January 4, 2016: Neurologic manifestations of hemolytic uremic syndrome
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.
- 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.
- 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.