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Henoch–Schönlein purpura nephritis complicated by reversible posterior leukoencephalopathy syndrome

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Abstract

We report a young female patient with Henoch–Schönlein purpura (HSP) nephritis complicated by reversible posterior leukoencephalopathy syndrome (RPLS). The patient suddenly showed generalized seizures and cortical blindness with severe hypertension due to renal insufficiency approximately 1 year after cessation of corticosteroid treatment for HSP nephritis. Magnetic resonance imaging (MRI) demonstrated bilateral abnormal signals mainly in the cerebellum and white matter of the occipital lobe. Clinical symptoms quickly improved in conjunction with disappearance of abnormal signals on brain MRI after starting control of hypertension and continuous hemodiafiltration with steroid pulse therapy and plasmapheresis. RPLS may be caused by vasculitis and also by hemodynamic change due to severe hypertension in HSP, particularly in patients with nephropathy. In such cases intensive treatment should be performed as soon as possible to avoid neurological sequelae.

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References

  1. Brogan PA, Dillon MJ (2000) Vasculitis from the pediatric perspective. Curr Rheumatol Rep 2:411–416

    Article  PubMed  CAS  Google Scholar 

  2. Belman AL, Leicher CR, Moshe SL, Mezey AP (1985) Neurologic manifestations of Schönlein–Henoch purpura: report of three cases and review of the literature. Pediatrics 75:687–692

    PubMed  CAS  Google Scholar 

  3. Ha TS, Cha SH (1996) Cerebral vasculitis in Henoch–Schönlein purpura: a case report with sequential magnetic resonance imaging. Pediatr Nephrol 10:634–636

    Article  PubMed  CAS  Google Scholar 

  4. Woolfenden AR, Hukin J, Poskitt KJ, Connolly MB (1998) Encephalopathy complicating Henoch–Schönlein purpura: reversible MRI changes. Pediatr Neurol 19:74–77

    Article  PubMed  CAS  Google Scholar 

  5. Perez C, Maravi E, Olier J, Guarch R (2000) MR imaging of encephalopathy in adult Henoch–Schönlein purpura. Am J Roentgenol 175:922–923

    CAS  Google Scholar 

  6. Bakkaloglu SA, Ekim M, Tumer N, Deda G, Erden I, Erdem T (2000) Cerebral vasculitis in Henoch–Schönlein purpura. Nephrol Dial Transplant 15:246–248

    Article  PubMed  CAS  Google Scholar 

  7. Chen CL, Chiou YH, Wu CY, Lai PH, Chung HM (2000) Cerebral vasculitis in Henoch–Schönlein purpura: a case report with sequential magnetic resonance imaging changes and treated with plasmapheresis alone. Pediatr Nephrol 15:276–278

    Article  PubMed  CAS  Google Scholar 

  8. Eun SH, Kim SJ, Cho DS, Chung GH, Lee DY, Hwang PH (2003) Cerebral vasculitis in Henoch–Schönlein purpura: MRI and MRA findings, treated with plasmapheresis alone. Pediatr Int 45:484–487

    Article  PubMed  Google Scholar 

  9. Ozcakar BZ, Ekim M, Fitoz S, Teber S, Hizel S, Acar B et al (2004) Hypertension induced reversible posterior leukoencephalopathy syndrome: a report of two cases. Eur J Pediatr 163:728–730

    Article  PubMed  Google Scholar 

  10. Hinchey J, Chaves C, Appignani B, Breen J, Pao L, Wang A et al (1996) A reversible posterior leukoencephalopathy syndrome. N Engl J Med 334:494–500

    Article  PubMed  CAS  Google Scholar 

  11. Counahan R, Winterborn MH, White RHR, Heaton JM, Meadow SR, Bluett NH et al (1977) Prognosis of Henoch–Schönlein nephritis in children. Br Med J 2:11–14

    Article  PubMed  CAS  Google Scholar 

  12. Ahn KJ, You WJ, Jeong SL, Lee JW, Kim BS, Lee JH et al (2004) Atypical manifestations of reversible posterior leukoencephalopathy syndrome: findings on diffusion imaging and ADC mapping. Neuroradiology (Epub ahead of print)

  13. Kumai Y, Toyoda K, Fujii K, Ibayashi S (2002) Hypertensive encephalopathy extending into the whole brainstem and deep structures. Hypertens Res 25:797–800

    Article  PubMed  Google Scholar 

  14. Calabresi P, Pisani A, Mercuri NB, Bernardi G (1995) On the mechanisms underlying hypoxia-induced membrane depolarization in striatal neurons. Brain 118:1027–1038

    Article  PubMed  Google Scholar 

  15. Abe K (2004) Reversible posterior leukoencephalopathy syndrome. Intern Med 43:900–901

    Article  PubMed  Google Scholar 

  16. Edvinsson L, Owman C, Sjoberg NO (1976) Autonomic nerves, mast cells, and amine receptors in human brain vessels. A histochemical and pharmacological study. Brain Res 115:377–393

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Third Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan

    Daimei Sasayama, Yasuhiro Shimojima, Takahisa Gono, Kazuma Kaneko, Masayuki Matsuda & Shu-ichi Ikeda

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  1. Daimei Sasayama
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  2. Yasuhiro Shimojima
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  3. Takahisa Gono
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  4. Kazuma Kaneko
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  5. Masayuki Matsuda
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  6. Shu-ichi Ikeda
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Correspondence to Masayuki Matsuda.

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Sasayama, D., Shimojima, Y., Gono, T. et al. Henoch–Schönlein purpura nephritis complicated by reversible posterior leukoencephalopathy syndrome. Clin Rheumatol 26, 1761–1763 (2007). https://doi.org/10.1007/s10067-006-0502-0

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  • DOI: https://doi.org/10.1007/s10067-006-0502-0

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