January 4, 2016: Neurologic manifestations of hemolytic uremic syndrome

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January 4, 2016

Neurologic manifestations of hemolytic uremic syndrome

Hemolytic uremic syndrome (HUS) is a rare, complement-mediated disorder manifesting with acute kidney injury, hemolytic anemia, and thrombocytopenia. The classic form of the disorder results after infection with shiga toxin-producing E. coli. Though, a smaller percentage of patient's demonstrate overactivation of the alternative complement pathway without associated infection, termed atypical HUS. Neurologic complications are found in up to 48% of patients with atypical HUS, and can include seizures and encephalopathy. Treatment of HUS includes plasma exchange, though neurologic symptoms often fail to respond to initial therapy. Eculizumab is a monoclonal antibody to complement protein C5. In small series, eculizumab can reverse neurologic symptoms in atypical HUS, providing class IV evidence for its use in this disorder.

  1. Gulleroglu K, Fidan K, Hancer VS, et al. Neurologic involvement in atypical hemolytic uremic syndrome and successful treatment with eculizumab. Pediatr Nephrol 2013; 28: 827-830.
  2. Zuber J, Fakhouri F, Roumenina LT, et al. Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathies. Nat Rev Nephrol 2012; 8: 643-657.

Submitted by Adam Numis, MD, University of California, Los Angeles.

Disclosures: Dr. Numis is a member of the Resident and Fellow Section Neurology.

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