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October 02, 2012; 79 (14) Articles

Neurologic manifestations of E coli infection–induced hemolytic-uremic syndrome in adults

Karin Weissenborn, Frank Donnerstag, Jan T. Kielstein, Meike Heeren, Hans Worthmann, Hartmut Hecker, Roland Schmitt, Mario Schiffer, Thomas Pasedag, Ramona Schuppner, Anita B. Tryc, Peter Raab, Hans Hartmann, Xiaoqi Q. Ding, Carsten Hafer, Jan Menne, Bernhard M.W. Schmidt, Eva Bültmann, Hermann Haller, Reinhard Dengler, Heinrich Lanfermann, Anja M. Giesemann
First published September 19, 2012, DOI: https://doi.org/10.1212/WNL.0b013e31826d5f26
Karin Weissenborn
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Frank Donnerstag
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Jan T. Kielstein
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Meike Heeren
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Hans Worthmann
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Hartmut Hecker
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Roland Schmitt
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Mario Schiffer
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Thomas Pasedag
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Ramona Schuppner
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Anita B. Tryc
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Peter Raab
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Hans Hartmann
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Xiaoqi Q. Ding
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Carsten Hafer
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Jan Menne
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Bernhard M.W. Schmidt
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Eva Bültmann
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Hermann Haller
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Reinhard Dengler
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Heinrich Lanfermann
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Anja M. Giesemann
From the Clinic for Neurology (K.W., M.H., H.W., T.P., R. Schuppner, A.B.T., R.D.), Institute for Diagnostic and Interventional Neuroradiology (F.D., P.R., X.Q.D., E.B., H.L., A.M.G.), Department of Nephrology and Hypertension (J.T.K., R. Schmitt, M.S., C.H., J.M., B.M.W.S., H. Haller), Institute for Biometry (H. Hecker), and Clinic for Pediatric Nephrology, Hepatology, and Metabolic Disorders (H. Hartmann), Hannover Medical School, Hannover, Germany.
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Full PDF
Citation
Neurologic manifestations of E coli infection–induced hemolytic-uremic syndrome in adults
Karin Weissenborn, Frank Donnerstag, Jan T. Kielstein, Meike Heeren, Hans Worthmann, Hartmut Hecker, Roland Schmitt, Mario Schiffer, Thomas Pasedag, Ramona Schuppner, Anita B. Tryc, Peter Raab, Hans Hartmann, Xiaoqi Q. Ding, Carsten Hafer, Jan Menne, Bernhard M.W. Schmidt, Eva Bültmann, Hermann Haller, Reinhard Dengler, Heinrich Lanfermann, Anja M. Giesemann
Neurology Oct 2012, 79 (14) 1466-1473; DOI: 10.1212/WNL.0b013e31826d5f26

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Abstract

Objective: To describe the neurologic and neuroradiologic complications of Shiga toxin producing Escherichia coli infection (STEC)–associated hemolytic-uremic syndrome (HUS) in adults.

Methods: All 52 adult patients with STEC O104:H4 infection cared for at Hannover Medical School during the outbreak in Germany through May–July 2011 are considered in this observational study. Forty-three of the 52 patients underwent a standard neurologic diagnostic procedure including clinical examination, Mini-Mental State Examination, and Glasgow Coma Scale Score. Thirty-six patients underwent EEG, and 26 had cerebral MRI, 9 of them repeatedly. Case records of 9 patients who had not been seen by a neurologist were analyzed retrospectively.

Results: Forty-eight of the 52 patients had HUS. All but 1 of these showed neurologic symptoms. Focal neurologic signs like double vision, difficulties in finding words, or hyperreflexia were present in 23, additional deficits in orientation, attention, memory, or constructive abilities in 9, and marked impairment of consciousness in 15. MRI showed brainstem, midbrain, thalamus, corpus callosum, and white matter lesions in half of the patients, predominantly in diffusion-weighted images. The extent of MRI lesions did not correlate with clinical symptoms. General slowing but no focal alteration was found in half of the patients examined by EEG.

Conclusion: Our findings suggest a toxic-metabolic pathology behind the neurologic impairment instead of multiple infarction due to microthrombosis. Future studies should aim to clarify if early antibiotic therapy or bowel cleansing might help to decrease the rate of neurologic complications in STEC-HUS.

GLOSSARY

ADC=
apparent diffusion coefficient;
CRP=
C-reactive protein;
HUS=
hemolytic-uremic syndrome;
LDH=
lactate dehydrogenase;
MHH=
Hannover Medical School;
MMSE=
Mini-Mental State Examination;
mRS=
modified Rankin Scale;
PRES=
posterior reversible encephalopathy syndrome;
ROI=
region of interest;
STEC=
Shiga toxin producing Escherichia coli infection;
TPE=
therapeutic plasma exchange;
WBC=
white blood cell

Footnotes

  • Supplemental data at www.neurology.org

  • Received December 28, 2011.
  • Accepted May 15, 2012.
  • Copyright © 2012 by AAN Enterprises, Inc.
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Letters: Rapid online correspondence

  • Delirium or Encephalopathy in Hemolytic Uremic Syndrome
    • Paul J Regal, Physician Delirium Researcher, Senior Lecturer, University of Newcastle, Australiaregalfamily@bigpond.com
    Submitted October 29, 2012
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