Neurological involvement in a child with atypical hemolytic uremic syndrome

Pediatr Nephrol. 2010 Dec;25(12):2539-42. doi: 10.1007/s00467-010-1606-y. Epub 2010 Aug 17.


We report the case of a 4-year-old boy, diagnosed with atypical hemolytic uremic syndrome (HUS) due to a hybrid factor H. He progressed to end-stage renal failure despite plasmatherapy and underwent bilateral nephrectomy because of uncontrolled hypertension. Three days after, he had partial complex seizures with normal blood pressure, normal blood count and normal magnetic resonance imaging (MRI), which recurred 1 month later. Eight months later, he had a third episode of seizures, with hemoglobin of 10 g/dl without schizocytes, low haptoglobin of 0.18 g/l, and moderate thrombocytopenia (platelets 98 × 10(9)/l). He remained hypertensive and deeply confused for 2 days. The third MRI showed bilateral symmetrical hyperintensities of the cerebral pedunculas, caudate nuclei, putamens, thalami, hippocampi, and insulae suggesting thrombotic microangiopathy secondary to a relapse of HUS rather than reversible posterior leukoencephalopathy syndrome (RPLS), usually occipital and asymmetrical. Plasmatherapy led to a complete neurological recovery within 2 days although hypertension had remained uncontrolled. The fourth MRI 10 weeks after, on maintenance plasmatherapy, was normal and clinical examination remained normal, except for high blood pressure. In conclusion, brain MRI allows differentiating thrombotic microangiopathy lesions from RPLS in atypical HUS, which is crucial since lesions may be reversible with plasmatherapy.

Publication types

  • Case Reports

MeSH terms

  • Atypical Hemolytic Uremic Syndrome
  • Child, Preschool
  • Complement Factor H / genetics
  • Diagnosis, Differential
  • Hemolytic-Uremic Syndrome / complications
  • Hemolytic-Uremic Syndrome / genetics
  • Hemolytic-Uremic Syndrome / therapy
  • Humans
  • Hypertension / etiology
  • Hypertension / therapy
  • Kidney Failure, Chronic / etiology*
  • Kidney Failure, Chronic / therapy
  • Magnetic Resonance Imaging
  • Male
  • Nephrectomy
  • Peritoneal Dialysis
  • Plasma Exchange
  • Posterior Leukoencephalopathy Syndrome / diagnosis
  • Predictive Value of Tests
  • Recurrence
  • Renal Dialysis
  • Seizures / etiology*
  • Seizures / pathology
  • Seizures / therapy
  • Thrombotic Microangiopathies / etiology*
  • Thrombotic Microangiopathies / pathology
  • Thrombotic Microangiopathies / therapy
  • Time Factors


  • Complement Factor H