Kararizou E, Davaki P, Spengos K, Karandreas N, Dimitracopoulos A, Vassilopoulos D
Section of Neuropathology, Neurological Clinic of University of Athens, Aeginition Hospital, Athens, Greece.
Clin Neuropathol. 2011 Jan-Feb;30(1):11-7. doi: 10.5414/npp30011.
The purpose of this study was to investigate the clinical, electrophysiological and pathological features of Churg Strauss syndrome (CSS) neuropathy.
Biopsies were selected from over 700 sural nerve biopsies. The diagnosis of vasculitis was based on established clinicopathological criteria. Complete laboratory, clinical, electrophysiological and pathological studies were performed in all cases.
Nerve biopsies of 9 patients were diagnosed as Churg-Strauss syndrome. The pathological features were vasculitis with predominant axonal degeneration and a varying pattern of myelinated fiber loss. The vasculitic changes were found mainly in small epineural blood vessels. Mononeuritis multiplex and distal symmetrical and asymmetrical sensorimotor neuropathy, were equally frequent.
We conclude that, Churg-Strauss syndrome complicated frequently with polyneuropathy, and as remission depends on immunosuppressive therapy, it is important to recognize it in the early stage. The diagnosis of polyneuropathy is based on clinical and electrophysiologic studies, but precise histology, immunolohistochemistry and morphometric study of the peripheral nerve biopsy may be decisive in establishing the diagnosis.
本研究旨在探讨变应性肉芽肿性血管炎（CSS）神经病变的临床、电生理及病理特征。
从700多例腓肠神经活检中选取样本。血管炎的诊断基于既定的临床病理标准。对所有病例均进行了完整的实验室、临床、电生理及病理研究。
9例患者的神经活检被诊断为变应性肉芽肿性血管炎。病理特征为血管炎，以轴索性变性为主，有不同类型的有髓纤维丢失。血管炎改变主要见于神经外膜小血管。多灶性单神经病以及远端对称性和非对称性感觉运动性神经病的发生率相同。
我们得出结论，变应性肉芽肿性血管炎常并发多发性神经病，由于缓解取决于免疫抑制治疗，早期识别很重要。多发性神经病的诊断基于临床和电生理研究，但外周神经活检的精确组织学、免疫组织化学及形态计量学研究对于确立诊断可能起决定性作用。
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