Hue V, Leclerc F, Martinot A, Vallee L, Saunier P
Service de Réanimation Infantile, Hôpital A.-Calmette, Lille.
Arch Fr Pediatr. 1992 Apr;49(4):369-71.
Two children with hemolytic-uremic syndrome (HUS) presented with alteration of consciousness and severe abnormal movements related to striatal involvement. They required dialysis and controlled ventilation. Steady improvement in neurologic function was seen during the following weeks, and abnormal movements disappeared. It is suggested that the pathogenesis of neurological dysfunction in these two children was related to intracerebral microangiopathy, because neither systemic arterial hypertension, nor metabolic disturbances were present. This hypothesis is supported by the cerebral blood flow study in one patient. Neuropathologic studies of patients with HUS who presented with CNS symptoms showed vascular thromboses within the CNS: CT scan demonstrated multiple small areas of infarction, particularly in the basal ganglia. The prognosis of HUS is generally worsened in case of severe CNS involvement, but abnormal movements may disappear without sequelae.
两名溶血尿毒综合征（HUS）患儿出现意识改变及与纹状体受累相关的严重异常运动。他们需要透析及控制通气。在接下来的几周内，神经功能稳步改善，异常运动消失。提示这两名患儿神经功能障碍的发病机制与脑内微血管病变有关，因为既没有全身性动脉高血压，也没有代谢紊乱。这一假说得到了一名患者脑血流研究的支持。出现中枢神经系统症状的HUS患者的神经病理学研究显示中枢神经系统内有血管血栓形成：CT扫描显示多个小梗死灶，尤其是在基底神经节。严重中枢神经系统受累时，HUS的预后通常会恶化，但异常运动可能消失且不留后遗症。
