Devilat M, Toso M, Morales M
Neuropediatric Unit, Military Hospital, Santiago, Chile.
Pediatr Neurol. 1993 Jan-Feb;9(1):67-70. doi: 10.1016/0887-8994(93)90015-5.
Two girls, 22 months and 12 years of age, presented with repeated cerebral infarctions in association with primary antiphospholipid syndrome. The younger patient also suffered from protein C deficiency, while the other one had protein S and complement C4 deficiencies. All other causes of cerebral infarction were excluded; however, vasculitis remains a possibility in one patient. Both girls developed spastic tetraparesis as a sequela of the previous infarctions. The two patients were treated with aspirin and prednisone, with remission of the infarctions during the next 8 months of observation. A primary deficiency of protein C or S is proposed which would produce cerebral thrombosis with exposure of phospholipids; this thrombosis then, like antigens, would generate antibodies acting on the thrombin-thrombomodulin complex, exacerbating the thrombotic process. The association of complement C4 deficiency is an additional risk factor.
两名女童，分别为22个月和12岁，因原发性抗磷脂综合征出现反复脑梗死。年幼患者还患有蛋白C缺乏症，而另一名患者有蛋白S和补体C4缺乏症。排除了所有其他脑梗死病因；不过，其中一名患者仍有可能患有血管炎。两名女童均因先前梗死遗留痉挛性四肢瘫。两名患者接受了阿司匹林和泼尼松治疗，在接下来8个月的观察期内梗死症状缓解。有人提出蛋白C或S原发性缺乏会在磷脂暴露时导致脑血栓形成；这种血栓形成随后会像抗原一样产生作用于凝血酶 - 血栓调节蛋白复合物的抗体，从而加剧血栓形成过程。补体C4缺乏的关联是另一个风险因素。
