title: Brain magnetic resonance imaging pattern and outcome in children with haemolytic-uraemic syndrome and neurological impairment treated with eculizumab.
abstract: OBJECTIVE
  The aim of this study was to describe the magnetic resonance imaging (MRI) findings and the neurological and neuropsychological outcomes in paediatric, diarrhoea-associated haemolytic-uraemic syndrome (D+HUS) with central nervous system impairment treated with eculizumab, a monoclonal antibody.
  
  
  METHODS
  The 14-month single-centre prospective study included seven children (three males, four females; age range 16 mo-7 y 8 mo; median age 3 y 7 mo) with typical D+HUS and acute neurological impairment. In the acute phase of the disease, neurological assessment and brain magnetic resonance imaging (MRI), including measurement of the apparent diffusion coefficient (ADC), were performed, and neuropsychological evaluation and brain MRI were also carried out 6 months after disease onset.
  
  
  RESULTS
  In the acute phase, basal ganglia and white matter abnormalities with ADC restriction were a common and reversible MRI finding. In all the surviving patients (5/7), follow-up MRI after 6 months was normal, indicating reversible lesions. Clinical and neuropsychological evaluations after 6 months were also normal.
  
  
  CONCLUSIONS
  This specific brain MRI pattern consisting of an ADC decrease in basal ganglia and white matter without major T2/fluid-attenuated inversion recovery (FLAIR) injury may be a key finding in the acute phase of the disease in favour of a vasculitis hypothesis. These reversible lesions were associated with a good neurological outcome. These results call for further evaluation of the potential role of eculizumab in the choice of treatment for severe D+HUS, particularly in the case of early neurological signs.
authors: Pediatric Neurology Department, Necker-Enfants Malades Hospital, APHP, Paris, France. cyril.gitiaux@nck.aphp.fr
authors: Cyril
authors: C
authors: Gitiaux
authors: Pauline
authors: P
authors: Krug
authors: David
authors: D
authors: Grevent
authors: Manoelle
authors: M
authors: Kossorotoff
authors: Sarah
authors: S
authors: Poncet
authors: Monika
authors: M
authors: Eisermann
authors: Mehdi
authors: M
authors: Oualha
authors: Nathalie
authors: N
authors: Boddaert
authors: Remi
authors: R
authors: Salomon
authors: Isabelle
authors: I
authors: Desguerre
mesh_terms: D000208:Acute Disease; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D001480:Basal Ganglia Diseases / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D001921:Brain / Q000473:pathology* / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D051056:Complement Inactivating Agents / Q000627:therapeutic use; D038524:Diffusion Magnetic Resonance Imaging; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007223:Infant; D056784:Leukoencephalopathies / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D011446:Prospective Studies; D016896:Treatment Outcome
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D051056:Complement Inactivating Agents; C481642:eculizumab