Authors: Bérengère Koehl Olivia Boyer Nathalie BiebuyckGougé Manoelle Kossorotoff Véronique FrémeauxBacchi Nathalie Boddaert Patrick Niaudet
Publish Date: 2010/08/17
Volume: 25, Issue: 12, Pages: 2539-2542
Abstract
We report the case of a 4yearold boy diagnosed with atypical hemolytic uremic syndrome HUS due to a hybrid factor H He progressed to endstage 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 018 g/l and moderate thrombocytopenia platelets 98 × 109/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
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