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Pediatr Nephrol (2010) 25:2539–2542DOI 10.1007/s00467-010-1606-y Neurological involvement in a child with atypical hemolyticuremic syndrome Bérengère Koehl & Olivia Boyer & Nathalie Biebuyck-Gougé & Manoelle Kossorotoff &Véronique Frémeaux-Bacchi & Nathalie Boddaert & Patrick Niaudet Received: 13 April 2010 / Revised: 1 July 2010 / Accepted: 2 July 2010 / Published online: 17 August 2010# IPNA 2010 Abstract We report the case of a 4-year-old boy, diagnosed the cerebral pedunculas, caudate nuclei, putamens, thalami, with atypical hemolytic uremic syndrome (HUS) due to a hippocampi, and insulae suggesting thrombotic microangi- hybrid factor H. He progressed to end-stage renal failure opathy secondary to a relapse of HUS rather than reversible despite plasmatherapy and underwent bilateral nephrectomy posterior leukoencephalopathy syndrome (RPLS), usually because of uncontrolled hypertension. Three days after, he occipital and asymmetrical. Plasmatherapy led to a com- had partial complex seizures with normal blood pressure, plete neurological recovery within 2 days although hyper- normal blood count and normal magnetic resonance tension had remained uncontrolled. The fourth MRI imaging (MRI), which recurred 1 month later. Eight months 10 weeks after, on maintenance plasmatherapy, was normal later, he had a third episode of seizures, with hemoglobin of and clinical examination remained normal, except for high 10 g/dl without schizocytes, low haptoglobin of 0.18 g/l, blood pressure. In conclusion, brain MRI allows differen- and moderate thrombocytopenia (platelets 98 × 109/l). He tiating thrombotic microangiopathy lesions from RPLS in remained hypertensive and deeply confused for 2 days. The atypical HUS, which is crucial since lesions may be third MRI showed bilateral symmetrical hyperintensities of Keywords Atypical hemolytic uremic syndrome .
B. Koehl : O. Boyer : N. Biebuyck-Gougé : P. Niaudet Cerebral thrombotic microangiopathy .
Reversible posterior leukoencephalopathy syndrome .
Hôpital Necker-Enfants Malades, Université Paris Descartes, Hôpital Necker-Enfants Malades,Paris, France Hemolytic uremic syndrome (HUS) is characterized by the triad of microangiopathic hemolytic anemia, thrombocyto- Hôpital Necker-Enfants Malades, Immunology Department, penia, and acute renal failure. In children, atypical HUS is Hôpital Européen-Georges Pompidou, Université Paris Descartes, often secondary to genetic abnormalities of the complement alternative pathway proteins. Among extra-renal complica- tions, central nervous system manifestations are observed in Pediatric Radiology, Hôpital Necker-Enfants Malades, 20% to 50% of cases and are responsible for increased P. Niaudet (*)Service de Néphrologie Pédiatrique, Hôpital Necker Enfants Malades,149 rue de Sèvres, We report the case of a previously healthy 4-year-old boy, 75743 Paris cedex 15, Francee-mail: first admitted because of a 3-week history of asthenia and vomiting, without diarrhea. On admission, he was anuric, hemodialysis, and antihypertensive treatment. He presented his blood pressure was 120/88 mmHg, and physical neurological symptoms for 2 days, including deep confu- examination was remarkable only for peripheral and sion and violent agitation. The clinical and biological palpebral edema. The first laboratory tests revealed: symptoms were more suggestive of hypertensive encepha- hemolytic anemia (hemoglobin 5.1 g/dl with schizocytes, lopathy than a HUS relapse, and a third MRI was haptoglobin 0.06 g/l), thrombocytopenia (platelets performed 2 days after the seizures (Fig. On FLAIR 108 × 109/l), renal insufficiency (plasma creatinine and T2 sequences, this brain MRI showed bilateral sym- 594 μmol/l, urea 42.6 mmol/l), and nephrotic syndrome metrical hyperintensities on the cerebral pedunculas, cau- (plasma albumin 23.5 g/l, proteinuria 23.4 g/l). Stool date nuclei, putamens, thalami, hippocampi, and insulae.
