IRCCS Istituto delle Scienze Neurologiche di Bologna, UOC Clinica Neurologica, Bologna, Italy.
Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna, Italia.
Cephalalgia. 2023 Jan;43(1):3331024221133386. doi: 10.1177/03331024221133386.
Recurrent Painful Ophthalmoplegic Neuropathy, previously known as Ophthalmoplegic Migraine, is a poorly characterized disorder mainly because there are few cases described. We report a new case of Recurrent Painful Ophthalmoplegic Neuropathy and a review of the literature to contribute to increasing the knowledge of the clinical features of this disorder.
A 45-year-old woman presented with adult-onset recurrent attacks of abducens and oculomotor palsy associated with diplopia followed by headache. Most notably, pain always presented many days after oculomotor impairment, a feature never described in the literature. A diagnosis of possible Recurrent Painful Ophthalmoplegic Neuropathy was made after excluding other possible mimicking disorders. Symptoms usually resolved gradually with corticosteroid therapy, albeit without a clear-cut benefit.Clinical data collected from 1989 to 2022 showed that adult onset in Recurrent Painful Ophthalmoplegic Neuropathy is not uncommon. While III cranial nerve palsy is typical, VI and IV nerve palsy have also been described.
Several hypotheses have been proposed, including nerve compression, ischemia or inflammation/demyelination, but none has been completely accepted.Diagnosis remains of exclusion; magnetic resonance imaging and blood exams are key in differential diagnosis.
Our case gives us the possibility to expand the clinical features of Recurrent Painful Ophthalmoplegic Neuropathy, also contributing to updating the pathophysiological hypotheses.
复发性疼痛性眼肌麻痹神经病，以前称为眼肌偏头痛，是一种特征不明显的疾病，主要是因为描述的病例很少。我们报告了一例新的复发性疼痛性眼肌麻痹神经病，并对文献进行了回顾，以增加对该疾病临床特征的认识。
一名 45 岁女性出现成年后复发性展神经和动眼神经麻痹伴复视，随后出现头痛。最值得注意的是，疼痛总是在眼肌功能障碍数天后出现，这一特征在文献中从未描述过。在排除其他可能的类似疾病后，诊断为可能的复发性疼痛性眼肌麻痹神经病。虽然皮质类固醇治疗后症状通常逐渐缓解，但没有明显的益处。从 1989 年到 2022 年收集的临床数据显示，复发性疼痛性眼肌麻痹神经病在成年发病并不罕见。III 颅神经麻痹是典型的，但也有 VI 和 IV 颅神经麻痹的描述。
已经提出了几种假说，包括神经压迫、缺血或炎症/脱髓鞘，但没有一种假说完全被接受。诊断仍然是排他性的；磁共振成像和血液检查是鉴别诊断的关键。
我们的病例使我们有可能扩展复发性疼痛性眼肌麻痹神经病的临床特征，也有助于更新病理生理学假说。
