变应性肉芽肿性血管炎的神经系统表现

Neurological manifestations of allergic granulomatous angiitis

目的研究变应性肉芽肿性血管炎(AGA,亦称ChurgStrauss综合征,CSS)相关神经病的发病率、临床类型、发病机制、诊断和治疗。方法14例确诊为CSS的患者从临床表现、实验室检查、神经电生理检查和活检病理检查等方面予以分析,并观察治疗效果。结果14例CSS患者中有12例累及周围神经系统,其中5例以周围神经病为首发症状。12例周围神经受累的患者中5例表现为多发性单神经病,4例表现为远端不对称的多发性神经病,2例表现为对称的多发性神经病。腓肠神经活检可见有髓神经纤维丢失,轴索变性。电生理检查发现感觉和运动传导波幅显著降低或消失。CSS的诊断应结合临床与病理所见。除1例患者外,激素治疗对CSS相关神经病有效。结论CSS患者中周围神经病很常见,早期诊断可改善预后。

Objective To investigate the frequency, types, pathogenesis, diagnosis and treatment of the neurological involvement from Churg-Strauss syndrome （CSS）. Methods Fourteen patients of CSS were assessed in terms of clinical, laboratory, electrophysiologic, pathological and outcome data. Results Of the 14 patients, 12 had peripheral neuropathy, and the initial symptom of 5 patients showed neurological involvement. Of the 12 patients with peripheral neuropathy, 5 had multiple mononeuropathy, 4 had distal asymmetric polyneuropathy, and 2 had symmetric polyneuropathy. Loss of myelinated fibers due to axonal degeneration was found in sural nerve biopsy. Markedly decreased or absent amplitudes of sensory and motor nerves also indicated the CSS associated peripheral neuropathy was axonal neuropathy. Diagnosis should be made on basis of the combination of clinical and pathological features. Corticosteroid therapy was effective for patients except one. Conclusions Peripheral neuropathy should be common in CSS. The earlier the diagnosis is made, the better the outcome is achieved.