Article

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. METHOD: 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. INTERPRETATION: 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.
CI - (c) 2013 Mac Keith Press.

Langue : ANGLAIS