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

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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.

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Acknowledgments

We thank all participating patients and families. We particularly thank all of the nursing, medical staff and colleagues in our hospitals, who worked so hard during the outbreak.

Funding

This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

Conflicts of interest

Both centers, UKE and MHH, enrolled a total of six patients in a single-arm multicenter trial on eculizumab treatment, which is sponsored by Alexion Pharmaceuticals (Cheshire, CT). Data on the treatment effect in these patients will be published elsewhere.

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Authors and Affiliations

  1. Department of Pediatrics, University Medical Center Hamburg, Hamburg, Germany

    Angela Bauer, Sebastian Loos, Johanna Lemke, Georg Hillebrand, Anja Lehnhardt, Markus J. Kemper & Jun Oh

  2. Department of Pediatric Kidney, Liver, and Metabolic Diseases, Hannover Medical School, Hannover, Germany

    Dirk Horstmann, Lars Pape, Dieter Haffner, Thurid Ahlenstiel, Anette Melk & Hans Hartmann

  3. Department of Child and Adolescent Psychiatry and Psychotherapy, University Medical Center Hamburg, Hamburg, Germany

    Carola Wehrmann & Carola Bindt

  4. Institute of Diagnostic and Therapeutic Neuroradiology, Hannover Medical School, Hannover, Germany

    Frank Donnerstag

  5. Department of Neuroradiology, University Medical Center Hamburg, Hamburg, Germany

    Ulrike Löbel

  6. Pediatric Nephrology, Department of Pediatrics, University Medical Center Hamburg, Martinistrasse 52, 20246, Hamburg, Germany

    Jun Oh

Authors
  1. Angela Bauer
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  2. Sebastian Loos
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  9. Lars Pape
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  15. Markus J. Kemper
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  16. Jun Oh
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  17. Hans Hartmann
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Corresponding author

Correspondence to Jun Oh.

Additional information

Angela Bauer and Sebastian Loos contributed equally to this work.

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Bauer, A., Loos, S., Wehrmann, C. et al. Neurological involvement in children with E. coli O104:H4-induced hemolytic uremic syndrome. Pediatr Nephrol 29, 1607–1615 (2014). https://doi.org/10.1007/s00467-014-2803-x

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  • DOI: https://doi.org/10.1007/s00467-014-2803-x

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