Neurologic complications of Churg-Strauss syndrome - a prospective monocentric study
Neurologic complications of Churg-Strauss syndrome - a prospective monocentric study
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DOI：
10.1111/j.1468-1331.2009.02902.x
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发表时间：
2010-04-01
影响因子：
5.1
通讯作者：
Grau, A. J.
中科院分区：
文献类型：
--
作者：
Wolf, J.;Bergner, R.;Grau, A. J.
背景：Churg-Strauss综合征（CSS）是一种罕见的系统性血管炎。关注神经系统受累的病例系列并不常见。通过这项研究，我们打算评估的频率和类型的神经系统表现和并发症的诊断时间，并在后续的患者与CSS.Methods：在这个单中心研究，连续患者的第一次诊断为CSS的基础上，美国流变学学院的标准，我们的医院包括在2001年和2007年之间。每例患者均接受定期随访，进行临床和电生理检查。结果：14例患者纳入研究。所有患者均有嗜酸性粒细胞增多和哮喘病史。14例患者中有12例有神经系统受累，主要表现为急性或亚急性多发性单神经病（8例）或轴突多发性神经病（3例）。3例患者患有脑神经病变，2例患者患有脑梗死。平均随访时间为31个月。免疫抑制治疗后，13例患者无其他神经系统并发症，1例患者发生脑梗死。最初的神经系统症状作为周围神经病变的结果改善，但后遗症的轴突damaging.Conclusions：即使在CSS的诊断时间，神经系统的表现是常见的，特别是作为一个多发性mononeuropathy。通过随后的免疫抑制治疗，可以在很大程度上避免新的神经系统并发症。
Background:Churg-Strauss syndrome (CSS) is a rare systemic vasculitis. Case series with a focus on neurologic involvement are not common. With this study, we intended to evaluate the frequency and types of neurologic manifestations and complications at time of diagnosis and during follow-up of patients with CSS.Methods:In this monocentric study, consecutive patients of our hospital with first diagnosis of CSS based on the criteria of the American College of Rheumatology were included between 2001 and 2007. Each patient underwent a periodic follow-up with clinical and electrophysiologic examination. Data were obtained prospectively.Results:Fourteen patients were included. All patients had a hypereosinophilia and a history of asthma. Twelve of 14 patients had a neurologic involvement, mainly as an acute or subacute multiplex mononeuropathy (eight patients) or an axonal polyneuropathy (three patients). Three patients suffered from a neuropathy of cranial nerves, and two patients had a cerebral infarct. Mean follow-up period was 31 months. With immunosuppressive therapy, 13 patients had no additional neurologic complications, one patient suffered from a cerebral infarct. Initial neurologic symptoms as a result of peripheral neuropathy improved, but sequelae of axonal damage were persistently detectable.Conclusions:Even at time of diagnosis of a CSS, neurologic manifestations are common, especially as a multiplex mononeuropathy. With a consequent immunosuppressive therapy, new neurologic complications can be avoided for the most part.