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Churg-Strauss 综合征伴发缺血性脑卒中与复发性紫癜。

Churg-Strauss syndrome with concomitant occurrence of ischemic stroke and relapsing purpura.

机构信息

Department of Cerebrovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.

出版信息

J Stroke Cerebrovasc Dis. 2012 Nov;21(8):911.e9-10. doi: 10.1016/j.jstrokecerebrovasdis.2011.11.010. Epub 2011 Dec 29.

DOI:10.1016/j.jstrokecerebrovasdis.2011.11.010
PMID:22209309
Abstract

A 77-year-old woman suffering from chronic bronchial asthma and chronic atrial fibrillation who had had a previous ischemic stroke presented to our emergency unit with gait disturbance. She had new-onset truncal ataxia, right hemiparesis, and right sensory disturbance related to the previous stroke. Her lower legs were slightly swollen and had a reddened appearance. Her medical history included mitral valve replacement because of severe mitral valve regurgitation. Her white blood cell count was 8600/μL, mainly consisting of eosinophils (4480/μL; 52.1%). Serum nonspecific immunoglobulin E was elevated to 1600 IU/mL (normal range <170 IU/mL). She was taking warfarin for secondary stroke prevention, and on admission her prothrombin time international normalized ratio was 3.06. Diffusion-weighted magnetic resonance imaging revealed a fresh infarct in the right cerebellum. No stenosis or occlusion was shown in the cervicocephalic arteries on magnetic resonance angiography or carotid ultrasound. No emboligenic diseases, except for atrial fibrillation, were identified. On day 3, an extensive itchy, purpuric rash appeared on her lower limbs. The rash remitted and recurred spontaneously for several weeks. A skin biopsy specimen of the purpuric lesions revealed massive eosinophilic infiltration of the dermis and eosinophilic vasculitis involving small vessels. We diagnosed the patient with Churg-Strauss syndrome (CSS). Skin lesions and eosinophilia disappeared after oral corticosteroid therapy. In this case, cerebellar infarction occurred with purpuric rash despite well-controlled anticoagulation. Patients with CSS may suffer from ischemic stroke when the condition of CSS deteriorates.

摘要

一位 77 岁的女性,患有慢性支气管哮喘和慢性心房颤动,曾患有缺血性中风,因步态不稳到我院急诊科就诊。她出现了新发的躯干性共济失调、右侧偏瘫和右侧感觉障碍,与之前的中风有关。她的小腿略微肿胀,呈红色外观。她的既往病史包括因严重二尖瓣反流而行二尖瓣置换术。她的白细胞计数为 8600/μL,主要为嗜酸性粒细胞(4480/μL;52.1%)。血清非特异性免疫球蛋白 E 升高至 1600 IU/mL(正常值<170 IU/mL)。她因二级预防中风而服用华法林,入院时凝血酶原时间国际标准化比值为 3.06。弥散加权磁共振成像显示右侧小脑有新鲜梗死灶。磁共振血管造影或颈动脉超声均未显示颈内动脉狭窄或闭塞。除心房颤动外,未发现其他栓塞性疾病。入院第 3 天,她的下肢出现广泛的瘙痒性、紫癜性皮疹。皮疹自行消退并反复发作数周。紫癜性皮损的皮肤活检标本显示真皮内大量嗜酸性粒细胞浸润和小血管的嗜酸性粒细胞血管炎。我们诊断该患者为变应性肉芽肿性血管炎(CSS)。皮质类固醇治疗后皮肤病变和嗜酸性粒细胞增多症消失。在本例中,尽管抗凝治疗得到很好的控制,但仍发生小脑梗死伴紫癜性皮疹。CSS 恶化时,CSS 患者可能会发生缺血性中风。

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