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Neurological involvement in a child with atypical hemolytic uremic syndrome

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Abstract

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 × 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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References

  1. Eriksson KJ, Boyd SG, Tasker RC (2001) Acute neurology and neurophysiology of haemolytic-uraemic syndrome. Arch Dis Child 84:434–435

    Article  CAS  PubMed  Google Scholar 

  2. Chou MC, Lai PH, Yeh LR, Li JY, Yuan MK, Liang HL, Chen C, Pan HB, Lo YK, Yang CF (2004) Posterior reversible encephalopathy syndrome: magnetic resonance imaging and diffusion-weighted imaging in 12 cases. Kaohsiung J Med Sci 20:381–388

    Article  PubMed  Google Scholar 

  3. Gomez-Lado C, Martinon-Torres F, Alvarez-Moreno A, Eiris-Punal J, Carreira-Sande N, Rodriguez-Nunez A, Castro-Gago M (2007) Reversible posterior leukoencephalopathy syndrome: an infrequent complication in the course of haemolytic-uremic syndrome. Rev Neurol 44:475–478

    CAS  PubMed  Google Scholar 

  4. Bas DF, Oguz KK, Topcuoglu MA (2008) Atypical reversible posterior leukoencephalopathy syndrome in thrombotic thrombocytopenic purpura. Intern Med 47:1931–1934

    Article  PubMed  Google Scholar 

  5. Kato A, Suzuki Y, Fujigaki Y, Yamamoto T, Yonemura K, Miyajima H, Hishida A (2002) Thrombotic thrombocytopenic purpura associated with mixed connective tissue disease. Rheumatol Int 22:122–125

    Article  PubMed  Google Scholar 

  6. Barnett ND, Kaplan AM, Bernes SM, Cohen ML (1995) Hemolytic uremic syndrome with particular involvement of basal ganglia and favorable outcome. Pediatr Neurol 12:155–158

    Article  CAS  PubMed  Google Scholar 

  7. DiMario FJ Jr, Bronte-Stewart H, Sherbotie J, Turner ME (1987) Lacunar infarction of the basal ganglia as a complication of hemolytic-uremic syndrome. MRI and clinical correlations. Clin Pediatr (Phila) 26:586–590

    Article  Google Scholar 

  8. Hager A, Staudt M, Klare B, von Einsiedel HG, Krageloh-Mann I (1999) Hemolytic-uremic syndrome with involvement of basal ganglia and cerebellum. Neuropediatrics 30:210–213

    Article  CAS  PubMed  Google Scholar 

  9. Jeong YK, Kim IO, Kim WS, Hwang YS, Choi Y, Yeon KM (1994) Hemolytic uremic syndrome: MR findings of CNS complications. Pediatr Radiol 24:585–586

    Article  CAS  PubMed  Google Scholar 

  10. Nakamura H, Takaba H, Inoue T, Saku Y, Saito F, Ibayashi S, Fujishima M (2003) MRI findings of hemolytic uremic syndrome with encephalopathy: widespread symmetrical distribution. J Neuroimaging 13:75–78

    PubMed  Google Scholar 

  11. Ogura H, Takaoka M, Kishi M, Kimoto M, Shimazu T, Yoshioka T, Sugimoto H (1998) Reversible MR findings of hemolytic uremic syndrome with mild encephalopathy. AJNR Am J Neuroradiol 19:1144–1145

    CAS  PubMed  Google Scholar 

  12. Signorini E, Lucchi S, Mastrangelo M, Rapuzzi S, Edefonti A, Fossali E (2000) Central nervous system involvement in a child with hemolytic uremic syndrome. Pediatr Nephrol 14:990–992

    Article  CAS  PubMed  Google Scholar 

  13. Steinborn M, Leiz S, Rudisser K, Griebel M, Harder T, Hahn H (2004) CT and MRI in haemolytic uraemic syndrome with central nervous system involvement: distribution of lesions and prognostic value of imaging findings. Pediatr Radiol 34:805–810

    Article  PubMed  Google Scholar 

  14. Theobald I, Kuwertz-Broking E, Schiborr M, Heindel W (2001) Central nervous system involvement in hemolytic uremic syndrome (HUS)—a retrospective analysis of cerebral CT and MRI studies. Clin Nephrol 56:S3–S8

    CAS  PubMed  Google Scholar 

  15. Toldo I, Manara R, Cogo P, Sartori S, Murer L, Battistella PA, Laverda AM (2009) Diffusion-weighted imaging findings in hemolytic uremic syndrome with central nervous system involvement. J Child Neurol 24:247–250

    Article  PubMed  Google Scholar 

  16. Casey SO, Sampaio RC, Michel E, Truwit CL (2000) Posterior reversible encephalopathy syndrome: utility of fluid-attenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions. AJNR Am J Neuroradiol 21:1199–1206

    CAS  PubMed  Google Scholar 

  17. Ren J, Utsunomiya I, Taguchi K, Ariga T, Tai T, Ihara Y, Miyatake T (1999) Localization of verotoxin receptors in nervous system. Brain Res 825:183–188

    Article  CAS  PubMed  Google Scholar 

  18. Takahashi K, Funata N, Ikuta F, Sato S (2008) Neuronal apoptosis and inflammatory responses in the central nervous system of a rabbit treated with Shiga toxin-2. J Neuroinflammation 5:11

    Article  PubMed  Google Scholar 

  19. Venables JP, Strain L, Routledge D, Bourn D, Powell HM, Warwicker P, Diaz-Torres ML, Sampson A, Mead P, Webb M, Pirson Y, Jackson MS, Hughes A, Wood KM, Goodship JA, Goodship TH (2006) Atypical haemolytic uraemic syndrome associated with a hybrid complement gene. PLoS Med 3:e431

    Article  PubMed  Google Scholar 

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Correspondence to Patrick Niaudet.

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Koehl, B., Boyer, O., Biebuyck-Gougé, N. et al. Neurological involvement in a child with atypical hemolytic uremic syndrome. Pediatr Nephrol 25, 2539–2542 (2010). https://doi.org/10.1007/s00467-010-1606-y

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  • DOI: https://doi.org/10.1007/s00467-010-1606-y

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