Objectives

This study analyses the neurological involvement and the mid-term outcome in paediatric patients with haemolytic uraemic syndrome during the German epidemic 2011 caused by E. coli O104:H4.

Design, Setting, Patients

Data regarding clinical signs, magnetic resonance imaging (MRI) and electroencephalography (EEG) findings during the acute disease were analysed in 50 patients aged 1.3–17.5 years (median 11.9). Follow-up visits were performed after 3 and 6 months. In addition 25 children underwent neuropsychological testing (WISC IV) after 6 to 9 months of follow-up.

Results

Neurological involvement was observed in 14/50 (28%) patients, including stupor or coma in 11/50 (22%), seizures in 11/50 (22%), visual disturbances in 4/50 (8%), hemiparesis in 2/50 (4%), and myocloni in 3/50 (6%). One patient died and one developed residual hemiparesis. EEG was more frequently abnormal in patients with neurological involvement (12/14 vs. 13/25, p=0.03). Cranial MRI was obtained in 11 patients with neurological involvement with abnormal findings in 5. After 3 and 6 months, 28/42 (67%) and 17/39 (44%) of the patients complained about on-going reduced performance. Neuropsychological testing showed a slightly lower global intelligence quotient in patients with neurological involvement (113.4±2.8 vs. 119.4±1.8, p=0.07), mainly due to a reduced processing speed index.

Conclusions

Neurological involvement was frequent in paediatric patients with haemolytic uraemic syndrome caused by E. coli O104:H4, but there was a high incidence of pathological EEG-findings even in patients without clinical signs of neurological involvement in the acute phase. Major neurological sequelae were rare and neuropsychological outcome favorable after 6 months.