[Striatal involvement with abnormal movements in hemolytic-uremic syndrome].

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.