culture, polymerase chain reaction (PCR) of Shiga-toxin Diffusion-weighted imaging sequence and ADC mapping (Stx) in stools and detection of serum antibodies against displayed no abnormality. These findings were suggestive lipopolysaccharides (LPS) were negative. CH50, C3, C4, of thrombotic microangiopathy, probably due to a relapse complement factor H (CFH) antigen, factor I antigen, CD46 of HUS, rather than RPLS-related abnormalities.
expression, and von Willebrandt factor (vWF) protease He was immediately treated with daily plasma activity were normal. Genetic analysis of complement exchanges (PE) that led to a complete neurological protein genes was negative at that time but a CFH hybrid recovery within 2 days although hypertension had remained uncontrolled. He received daily PE for 1 week, progres- He was started on peritoneal dialysis and plasmatherapy.
sively tapered to twice a week. After a total of 10 sessions After three plasma infusions and seven plasma exchange within 4 weeks, plasma exchanges were replaced by weekly sessions, urine output gradually increased and plasma plasma infusions during hemodialysis sessions. The fourth creatinine progressively decreased to 117 μmol/l. He brain MRI after 10 weeks of weekly plasma infusion was remained severely hypertensive. Two weeks later, a first normal and clinical examination remained normal, except relapse was treated with seven plasma exchanges, which improved hematological parameters, but renal functionworsened. Hematological remission persisted, but despiteplasma exchanges twice a week for 4 months followed by plasma infusions twice a week for 2 months, he progressedto end-stage renal failure. Eight months after the onset, he Central nervous system involvement (irritability, drowsi- was started on chronic hemodialysis. One month later, he ness, convulsions, cortical blindness, hemiparesia or coma) underwent bilateral nephrectomy because of uncontrolled is the most frequent extrarenal complication in HUS, and hypertension despite five anti-hypertensive drugs. Three MRI findings are diverse. Two situations may be radiolog- days after nephrectomy, he presented partial complex ically delineated: RPLS-related lesions and microangio- epileptic seizures of unclear etiology, without hemolysis pathic lesions. The clinical symptoms in both situations are or thrombopenia. The blood pressure, controlled by antihypertensive treatment of IV nicardipine followed by Brain MRI RPLS-related lesions are associated with a oral labetalol and nifedipine, was at that time stable and posterior white matter hyperintensity and sometimes poste- normal. Brain magnetic resonance imaging (MRI) 3 days rior cortex hyperintensity probably secondary to high after seizures was normal. The electroencephalogram blood pressure during the acute phase of HUS. White- (EEG) showed a posterior basic rhythm disrupted by many matter hyperintensity is predominant in parieto-occipital slow and monomorphic theta and delta waves.
One month later, he had a recurrence of generalized Another type of brain MRI lesions is also described seizures without hemolysis (hemoglobin 9.4 g/dl without during the acute phase of HUS, with bilateral and schizocytes, platelet 220 × 109/l) or severe hypertension.
symmetrical thalami, brainstem, and deep white matter The second brain MRI, 6 days after admission, was normal.
involvement but with diffusion weighted imaging EEG was similar to the previous one, with posterior Bilateral basal ganglia involvement is characterized by abnormalities that could be consistent with reversible hypersignal on T2-weighted and hypo-signal on T1- posterior leukoencephalopathy syndrome (RPLS). He re- weighted images, sometimes extending to the surrounding ceived antiepileptic medication (Levetiracetam).
