Sakamoto Naohiro, Hongo Yu, Takazaki Hiroshi, Kaida Kenichi, Ikewaki Katsunori, Suzuki Kazushi
Department of Neurology, Anti-aging and Vascular medicine, Division of Internal Medicine, National Defense Medical College.
Department of Neurology, Saitama Medical Center, Saitama Medical University.
Rinsho Shinkeigaku. 2022 Apr 27;62(4):281-285. doi: 10.5692/clinicalneurol.cn-001691. Epub 2022 Mar 29.
The patient was a 14-year-old boy with two previous episodes of self-remitting right ophthalmoplegia with right temporal pain at ages 9 and 12. In 2019, he developed right eyelid ptosis and diplopia 2 days after a pulsating right-sided temporoparietal headache. Recurrent headaches with ophthalmoplegia responded to high-dose steroid therapy, and the clinical features resembled recurrent painful ophthalmoplegic neuropathy (RPON). RPON generally presents with MRI findings of hypertrophy and inflammation at the root of the oculomotor nerve, a vulnerable site of the blood-brain barrier. However, the imaging features in this case were different from those in typical cases of RPON, and oculomotor nerve inflammation was found in the cavernous sinus. The order of onset of headache and oculomotor nerve palsy differed in each recurrence, suggesting that both autoimmune and vascular mechanisms may have been involved in the onset of the disease in our case.
该患者是一名14岁男孩，曾在9岁和12岁时出现过两次自行缓解的右侧眼肌麻痹并伴有右侧颞部疼痛。2019年，他在一次搏动性右侧颞顶部头痛2天后出现右侧眼睑下垂和复视。伴有眼肌麻痹的复发性头痛对大剂量类固醇治疗有反应，临床特征类似于复发性疼痛性眼肌麻痹性神经病（RPON）。RPON通常表现为动眼神经根部肥大和炎症的MRI表现，而动眼神经根部是血脑屏障的一个薄弱部位。然而，该病例的影像学特征与典型的RPON病例不同，且在海绵窦发现了动眼神经炎症。每次复发时头痛和动眼神经麻痹的发作顺序不同，提示在我们的病例中，自身免疫和血管机制可能都参与了疾病的发作。
