Steele J C, Vasuvat A
J Neurol Neurosurg Psychiatry. 1970 Dec;33(6):828-32. doi: 10.1136/jnnp.33.6.828.
This report of 14 Thai patients describes the clinical features of a symptom-complex we have termed recurrent multiple cranial nerve palsies. While the disorder is common in South Eastern Asia, patients suffering similar symptoms have been seen in Europe and America. Characteristically, a long prodromal headache precedes the abrupt onset of multiple cranial nerve palsies affecting predominantly, although not exclusively, the oculomotor and facial nerves. Symptoms are self-limited and steroid therapy hastens recovery in most cases. Recurrence after many months or years is not uncommon and different cranial nerves may then be affected. Wider neurological involvement seems not to occur. Although the erythrocyte sedimentation rate is frequently elevated there is no other evidence of co-existing systemic disease. The single necropsy study thus far reported indicates that the pathogenesis of the syndrome is granulomatous inflammation of the meninges (dura), adjacent to, and involving the perineurium of cranial nerves. It is uncertain whether this inflammation is a response to a single agent, as yet unidentified, or a focal and selective hypersensitivity reaction of connective tissue to a number of different factors. As a clinical entity the syndrome is distinctive, international, and deserving of wide recognition.
这份关于14例泰国患者的报告描述了一种我们称为复发性多发性颅神经麻痹的症状复合体的临床特征。虽然这种疾病在东南亚很常见，但在欧洲和美洲也见过有类似症状的患者。其特征是，在多发性颅神经麻痹突然发作之前，会有一段较长的前驱性头痛，主要影响动眼神经和面神经，但并非仅限于此。症状具有自限性，在大多数情况下，类固醇疗法可加速恢复。数月或数年之后复发并不罕见，届时可能会累及不同的颅神经。似乎不会出现更广泛的神经系统受累情况。虽然红细胞沉降率经常升高，但没有其他证据表明存在并存的全身性疾病。迄今为止报道的唯一一例尸检研究表明，该综合征的发病机制是脑膜（硬脑膜）的肉芽肿性炎症，累及颅神经的神经外膜并与之相邻。尚不确定这种炎症是对一种尚未确定的单一因素的反应，还是结缔组织对多种不同因素的局部和选择性过敏反应。作为一种临床实体，该综合征具有独特性、国际性，值得广泛认可。
