi: 10.11607/ofph.2656.
Recurrent painful ophthalmoplegic neuropathy (RPON) is a very rare disease characterized by recurrent attacks (at least two) of unilateral headache associated with ipsilateral ophthalmoplegia due to paresis of one or more cranial motor nerves, not due to any orbital, parasellar, or posterior fossa lesions. The differential diagnoses for this condition are broad. In addition to disability during an acute attack, this disease could also cause a permanent neurologic deficit. The understanding of RPON pathogenesis has changed over time, leading to a change in the classification of this disorder between editions of the International Classification of Headache Disorders, in which the condition was moved from the chapter on migraine to the chapter on cranial neuralgias and central causes of facial pain. There is no consensus on the pathogenesis of RPON. It is possible that multiple pathogenic mechanisms underlie various clinical forms of the disease. A depiction of pathologic analyses of patients with radiologically confirmed changes in the affected nerves during and outside of attacks would significantly contribute to knowledge of its pathogenesis. Brain imaging should be performed in each patient during an acute RPON attack and at a regular schedule between attacks. Further case reports and case series are required before further conclusions can be made regarding RPON pathogenesis and proposals for treatment options.
复发性单侧头痛性眼肌麻痹（RPON）是一种非常罕见的疾病，其特征为反复发作（至少两次）单侧头痛，伴有同侧眼肌麻痹，由一条或多条颅神经麻痹引起，并非由于眶内、鞍旁或颅后窝病变所致。该病症的鉴别诊断非常广泛。除了在急性发作期间的残疾外，这种疾病还可能导致永久性的神经功能缺损。随着时间的推移，人们对 RPON 发病机制的认识发生了变化，导致该疾病在国际头痛疾病分类的各版本之间的分类发生了变化，从偏头痛章节转移到颅神经痛和面部疼痛的中枢性原因章节。目前对于 RPON 的发病机制尚无共识。可能存在多种发病机制，导致疾病的各种临床表现。在发作期间和发作之外对受影响神经的影像学证实改变的患者进行病理分析的描述，将极大地有助于了解其发病机制。在急性 RPON 发作期间和发作之间的定期时间内应在每位患者中进行脑部成像。需要进一步的病例报告和病例系列研究，才能进一步得出关于 RPON 发病机制和治疗方案建议的结论。
