Department of Neurology and Laboratory of Neuroscience, Dino Ferrari Centre, Università degli Studi di Milano-IRCCS Istituto Auxologico Italiano, Milan, Italy.
J Neurol Sci. 2011 Feb 15;301(1-2):107-11. doi: 10.1016/j.jns.2010.10.016. Epub 2010 Nov 20.
Churg-Strauss syndrome (CSS) is a rare systemic vasculitis, almost invariably accompanied by asthma, nasal polyposis, paranasal sinus abnormalities, and increased peripheral blood eosinophil count. Neurological involvement as peripheral neuropathy is a common feature, whereas cerebral involvement is extremely rare. Herein, we report the case of a young man who presented with sudden onset of right-side emiparesis and aphasia, whose head CT scan showed the presence of large haemorrhage in the left striatum nucleus involving part of the temporal lobe. Based on clinical and laboratory findings (asthma, eosinophilia >10%, paranasal sinus abnormalities and mononeuritis multiplex) a diagnosis of CSS was made. Cerebral angiography resulted normal, excluding the presence of vascular malformations or signs of vessel abnormalities. Pharmacotherapy with (intravenous and afterwards oral) corticosteroid and immunosuppressors (cyclophosphamide and then azathioprine) was initiated. The outcome was good with neurological follow-up showing a nearly complete recover. Our case points out that intracerebral haemorrhage can be, despite rare, a presenting feature of CSS. Previously reported patients affected by cerebral haemorrhage and CSS are summarized and briefly reviewed.
变应性肉芽肿性血管炎（CSS）是一种罕见的系统性血管炎，几乎总是伴有哮喘、鼻息肉、副鼻窦异常和外周血嗜酸性粒细胞计数增加。神经受累表现为多发性单神经病是常见特征，而脑受累极为罕见。在此，我们报告了一例年轻男性，突发右侧偏瘫和失语，头部 CT 扫描显示左侧纹状体核有大量出血，累及部分颞叶。根据临床和实验室发现（哮喘、嗜酸性粒细胞计数>10%、副鼻窦异常和单神经病），诊断为 CSS。脑血管造影结果正常，排除了血管畸形或血管异常的存在。采用（静脉和随后口服）皮质类固醇和免疫抑制剂（环磷酰胺和随后的硫唑嘌呤）进行药物治疗。神经学随访显示，患者的病情几乎完全恢复，预后良好。我们的病例表明，尽管罕见，但颅内出血也可能是 CSS 的表现特征。总结并简要回顾了先前报道的患有脑出血和 CSS 的患者。
