Behrens R, Holzhausen H J, von Poblozki A, Stephan M, Kämpfe D, Schwartze G, Fleig W E
Klinik und Poliklinik für Innere Medizin I, Martin-Luther-Universität Halle-Wittenberg.
Dtsch Med Wochenschr. 1998 Jan 2;123(1-2):6-11. doi: 10.1055/s-2007-1023891.
A 39-year-old woman in good health suddenly developed painful paraesthesia and incomplete paresis of all four limbs. Furthermore, arterial hypertension and tachycardia were found. The patient reported a history of chronic allergic rhinitis for 10 years and asthma one year.
Laboratory data showed an increased leucocyte count with a substantial increase in eosinophils in peripheral blood as well as in bone marrow. Nonspecific inflammatory markers such as erythrocyte sedimentation rate, C-reactive protein, fibrinogen as well as eosinophil cationic protein (ECP) were also increased. Histological investigations of a skin-muscle biopsy of the quadriceps revealed necrotizing vasculitis with extravascular granulomata and histiocytic giant cells.
DIAGNOSIS, TREATMENT AND COURSE: Based on the diagnosis of Churg-Strauss syndrome treatment with methylprednisolone (500 mg every second day) was started, but was found to be ineffective after 10 days. Symptoms responded well to a subsequent course of 150 mg cyclophosphamide combined with 50 mg prednisolone per day. Pareses and pain were significantly reduced and all qualitative nerve functions returned to normal within two weeks of treatment. Laboratory parameters, especially the ECP, were similarly normalized.
Churg-Strauss syndrome should be considered in the differential diagnosis of unexplained polyneuropathy. Determination of ECP may not only help in the diagnosis but does also facilitate monitoring of treatment and of further course of the disease.
一名39岁身体健康的女性突然出现四肢疼痛性感觉异常和不完全性麻痹。此外,还发现有动脉高血压和心动过速。患者报告有10年慢性变应性鼻炎病史和1年哮喘病史。
实验室数据显示白细胞计数增加,外周血及骨髓中的嗜酸性粒细胞大幅增多。非特异性炎症标志物如红细胞沉降率、C反应蛋白、纤维蛋白原以及嗜酸性粒细胞阳离子蛋白(ECP)也升高。股四头肌皮肤肌肉活检的组织学检查显示为坏死性血管炎,伴有血管外肉芽肿和组织细胞巨细胞。
诊断、治疗与病程:基于变应性肉芽肿性血管炎综合征的诊断,开始用甲泼尼龙治疗(隔日500mg),但10天后发现无效。随后采用每日150mg环磷酰胺联合50mg泼尼松龙的治疗方案,症状反应良好。治疗两周内,麻痹和疼痛明显减轻,所有神经功能质性指标恢复正常。实验室参数,尤其是ECP,同样恢复正常。
在不明原因的多发性神经病鉴别诊断中应考虑变应性肉芽肿性血管炎综合征。测定ECP不仅有助于诊断,还便于监测治疗及疾病的进一步发展过程。