Proximal Cerebral Artery Stenosis in a Patient with Hemolytic Uremic Syndrome

  • Vergouwen M
  • Adriani K
  • Roos Y
  • et al.
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Neurologic complications occur in 20%–50% of patients with hemo-lytic uremic syndrome (HUS). Macrovascular changes are not part of the syndrome. Here, we report a patient with a familial form of HUS who presented with hemodynamic transient ischemic attacks, as a result of severe proximal stenoses of both middle and anterior cere-bral arteries. A 15-year-old girl experienced neurologic symptoms several times in the past few months, consisting of sensory or motor symptoms on either side of the body, with a maximal duration of 5 minutes. All episodes occurred during hemodialysis. She developed end stage re-nal disease at 3 years of age due to nondiarrheal HUS (D-HUS) and was subsequently treated with peritoneal dialysis and frequent home hemodialysis. She had undergone renal transplantation twice; both renal grafts failed rapidly as a result of recurrent HUS, despite treat-ment with plasmapheresis. Because of persistent hypertension, both native kidneys had been removed previously. Two of her sisters also developed D-HUS. Mutation analysis revealed a factor H mutation in all 3 girls, consistent with the diagnosis of a familial HUS. Because she often had low blood pressure during hemodialysis (130/70 mm Hg before dialysis; 80/50 mm Hg during dialysis), the episodes were sug-gestive of hemodynamic transient ischemic attacks. MR imaging of the brain showed no abnormalities. However, at MR angiography, severe stenoses were observed in the proximal segments of both mid-dle and anterior cerebral arteries (Fig 1). Symptoms did not recur after strict blood pressure guidance during hemodialysis. Her 2 younger sisters with D-HUS had never experienced neurologic com-plications and declined MR angiography. The cause of the observed intracranial stenoses in our patient is unknown. Because, to our knowledge, macrovascular stenoses and vasospasm have not been described to be part of HUS, the observed findings in our patient might be a complication associated with the chronic renal replacement therapy. Our patient has been on hemodi-alysis treatment for more than 12 years. Outcome studies have re-vealed that cardiovascular disease is a relatively prominent complica-tion in children with end stage renal disease and that cerebrovascular accidents account for most deaths in this group. 1,2 Although our pa-tient indeed had signs of left ventricular hypertrophy and increased arterial wall stiffening, radiologic evaluation revealed no apparent signs of calcifications or patchy arterial wall thickening, and no other loci of arterial wall narrowing were found. It could be hypothesized that the proximal cerebral artery stenoses are secondary to the use of cyclosporin A, because the occurrence of vasospasm has previously been described in patients treated with cy-closporin A. 3 Our patient was treated with cyclosporin in the past because of 2 kidney transplantations. However, it is unlikely that prior cyclosporin use is the cause because we administered it only twice during a short period of time, for a maximum of 2 months. Because both kidney transplants were rejected, cyclosporin use had been stopped 4 years before the onset of transient ischemic attacks. The patient never had clinical signs of a cyclosporin-associated encepha-lopathy. Furthermore, in the cases described in the literature, cyclo-sporin-associated vasospasm was reversible within 1 month after cy-closporin cessation. 3




Vergouwen, M. D. I., Adriani, K. S., Roos, Y. B. W. E. M., Groothoff, J. W., & Majoie, C. B. L. M. (2008). Proximal Cerebral Artery Stenosis in a Patient with Hemolytic Uremic Syndrome. American Journal of Neuroradiology, 29(5), e34–e34.

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