Galan E, Kousseff B G
Department of Pediatrics, University of South Florida, Tampa 33617-3451, USA.
Pediatr Neurol. 1995 Apr;12(3):242-5. doi: 10.1016/0887-8994(95)00003-x.
Two unrelated male patients with clinical manifestations of Ehlers-Danlos syndrome type III and peripheral neuropathy are presented. At age 13 years, one developed bilateral brachial plexus palsy unrelated to trauma and 2 years later, a right lumbosacral plexopathy. The other presented at age 3 years with a left brachial plexopathy after sustaining a fracture of the lateral condyle of the right humerus. In both patients, nerve conduction velocities demonstrated conduction block across the brachial plexus and recovery was incomplete, indicating that peripheral neuropathy is a serious complication of Ehlers-Danlos syndrome. Its prompt diagnosis facilitates the care of patients with this syndrome. Increased ligament laxity/stretchability and mechanical trauma may play an important role in the pathogenesis of the neuropathy.
本文介绍了两名患有III型埃勒斯-当洛综合征临床表现和周围神经病变的无关男性患者。一名患者13岁时出现与创伤无关的双侧臂丛神经麻痹,2年后出现右侧腰骶丛病变。另一名患者3岁时在右肱骨外侧髁骨折后出现左侧臂丛病变。两名患者的神经传导速度均显示臂丛神经存在传导阻滞且恢复不完全,表明周围神经病变是埃勒斯-当洛综合征的严重并发症。其及时诊断有助于该综合征患者的护理。韧带松弛/伸展性增加和机械性创伤可能在神经病变的发病机制中起重要作用。