Original Article

Pediatric Nephrology

, Volume 29, Issue 9, pp 1607-1615

First online:

Neurological involvement in children with E. coli O104:H4-induced hemolytic uremic syndrome

  • Angela BauerAffiliated withDepartment of Pediatrics, University Medical Center Hamburg
  • , Sebastian LoosAffiliated withDepartment of Pediatrics, University Medical Center Hamburg
  • , Carola WehrmannAffiliated withDepartment of Child and Adolescent Psychiatry and Psychotherapy, University Medical Center Hamburg
  • , Dirk HorstmannAffiliated withDepartment of Pediatric Kidney, Liver, and Metabolic Diseases, Hannover Medical School
  • , Frank DonnerstagAffiliated withInstitute of Diagnostic and Therapeutic Neuroradiology, Hannover Medical School
  • , Johanna LemkeAffiliated withDepartment of Pediatrics, University Medical Center Hamburg
  • , Georg HillebrandAffiliated withDepartment of Pediatrics, University Medical Center Hamburg
  • , Ulrike LöbelAffiliated withDepartment of Neuroradiology, University Medical Center Hamburg
  • , Lars PapeAffiliated withDepartment of Pediatric Kidney, Liver, and Metabolic Diseases, Hannover Medical School
    • , Dieter HaffnerAffiliated withDepartment of Pediatric Kidney, Liver, and Metabolic Diseases, Hannover Medical School
    • , Carola BindtAffiliated withDepartment of Child and Adolescent Psychiatry and Psychotherapy, University Medical Center Hamburg
    • , Thurid AhlenstielAffiliated withDepartment of Pediatric Kidney, Liver, and Metabolic Diseases, Hannover Medical School
    • , Anette MelkAffiliated withDepartment of Pediatric Kidney, Liver, and Metabolic Diseases, Hannover Medical School
    • , Anja LehnhardtAffiliated withDepartment of Pediatrics, University Medical Center Hamburg
    • , Markus J. KemperAffiliated withDepartment of Pediatrics, University Medical Center Hamburg
    • , Jun OhAffiliated withDepartment of Pediatrics, University Medical Center HamburgPediatric Nephrology, Department of Pediatrics, University Medical Center Hamburg Email author 
    • , Hans HartmannAffiliated withDepartment of Pediatric Kidney, Liver, and Metabolic Diseases, Hannover Medical School

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Abstract

Background

The aim of this study was to analyze the neurological involvement and outcome in pediatric patients with hemolytic uremic syndrome (HUS) during the 2011 epidemic caused by Escherichia coli O104:H4.

Methods

Clinical data and data from magnetic resonance imaging (MRI) scans and electroencephalography (EEG) during the acute phase of the disease and during follow-up at 3 and 6 months were analyzed in 50 patients. Twenty-five of these patients underwent neuropsychological testing (WISC IV) during follow-up.

Results

Neurological involvement (stupor or coma, seizures, visual disturbances, paresis, myocloni) was initially observed in 14/50 (28 %) patients. One patient died. EEG abnormalities were more frequent in patients with neurological involvement than in those without (12/14 vs. 13/25, respectively). Cranial MRI scans were analyzed in nine patients with neurological involvement, of whom five showed abnormal findings. At the 3- and 6-month follow-ups, EEG abnormalities were found in 14/40 (35 %) and 7/36 (19 %) patients, respectively, whereas 28/42 (67 %) and 17/39 (44 %) patients, respectively, complained about on-going reduced performance. Neuropsychological testing showed a slightly lower global intelligence quotient in patients with neurological involvement versus those without (113.4 ± 2.8 vs. 119.4 ± 1.8, respectively).

Conclusions

Neurological involvement was frequent in our cohort. Accordingly, the incidence of pathological EEG findings was high, even in patients without clinical signs of neurological involvement. Nevertheless, major neurological sequelae were rare, and neuropsychological outcome was favorable after 6 months.

Keywords

Hemolytic uremic syndrome E. coli O104:H4 Children Neurology Outcome