Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
권호 표지

Clinical and Radiological Analysis of Reversible Posterior Leukoencephalopathy Syndrome in Children

소아에서 가역성 후두부 백질 뇌증 증후군의 임상적, 방사선학적 특성에 대한 고찰

  • Lim, Hae-Ri (Department of Pediatrics, college of Medicine, Kyungpook National University) ;
  • Seo, Hye-Eun (Department of Pediatrics, college of Medicine, Kyungpook National University) ;
  • Kwon, Sun-Hak (Department of Pediatrics, college of Medicine, Kyungpook National University)
  • 임해리 (경북대학교 의과대학 소아과학교실) ;
  • 서혜은 (경북대학교 의과대학 소아과학교실) ;
  • 권순학 (경북대학교 의과대학 소아과학교실)
  • Received : 2007.06.22
  • Accepted : 2007.07.12
  • Published : 2007.09.15
Download PDF
⟨ Previous Next ⟩

Abstract

Purpose : Reversible posterior leukoencephalopathy syndrome is a complex disorder with characteristic clinical and radiologic findings that mainly involve the white/gray matter of the parieto-occipital lobes. The purpose of this study was to determine its clinical and radiological characteristics. Methods : A total of 15 pateints were involved in the study. Their medical records and radiological features of brain MRI were retrospectively reviewed and analyzed. Results : Fifteen pateints were involved. (9 males and 5 females). The patients' ages ranged from 2-20 years (mean age : 10 years). The brain MRI revealed fairly symmetric areas of increased T2 signal involving both white and gray matter of parieto-occipital regions. The condition seemed to be associated with cyclosporin A and steroid therapy or a variety of other conditions in which blood pressure rises acutely. Conclusion : Reversible posterior leukoencephalopathy syndrome is a complicated neurological condition, but a better understanding of this complex syndrome may obviate unnecessary investigations and lead to prompt and appropriate management of the associated problems.

목 적 : 가역성 후두부 백질 뇌증 증후군은 두정-후두엽의 백질 혹은 회질을 침범하는 특징을 가지는 복합 증후군이다. 이번 연구에서는 이 증후군의 임상적, 방사선학적 특징을 분석하였다. 방 법 : 총 15명의 환아를 대상으로 하였으며. 각 환아에서 급성기 동안의 평균 동맥압을 측정하고 신경학적 사건이 발생하기 수일에서 수주 전 안정 상태의 평균 동맥압을 조사하여 비교하였다. 그리고 이들의 뇌자기 공명 영상을 분석 비교하였으며 모든 연구는 후향적으로 이루어졌다. 결 과 : 15명의 환아(남아 : 9명, 여아 : 6명)의 연령은 2-20세(평균 연령:10세)였다. 뇌 자기공명 영상은 두정-후두엽의 백질 및 피질에 T2-weighted image에서 신호 강도의 증가를 보였다. 대개의 경우에서 스테로이드나 싸이클로스포린 복용 혹은 급격하게 혈압이 증가하는 사건과 관련성을 보였다. 결 론 : 가역성 후두부 백질 뇌증 증후군은 복합적인 신경학적 질환으로 이 복합 증후군에 대한 더 많은 연구를 함으로써 불필요한 검사를 줄이고 즉각적이고 합당한 치료를 환아에게 행할 수 있을 것으로 생각한다.

