Extensive hemispheric diffusion restriction in haemolytic uremic syndrome

Abstract

Haemolytic uremic syndrome (HUS) is a rare multi-organ disease affecting predominantly children between 1 and 4 years of age with an incidence of 1 per 50,000. It is caused by verotoxin-producing enterohaemorrhagic Escherichia coli (EHEC) infection. Neurological symptoms are reported in 20–50% of cases and represent the major cause of morbidity and mortality in patients with HUS. The neurological symptoms vary widely and may include visual disturbances, seizures, alteration of consciousness, hemiparesis, and brainstem symptoms. 1 Toldo I. Manara R. Cogo P. et al. Diffusion-weighted imaging findings in hemolytic uremic syndrome with central nervous system involvement. J Child Neurol. 2009; 24: 247-250 Crossref PubMed Scopus (13) Google Scholar The pathogenesis of the central nervous involvement is not fully understood. Putative mechanisms include thrombotic microangiopathy of cerebral arterioles, haemorrhage due to secondary coagulopathy, direct verotoxin neurotoxicity, and metabolic derangements. 1 Toldo I. Manara R. Cogo P. et al. Diffusion-weighted imaging findings in hemolytic uremic syndrome with central nervous system involvement. J Child Neurol. 2009; 24: 247-250 Crossref PubMed Scopus (13) Google Scholar , 2 Eriksson K.J. Boyd S.G. Tasker R.C. Acute neurology and neurophysiology of haemolytic-uraemic syndrome. Arch Dis Child. 2001; 84: 434-435 Crossref PubMed Scopus (59) Google Scholar , 3 Kaplan B.S. Meyers K.E. Schulman S.L. The pathogenesis and treatment of hemolytic uremic syndrome. J Am Soc Nephrol. 1998; 9: 1126-1133 PubMed Google Scholar , 4 Upadhyaya K. Barwick K. Fishaut M. et al. The importance of nonrenal involvement in hemolytic-uremic syndrome. Pediatrics. 1980; 65: 115-120 PubMed Google Scholar We report the case of child with typical HUS. Details of the clinical course and treatment have been reported by Lapeyraque et al. 5 Lapeyraque A.L. Malina M. Fremeaux-Bacchi V. et al. Complement blockade in severe Shiga-toxin-associated HUS. N Engl J Med. May 2011; PubMed Google Scholar