Neurological Complications of Hemolytic-Uremic Syndrome

Abstract

Of 78 children identified with hemolytic-uremic syndrome at the Children's Hospital, Boston, from 1976 to 1986, 16 patients (20.5%) had neurological manifestations during their hospitalization. The most common manifestations were significant alterations in consciousness (coma, stupor) in 12 patients, and either generalized or partial seizures in ten patients. Others included hemiplegia (4 patients), decerebrate posturing (3), cortical blindness (2), hallucinations (1), and dystonic posturing (1). Cranial computed tomographic scans were abnormal in eight of 11 patients scanned. The abnormalities included diffuse cerebral edema (4 patients), large vessel infarctions (3), diffuse multiple small infarcts (4), and multiple hemorrhages (1). Five patients died as a result of their central nervous system complications, and six had neurological sequelae at discharge. Five patients recovered and at discharge had no evidence of neurological dysfunction.

Acute Neurological Involvement in Diarrhea-Associated Hemolytic Uremic Syndrome

“…This prevalence is significantly lower than those estimated in the seven series reported in the literature, but one should keep in mind that (1) the figures given by the French national survey are reliable, (2) our series is supposed to be complete because every pediatric nephrology unit has been directly and individually contacted for this series, and (3) isolated seizures were excluded from our series. The prognosis of neurologic involvement in our series was intermediate among the three most important series (6,8,11) and those of Hahn et al (10). The age of patients who died or continued to have severe disabilities was not significantly lower compared with patients who fully recovered in our series as well in all series of the literature.…”

“…A search for DϩHUS with neurologic involvement in the literature resulted in retrieval of seven series that totaled approximately 150 patients (5)(6)(7)(8)(9)(10)(11). The French national survey of DϩHUS numbered 1308 cases between 1993 and 2007, and our patients, who were recruited from the same centers that participate to the French survey, include 39 cases in the same period, suggesting that the frequency of neurologic involvement is approximately 3%.…”

Haemolytic-Uraemic Syndrome: Neurologic Symptoms, Neuroimaging and Neurocognitive Outcome

“…Clinical course of patients with these lesions was favorable, and MRI lesions became smaller on follow-up controls. Brain MRI sequenced controls of patients 6 and 21 reinforce the hypothesis of vasculitic lesion as the main cause of tissue damage, although direct neuronal toxicity could not be disclosed (Hahn et al, 1989). In contrast with previous reports (Theobald et al, 2001), basal ganglia necrosis has not proved to be a bad prognosis factor in our series: our patients did not present extrapyramidal signs, as other authors have reported (Di Mario et al, 1987;Barnett et al, 1995).…”

“…VT receptors are present in various troncoencephalic nuclei, the amygdala and the hippocampus, and in the posterior root neurons of the ganglia. This suggests VT may induce primary neuronal damage as well as a vasculitic lesion (Hahn et al, 1989;Hamano et al, 1993;Rivero et al, 2004). This probably happens also at the basal ganglia, especially at putamen nucleae, the most frequent localization of CNS lesions (Nakamura et al, 2003).…”

“…Physiopathology is not yet well understood, but experimental and in vivo studies (Ren et al, 1999;Carter, 1986;Cimolai, 1896) have proved that Escherichia coli VT induces thrombopenia through consuming, kidnapping, aggregation and platelet dysfunction mechanisms; plasminogen inhibitor activity is also enhanced, and therefore fibrinolysis is inhibited. Released factors such as TNF, IL, FvW monomers, free radicals, thromboxane, etc., provoke endothelial lesions and vasculitic events in several organs, especially kidneys, digestive system and brain (Seth et al, 1896;Miller & Kin, 1987;Montoliu, 1989;Hahn et al, 1989;Erikson et al, 2001;Steinborn et al, 2004;Rivero et al, 2004). VT receptors are present in various troncoencephalic nuclei, the amygdala and the hippocampus, and in the posterior root neurons of the ganglia.…”