CNS Manifestations of the Hemolytic-Uremic Syndrome

Abstract

To define the CNS manifestations of the hemolytic-uremic syndrome (HUS), the records of 61 consecutive episodes of HUS in 60 patients were reviewed. Major CNS signs (seizures and/or coma) occurred in 30 of the 61 episodes. Twenty-four children had seizures. Analysis of blood pressure and metabolic parameters or admission to the hospital did not predict which child would exhibit CNS signs. However, during the subsequent course of the illness, children with CNS dysfunction had lower minimum serum sodium concentrations and more severe azotemia and were more likely to require dialysis than children without CNS signs. Of the 61 episodes, there were three deaths, and three children suffered major CNS sequelae. Coma at the time of admission and elevated CSF protein levels were features associated with poor outcome.

Deleterious consequences of Shiga toxin in the CNS

“…A mechanical connec tion between BBB and cerebrospinal fluid (CSF) disruptions was demonstrated by the presence of circulating Stx in the CSF of rabbits and mice, which suggests movement from the choroid plexus across the ventricular ependymal layer to brain neural cells (117,118). An increase in protein concentration in the CSF of STEC patients (119)(120)(121) and in previously described experimental models (117,118) has also been observed, which may support the idea of BBB-CSF weakening.…”

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%.…”

Neurological Sequelae in Adults After E coli O104

“… 19 Most frequent are hemiparesis, cortical blindness, and epilepsy. 20 , 21 In contrast none of our adult patients showed seizures or clinical signs such as paresis or aphasia in the long-term follow-up. However, 27% of our patients reported visual disturbances similar to those previously observed in preschool children.…”