white matter [–Thalami and brainstem involvement Eight months later, he had a third episode of seizures, have also been described [, Although these lesions with decreased hemoglobin level (10 g/dl) without schizo- appear to be fairly characteristic of HUS, the pathogenic cytes, low but detectable haptoglobin of 0.18 g/l, and mechanism remains incompletely understood [ moderate thrombocytopenia (platelets 98 × 109/l). High In our patient presenting with atypical HUS, the MRI blood pressure persisted, despite bilateral nephrectomy, features were not consistent with the diagnosis of posterior Fig. 1 Brain MRI 2 days afterthe third seizures episode. a–c Axial T2-weighted sequences,b–d coronal FLAIR sequences,a, b bilateral cerebral pedunculahypersignal (arrows), c, dbilateral hypersignal of caudatenuclei (1), putamens (2) andthalami (3) reversible encephalopathy syndrome, although clinical and similar neurological events were secondary to three biological data were suggestive of hypertensive complica- independent causes. We may hypothesize a delay between tions. The MRI lesions were similar to those observed in clinical signs and radiological images or a lack of MRI typical HUS, suggesting a HUS relapse because the patient sensitivity. Thus, the normality of the fourth MRI does not displayed the characteristic bilateral and symmetrical basal rule out the persistence of brain TMA lesions, but the ganglia involvement described in the literature, with disappearance of the MRI hyperintensities observed after brainstem and thalami lesions also reported in a few the third burst of seizures is reassuring.
publications. The similarity of radiological lesions between We report the first case of atypical HUS with cerebral typical and atypical HUS favors a common pathogenesis, MRI images suggestive of TMA. In our patient, the genetic i.e. cerebral thrombotic microangiopathy. In typical HUS analyses found a hybrid CFH/CFHL1 gene. Similar to CFH (post-diarrhea HUS), it has been suggested that vascular point mutations, this hybrid gene is a susceptibility factor endothelial injury caused by Stx plays a crucial role in the for atypical HUS [Venables et al. ] described a development of neurological involvement , Since pedigree of eight individuals from four generations of the MRIs following the first and the second seizures were atypical HUS segregating with this hybrid CFH/CFHL1 normal, we cannot exclude other etiologies for the two first gene. Two displayed neurological symptoms: grand mal episodes, such as drug side effects (anesthetics, antibiotics) convulsion in a 19-year-old woman who died 8 weeks after or a hypertensive peak. However, drug doses were adapted presentation and a short history of headaches and lethargy to renal function, blood pressure was normal at the time of in a 28-year-old man. However, no imaging details are the seizures, and it seems very unlikely that the three provided. The prevalence of neurological involvement in this family with a hybrid CFH is not higher than that of 6. Barnett ND, Kaplan AM, Bernes SM, Cohen ML (1995) other CFH mutation-related HUS, suggesting that the Hemolytic uremic syndrome with particular involvement of basalganglia and favorable outcome. Pediatr Neurol 12:155–158 neurological involvement is secondary to the thrombotic 7. DiMario FJ Jr, Bronte-Stewart H, Sherbotie J, Turner ME (1987) microangiopathy and not a consequence of the genetic Lacunar infarction of the basal ganglia as a complication of defect. A larger number of patients with a hybrid CFH/ hemolytic-uremic syndrome. MRI and clinical correlations. Clin CFHL1 gene is needed to confirm this hypothesis. Brain 8. Hager A, Staudt M, Klare B, von Einsiedel HG, Krageloh-Mann I MRI findings were very useful to guide diagnosis and (1999) Hemolytic-uremic syndrome with involvement of basal therapeutic strategy in our patient, as it led to the diagnosis ganglia and cerebellum. Neuropediatrics 30:210–213 of an HUS relapse rather than hypertensive encephalopathy.
9. Jeong YK, Kim IO, Kim WS, Hwang YS, Choi Y, Yeon KM This was confirmed by the successful intensive plasma- (1994) Hemolytic uremic syndrome: MR findings of CNScomplications. Pediatr Radiol 24:585–586 therapy with complete clinical and radiological remission, 10. Nakamura H, Takaba H, Inoue T, Saku Y, Saito F, Ibayashi S, although blood pressure had remained uncontrolled.
