Ataia I, Casaulta C, von Vigier R O, Pfammatter J P, Brekenfeld C, Sauvain M J, Steinlin M
Medizinische Universitäts-Kinderklinik, Inselspital, Bern.
Praxis (Bern 1994). 2008 Mar 19;97(6):322-5. doi: 10.1024/1661-8157.97.6.322.
A 13-year-old girl presented to our emergency with a one week history of fever and skin rash and new onset of chorea for the last three days. There was a long standing history of right predominant headache; followed by personality change, fatigue, arthralgia and weight loss over the last few months. Previous investigations by head CT and ophthalmological examination did not explain the symptoms. Further investigations revealed peri- and pancarditis with aortic insufficiency, a renal involvement with elevated creatinin, protein- and hematuria and a hemolytic anemia. Diagnosis of lupus eythematodes was confirmed by high ANA, anti-dsDNS and Anticardiolipin antibodies. Within the first 48 hours after admission there was significant deterioration with reduced vigilance and dysarthria. MRI of the brain and dopplersonography of cerebral vessels showed a complete thrombosis of the right medial cerebral artery with a small net of collaterals, irregularities of the left cerebral artery due to vasculitis and several subacute leftsided ischemias. Immunosuppressive therapy with high-dose corticosteroids and cyclophosphamid together with antithrombotic therapy induced an improvement of neurologic, renal and cardiac function.
一名13岁女孩因发热和皮疹一周、近三天出现新的舞蹈病症状前来我院急诊科就诊。她有长期以右侧为主的头痛病史,在过去几个月里还出现了性格改变、疲劳、关节痛和体重减轻。此前的头部CT和眼科检查均无法解释这些症状。进一步检查发现有全心炎伴主动脉瓣关闭不全、肾脏受累表现为肌酐升高、蛋白尿和血尿以及溶血性贫血。高滴度抗核抗体、抗双链DNA抗体和抗心磷脂抗体确诊为红斑狼疮。入院后48小时内病情显著恶化,出现意识不清和构音障碍。脑部MRI和脑血管多普勒超声显示右侧大脑中动脉完全血栓形成,伴有少量侧支循环,左侧大脑动脉因血管炎出现不规则改变,左侧有几处亚急性缺血灶。大剂量皮质类固醇和环磷酰胺免疫抑制治疗联合抗血栓治疗使神经、肾脏和心脏功能得到改善。