Keywords

References

  1. Yokobori S, Yokota H, Yamamoto Y. Pediatric Posterior Reversible Leukoencephalopathy Syndrome and NSAID-induced acute tubular intersitial nephritis. Pediatr Neurol 2006; 34:245-7 https://doi.org/10.1016/j.pediatrneurol.2005.07.012
  2. Hinchey J, Chaves C, Appignani B, Breen J, Pao L, Wang A, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996;334:494-500 https://doi.org/10.1056/NEJM199602223340803
  3. Kwon S, Koo J, Lee S. Clnical spectrum of reversible posterior leukoencephalopathy syndrome. Pediatr Neurol 2001; 24:361-4 https://doi.org/10.1016/S0887-8994(01)00265-X
  4. Ishikura K, Ikeda M, Hamasaki Y, Hataya H, Shishido S, Asanuma H, et al. Posterior reversible encephalopathy syndrome in children: Its high prevalance and more extensive imaging findings. Am J of Kidney Dis 2006:48;231-8 https://doi.org/10.1053/j.ajkd.2006.04.076
  5. Henderson RD, Rajah T, Nicol AJ, Read SJ. Posterior leukoencephalopathy following intrachecal chemotherapy with MRA documented vasospasm. Neurology 2003;60:326-8 https://doi.org/10.1212/01.WNL.0000042095.49520.1E
  6. Catherine M, David A, Manoj K, James M. Tacrolimus leukoencephalopathy: A neuropathologic confirmation. Neurology 2004:63;1132-3 https://doi.org/10.1212/01.WNL.0000138568.60774.E4
  7. Maryam P, Iris D, Jeannie Y, Katrina J, Paul R. Posterior reversible leukoencephalopathy in a case of postpartum eclampsia. Am J of Obsterics and Gynecology 2000:193;885-6
  8. Antunes NL, Small TN, George D, Boulad F, Lis E. Posterior leukoence- phalopathy syndrome may not be reversible. Pediatr Neurol 1999;20:241-3 https://doi.org/10.1016/S0887-8994(98)00148-9
  9. Schwarz RB, Mulkern RV, Gudbjartsson H, Jolesz F. Diffusion- weighted MR imaging in hypertensive encephalopathy: Clues to pathogenesis. Am J Neuroradiol 1998;19:859-62
  10. Pavlaskis SG, Frank Y, Chusid R. Hypertensive encephalopathy, reversible occipitoparietal encephalopathy, or reversible posterior leukoencephalopathy: Three names for an old syndrome J Child Neurol 1999;14:277-81 https://doi.org/10.1177/088307389901400502
  11. Truwit CL, Denarco CP, Lake JR, Demarco T. MR imaging of reversible cyclospoline A induced neurotoxicity. Am J Neuroradiol 1991;12:651-9
  12. Schwartz RB, Bravo SM, Klufas RA, et al. Cyclosporine neurotoxicity and its relationship to hypertensive encephalopathy: CT and MR findings in 16 cases. Am J Radiol 1995;165:627-31
  13. Wright RR, Mathews HD. Hypertensive encephalopathy in childhood. J Child Neurol 1996;11:193-6 https://doi.org/10.1177/088307389601100305
  14. Jones BV, Egelhoff JC, Petterson J. Hypertensive encephalopathy in children. Am J Neuroradiol 1997;18:101-6
  15. Gleeson JG, Duplessis AJ, Marnes PD, Riviello JJ Jr. Cyclosporin A acute encephalopathy and seizure syndrome in childhood: Clinical features and risk of seizure recurrence. J Child Neurol 1998;13:336-44 https://doi.org/10.1177/088307389801300706
  16. Sanders TG, Clayman DA, Sanchez-Ramos L, Vines FS, Russo L. Brain in eclampsia: MR imaging with clinical correlation. Radiology 1991;180:475-8 https://doi.org/10.1148/radiology.180.2.2068315
  17. Taylor MB, Jackson A, Weller JM. Dynamic susceptibility contrast enhanced MRI in reversible posterior leukoencephalopathy syndrome associated hemolytic uremic syndrome. Br J radiol 2000;73:438-42 https://doi.org/10.1259/bjr.73.868.10844872
  18. Mathy I, Gille M, Van Raemdonck F, Delbecq J, Depre A. Neurological complication of intravenous immunoglobulin (IVIg) therapy: An illustrative case of acute encephalopathy following IVIg therapy and and review fo the literature. Acta neurol Bleg 1998;98:347-51
  19. Ito Y, Arahata Y, Goto Y, M Hirayama, M Nagamutsu, T Yasuda, et al. Cisplatin neurotoxicity presenting as reversible posterior leukoencephalopathy syndrome. Am J Neuroradiol 1998;19:415-7
  20. Laguna P, Martin T, Marchena MJ, Moya M. Posterior leukoencephalopathy reversible sydrome secondary to panarteritis nodosa. Med Clin(Barc) 1997;37:64-6
  21. Arai M, Shigeno K, Wada M. A reversible posterior leukoencephalopathy sydrome in a patient with classical polyateritis nodosa. Rinsho Shinkeigaku 1997;37:64-6
  22. Ito Y, Niwa H, lida T, Nagamatsu M, Yasuda T, Uanagi T, et al. Post-transfusion reversible posterior leukoencephalopathy syndrome with cerebral vasoconstriction. Neurology 1997;49:1174-5 https://doi.org/10.1212/WNL.49.4.1174
  23. Delanty N, Vaughan C, Frucht S, Stubgen P. Erythropoietin associated hypertensive posterior leukoencephalopathy. Neurology 1997;49:636-9
  24. Phillips DJ, Davies AH, Bliss BP. Hypertensive encephalopathy secondary to pheochromocytoma: The cause of death after carotid endarterectomy. J Cardiovasc Surg(torino) 1994;35:533-5
  25. Pavlakis SG, frank Y, Kalina P, Chandra M, Lu D. Occipital- parietal encephalopathy: A new name for an old syndrome. Pediatr Neurol 1997;16:145-8 https://doi.org/10.1016/S0887-8994(96)00292-5
  26. Cooney MJ. Bradley WG, Symko SC, Patel ST, Groncy PK. Hypertensive encephalopathy: Complication in children treated for myeloproliperative disorders-report of three cases. Radiology 2000;214:711-6 https://doi.org/10.1148/radiology.214.3.r00mr19711