Glocker F X, Seifert C, Lücking C H
Department of Neurology and Clinical Neurophysiology, University of Freiburg, Breisacher Strasse 64, D-79106 Freiburg, Germany.
Muscle Nerve. 1999 Sep;22(9):1279-82. doi: 10.1002/(sici)1097-4598(199909)22:9<1279::aid-mus18>3.0.co;2-#.
Heerfordt's syndrome is characterized by fever, uveitis, swelling of the parotid gland, and facial nerve palsy and represents a variety of neurosarcoidosis. Since the first description of the syndrome, discussion about the lesion site has been controversial and has included the assumption of direct nerve compression by parotid gland swelling or a lesion within the facial canal in light of observations of accompanying taste disturbance. We report on a 26-year-old man with typical Heerfordt's syndrome who developed bilateral facial nerve palsy. Electrical and magnetic stimulation of the whole facial motor path provided strong evidence for a pathological process that: (i) began in the cerebellopontine angle; (ii) spread distally into the facial canal; and (iii) could be characterized by proximal demyelination. The patient recovered completely within 6 weeks under immunosuppressive therapy with steroids.
黑尔福特综合征的特征为发热、葡萄膜炎、腮腺肿大及面神经麻痹,是神经结节病的一种表现形式。自该综合征首次被描述以来,关于其病变部位的讨论一直存在争议,鉴于伴有味觉障碍的观察结果,曾有观点认为是腮腺肿大直接压迫神经或面神经管内存在病变。我们报告了一名患有典型黑尔福特综合征的26岁男性,他出现了双侧面神经麻痹。对面部运动全路径进行电刺激和磁刺激,有力地证明了一个病理过程:(i)始于小脑脑桥角;(ii)向远侧蔓延至面神经管;(iii)其特征为近端脱髓鞘。该患者在接受类固醇免疫抑制治疗后6周内完全康复。