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February 18, 2014; 82 (7) Article

Clinical and radiologic features of encephalopathy during 2011 E coli O111 outbreak in Japan

Jun-ichi Takanashi, Hiromichi Taneichi, Takako Misaki, Yuichiro Yahata, Akihisa Okumura, Yoh-ichi Ishida, Toshio Miyawaki, Nobuhiko Okabe, Tetsutaro Sata, Masashi Mizuguchi
First published January 17, 2014, DOI: https://doi.org/10.1212/WNL.0000000000000120
Jun-ichi Takanashi
From the Department of Pediatrics (J.-i.T.), Kameda Medical Center, Kamogawa; Department of Pediatrics (H.T., T. Miyawaki), University of Toyama; Infectious Disease Surveillance Center (T. Misaki, Y.Y., N.O.), National Institute of Infectious Diseases, Tokyo; Department of Pediatrics (A.O.), Juntendo University Faculty of Medicine, Tokyo; Department of Nephrology (Y.-i.I.), Toyama City Hospital, Toyama; Toyama Institute of Health (T.S.), Toyama; Department of Developmental Medical Sciences (M.M.), Graduate School of Medicine, the University of Tokyo, Japan.
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Hiromichi Taneichi
From the Department of Pediatrics (J.-i.T.), Kameda Medical Center, Kamogawa; Department of Pediatrics (H.T., T. Miyawaki), University of Toyama; Infectious Disease Surveillance Center (T. Misaki, Y.Y., N.O.), National Institute of Infectious Diseases, Tokyo; Department of Pediatrics (A.O.), Juntendo University Faculty of Medicine, Tokyo; Department of Nephrology (Y.-i.I.), Toyama City Hospital, Toyama; Toyama Institute of Health (T.S.), Toyama; Department of Developmental Medical Sciences (M.M.), Graduate School of Medicine, the University of Tokyo, Japan.
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Takako Misaki
From the Department of Pediatrics (J.-i.T.), Kameda Medical Center, Kamogawa; Department of Pediatrics (H.T., T. Miyawaki), University of Toyama; Infectious Disease Surveillance Center (T. Misaki, Y.Y., N.O.), National Institute of Infectious Diseases, Tokyo; Department of Pediatrics (A.O.), Juntendo University Faculty of Medicine, Tokyo; Department of Nephrology (Y.-i.I.), Toyama City Hospital, Toyama; Toyama Institute of Health (T.S.), Toyama; Department of Developmental Medical Sciences (M.M.), Graduate School of Medicine, the University of Tokyo, Japan.
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Yuichiro Yahata
From the Department of Pediatrics (J.-i.T.), Kameda Medical Center, Kamogawa; Department of Pediatrics (H.T., T. Miyawaki), University of Toyama; Infectious Disease Surveillance Center (T. Misaki, Y.Y., N.O.), National Institute of Infectious Diseases, Tokyo; Department of Pediatrics (A.O.), Juntendo University Faculty of Medicine, Tokyo; Department of Nephrology (Y.-i.I.), Toyama City Hospital, Toyama; Toyama Institute of Health (T.S.), Toyama; Department of Developmental Medical Sciences (M.M.), Graduate School of Medicine, the University of Tokyo, Japan.
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Akihisa Okumura
From the Department of Pediatrics (J.-i.T.), Kameda Medical Center, Kamogawa; Department of Pediatrics (H.T., T. Miyawaki), University of Toyama; Infectious Disease Surveillance Center (T. Misaki, Y.Y., N.O.), National Institute of Infectious Diseases, Tokyo; Department of Pediatrics (A.O.), Juntendo University Faculty of Medicine, Tokyo; Department of Nephrology (Y.-i.I.), Toyama City Hospital, Toyama; Toyama Institute of Health (T.S.), Toyama; Department of Developmental Medical Sciences (M.M.), Graduate School of Medicine, the University of Tokyo, Japan.
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Yoh-ichi Ishida
From the Department of Pediatrics (J.-i.T.), Kameda Medical Center, Kamogawa; Department of Pediatrics (H.T., T. Miyawaki), University of Toyama; Infectious Disease Surveillance Center (T. Misaki, Y.Y., N.O.), National Institute of Infectious Diseases, Tokyo; Department of Pediatrics (A.O.), Juntendo University Faculty of Medicine, Tokyo; Department of Nephrology (Y.-i.I.), Toyama City Hospital, Toyama; Toyama Institute of Health (T.S.), Toyama; Department of Developmental Medical Sciences (M.M.), Graduate School of Medicine, the University of Tokyo, Japan.
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Toshio Miyawaki
From the Department of Pediatrics (J.-i.T.), Kameda Medical Center, Kamogawa; Department of Pediatrics (H.T., T. Miyawaki), University of Toyama; Infectious Disease Surveillance Center (T. Misaki, Y.Y., N.O.), National Institute of Infectious Diseases, Tokyo; Department of Pediatrics (A.O.), Juntendo University Faculty of Medicine, Tokyo; Department of Nephrology (Y.-i.I.), Toyama City Hospital, Toyama; Toyama Institute of Health (T.S.), Toyama; Department of Developmental Medical Sciences (M.M.), Graduate School of Medicine, the University of Tokyo, Japan.
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Nobuhiko Okabe
From the Department of Pediatrics (J.-i.T.), Kameda Medical Center, Kamogawa; Department of Pediatrics (H.T., T. Miyawaki), University of Toyama; Infectious Disease Surveillance Center (T. Misaki, Y.Y., N.O.), National Institute of Infectious Diseases, Tokyo; Department of Pediatrics (A.O.), Juntendo University Faculty of Medicine, Tokyo; Department of Nephrology (Y.-i.I.), Toyama City Hospital, Toyama; Toyama Institute of Health (T.S.), Toyama; Department of Developmental Medical Sciences (M.M.), Graduate School of Medicine, the University of Tokyo, Japan.
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Tetsutaro Sata
From the Department of Pediatrics (J.-i.T.), Kameda Medical Center, Kamogawa; Department of Pediatrics (H.T., T. Miyawaki), University of Toyama; Infectious Disease Surveillance Center (T. Misaki, Y.Y., N.O.), National Institute of Infectious Diseases, Tokyo; Department of Pediatrics (A.O.), Juntendo University Faculty of Medicine, Tokyo; Department of Nephrology (Y.-i.I.), Toyama City Hospital, Toyama; Toyama Institute of Health (T.S.), Toyama; Department of Developmental Medical Sciences (M.M.), Graduate School of Medicine, the University of Tokyo, Japan.
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Masashi Mizuguchi
From the Department of Pediatrics (J.-i.T.), Kameda Medical Center, Kamogawa; Department of Pediatrics (H.T., T. Miyawaki), University of Toyama; Infectious Disease Surveillance Center (T. Misaki, Y.Y., N.O.), National Institute of Infectious Diseases, Tokyo; Department of Pediatrics (A.O.), Juntendo University Faculty of Medicine, Tokyo; Department of Nephrology (Y.-i.I.), Toyama City Hospital, Toyama; Toyama Institute of Health (T.S.), Toyama; Department of Developmental Medical Sciences (M.M.), Graduate School of Medicine, the University of Tokyo, Japan.
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Citation
Clinical and radiologic features of encephalopathy during 2011 E coli O111 outbreak in Japan
Jun-ichi Takanashi, Hiromichi Taneichi, Takako Misaki, Yuichiro Yahata, Akihisa Okumura, Yoh-ichi Ishida, Toshio Miyawaki, Nobuhiko Okabe, Tetsutaro Sata, Masashi Mizuguchi
Neurology Feb 2014, 82 (7) 564-572; DOI: 10.1212/WNL.0000000000000120