Fujishima M (2003) MRI findings of hemolytic uremic syndrome In conclusion, brain MRI allows the differentiation of with encephalopathy: widespread symmetrical distribution. J thrombotic microangiopathy lesions from a hypertensive 11. Ogura H, Takaoka M, Kishi M, Kimoto M, Shimazu T, Yoshioka complication such as RPLS in patients with atypical HUS T, Sugimoto H (1998) Reversible MR findings of hemolytic and neurological manifestations. Therapeutic implications uremic syndrome with mild encephalopathy. AJNR Am J Neuro- are important, since treatment would consist of blood pressure control in one case and intensive plasmatherapy 12. Signorini E, Lucchi S, Mastrangelo M, Rapuzzi S, Edefonti A, Fossali E (2000) Central nervous system involvement in a child in the other case. An accurate diagnosis of neurological with hemolytic uremic syndrome. Pediatr Nephrol 14:990–992 involvement is particularly important as lesions may be 13. Steinborn M, Leiz S, Rudisser K, Griebel M, Harder T, Hahn H reversible after appropriate treatment.
(2004) CT and MRI in haemolytic uraemic syndrome with centralnervous system involvement: distribution of lesions and prognos-tic value of imaging findings. Pediatr Radiol 34:805–810 14. Theobald I, Kuwertz-Broking E, Schiborr M, Heindel W (2001) Central nervous system involvement in hemolytic uremic syn-drome (HUS)—a retrospective analysis of cerebral CT and MRIstudies. Clin Nephrol 56:S3–S8 1. Eriksson KJ, Boyd SG, Tasker RC (2001) Acute neurology and 15. Toldo I, Manara R, Cogo P, Sartori S, Murer L, Battistella PA, neurophysiology of haemolytic-uraemic syndrome. Arch Dis Laverda AM (2009) Diffusion-weighted imaging findings in hemolytic uremic syndrome with central nervous system involve- 2. Chou MC, Lai PH, Yeh LR, Li JY, Yuan MK, Liang HL, Chen C, Pan HB, Lo YK, Yang CF (2004) Posterior reversible encepha- 16. Casey SO, Sampaio RC, Michel E, Truwit CL (2000) Posterior lopathy syndrome: magnetic resonance imaging and diffusion- reversible encephalopathy syndrome: utility of fluid-attenuated weighted imaging in 12 cases. Kaohsiung J Med Sci 20:381–388 inversion recovery MR imaging in the detection of cortical and 3. Gomez-Lado C, Martinon-Torres F, Alvarez-Moreno A, Eiris- subcortical lesions. AJNR Am J Neuroradiol 21:1199–1206 Punal J, Carreira-Sande N, Rodriguez-Nunez A, Castro-Gago M 17. Ren J, Utsunomiya I, Taguchi K, Ariga T, Tai T, Ihara Y, (2007) Reversible posterior leukoencephalopathy syndrome: an Miyatake T (1999) Localization of verotoxin receptors in nervous infrequent complication in the course of haemolytic-uremic 18. Takahashi K, Funata N, Ikuta F, Sato S (2008) Neuronal apoptosis 4. Bas DF, Oguz KK, Topcuoglu MA (2008) Atypical reversible and inflammatory responses in the central nervous system of a posterior leukoencephalopathy syndrome in thrombotic thrombo- rabbit treated with Shiga toxin-2. J Neuroinflammation 5:11 cytopenic purpura. Intern Med 47:1931–1934 19. Venables JP, Strain L, Routledge D, Bourn D, Powell HM, 5. Kato A, Suzuki Y, Fujigaki Y, Yamamoto T, Yonemura K, Warwicker P, Diaz-Torres ML, Sampson A, Mead P, Webb M, Miyajima H, Hishida A (2002) Thrombotic thrombocytopenic Pirson Y, Jackson MS, Hughes A, Wood KM, Goodship JA, purpura associated with mixed connective tissue disease. Rheu- Goodship TH (2006) Atypical haemolytic uraemic syndrome associated with a hybrid complement gene. PLoS Med 3:e431


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