Neurological outcome in pediatric patients with hemolytic uremic syndrome caused by Escherichia coli O104:H4

Aims: This study analyzes the central nervous system (CNS) involvement in pediatric patients with hemolytic uremic syndrome (STEC-HUS) during the German epidemic 2011 caused by Escherichia coli O104:H4 and compares the neurological outcome between patients with and without CNS involvement. Methods: Data of 50 patients aged 11.9 (1.3 to 17.5) years treated in two hospitals in northern Germany were analyzed regarding the acute disease and after 3 and 6 months of follow-up. Neurological symptoms and electroencephalography (EEG) were evaluated. In addition 25 children underwent neuropsychological testing (WISC IV) 6 to 9 months after HUS. Results: Acute CNS involvement was observed in 14/50 (28%) patients; stupor or coma in 11, seizures in 11, visual disturbances in 4, hemiparesis in 2, and myocloni in 3. EEG was recorded in 39/50 and was abnormal more frequently in patients with CNS involvement than in those without (12/14 vs. 13/25, p = 0.03). Around 3 and 6 months after HUS 28/42 (67%), respectively, 17/39 (44%) patients complained about ongoing reduced performance, EEG was abnormal in 14/40 (35%), respectively, 7/36 (19%). Only 1 patient showed persistent hemiparesis. Neuropsychological testing showed a slightly lower global IQ in patients with CNS involvement than in those without (113.4 ± 2.8 vs. 119.4 ± 1.8, p = 0.07), mainly because of a reduced processing speed index. Conclusion: CNS involvement was frequent in pediatric STEC-HUS patients. Although major neurological sequels were rare and neuropsychological outcome favorable, patients with as well as without CNS involvement complained about reduced performance 3 to 6 months following HUS.