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Abstract

Objective: To elucidate the clinical and radiologic features and analyze factors associated with neurologic outcomes of encephalopathy secondary to Shiga toxin–producing Escherichia coli (STEC) O111.

Methods: We reviewed medical records and neuroimaging in 22 patients with neurologic symptoms among 86 with STEC O111 infection.

Results: Twenty-one (6 males and 15 females, 10 children and 11 adults) of the 22 patients were diagnosed with encephalopathy. All patients with encephalopathy also presented with hemolytic-uremic syndrome. Five patients died, from day 1 to 6 months (days 1–5 in 4 patients), due to progressive encephalopathy with severe cerebral edema observed in neuroimaging (4 patients). Fifteen of the 16 surviving patients clinically recovered completely. Statistical analysis revealed differences between patients with poor (n = 6) and good (n = 15) outcomes in the interval from hemolytic-uremic syndrome presentation to encephalopathy, creatinine levels, and the methylprednisolone administration ratio.

Conclusion: We note a high incidence of encephalopathy in the Toyama STEC O111 outbreak. All fatal cases resulted from progressive encephalopathy. Methylprednisolone pulse therapy represents a possible therapeutic choice.

Classification of evidence: This study provides Class III evidence that methylprednisolone pulse therapy increases the probability of a good outcome for patients with encephalopathy associated with STEC O111.

GLOSSARY

ADC=
apparent diffusion coefficient;
Gb3=
globotriaosylceramide;
HUS=
hemolytic-uremic syndrome;
IL-1β=
interleukin-1β;
IVIg=
IV immunoglobulin;
mPSL=
methylprednisolone;
STEC=
Shiga toxin–producing Escherichia coli;
Stx=
Shiga toxin;
TNF-α=
tumor necrosis factor-α

Footnotes

  • Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Supplemental data at www.neurology.org

  • Received April 11, 2013.
  • Accepted in final form November 12, 2013.
  • © 2014 American Academy of Neurology
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