Cheng Meng-Ju, Huang Pai-Hao, Liao Pin-Wen, Chen Jen-Tse, Chiang Tsuey-Ru
Department of Neurology, Cathay General Hospital, Taipei, Taiwan.
Acta Neurol Taiwan. 2012 Dec;21(4):169-75.
Churg-Strauss syndrome (CSS) is a rare autoimmune disease with small-vessel vasculitis. Neurological manifestation of CSS is common. Central nervous system is less frequently involved than that of peripheral nervous system.
We report a case of 60-year-old man who presented with acute onset of right hemiparesis and impaired cognition. The presence of hypereosinophilia, asthma, sinusitis and extravascular eosinophil accumulation led to the diagnosis of Churg-Strauss syndrome. Brain magnetic resonance imaging (MRI) revealed multiple infarcts in bilateral cerebral and cerebellar hemispheres. The neurophysiology study did not reveal peripheral neuropathy. The patient was effectively treated with methylprednisolone, cyclophosphamide and warfarin.
Symptoms and signs of central nervous system can be the initial neurological manifestation of CSS patients. CSS should be considered while patients have stroke and hypereosinophilia. In our patient, there is a good response to timely steroid, immunosuppressant and anticoagulant therapies.
变应性肉芽肿性血管炎（CSS）是一种罕见的伴有小血管血管炎的自身免疫性疾病。CSS的神经系统表现较为常见。中枢神经系统受累的频率低于周围神经系统。
我们报告一例60岁男性患者，其急性起病，表现为右侧偏瘫和认知障碍。高嗜酸性粒细胞血症、哮喘、鼻窦炎及血管外嗜酸性粒细胞积聚的存在，导致变应性肉芽肿性血管炎的诊断。脑磁共振成像（MRI）显示双侧大脑半球和小脑半球多发梗死灶。神经生理学研究未发现周围神经病变。该患者接受甲泼尼龙、环磷酰胺和华法林治疗有效。
中枢神经系统的症状和体征可能是CSS患者最初的神经学表现。当患者出现卒中及高嗜酸性粒细胞血症时，应考虑CSS。在我们的患者中，及时使用类固醇、免疫抑制剂和抗凝治疗有良好疗效。
