Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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Parry-Romberg syndrome with ipsilateral hemipons involvement presenting as monoplegic ataxia

  • Lee, Yun-Jin (Department of Pediatrics, Pusan National University Children's Hospital, Pusan National University School of Medicine) ;
  • Chung, Kee-Yang (Department of Dermatology, Severance Hospital, Yonsei University College of Medicine) ;
  • Kang, Hoon-Chul (Department of Pediatrics, Pediatric Epilepsy Clinic, Severance Children's Hospital, Brain Research Institute, Yonsei University College of Medicine) ;
  • Kim, Heung Dong (Department of Pediatrics, Pediatric Epilepsy Clinic, Severance Children's Hospital, Brain Research Institute, Yonsei University College of Medicine) ;
  • Lee, Joon Soo (Department of Pediatrics, Pediatric Epilepsy Clinic, Severance Children's Hospital, Brain Research Institute, Yonsei University College of Medicine)
  • 투고 : 2013.07.18
  • 심사 : 2014.01.09
  • 발행 : 2015.09.10
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초록

Parry-Romberg syndrome (PRS) is a rare, acquired disorder characterized by progressive unilateral facial atrophy of the skin, soft tissue, muscles, and underlying bony structures that may be preceded by cutaneous induration. It is sometimes accompanied by ipsilateral brain lesions and neurological symptoms. Here we present the case of a 10-year-old girl with right-sided PRS and recurrent monoplegic ataxia of the left leg. At 4 years of age, she presented with localized scleroderma over the right parietal region of her scalp; her face gradually became asymmetric as her right cheek atrophied. Brain magnetic resonance imaging revealed hemiatrophy of the face and skull base, and T2-weighted images showed increased signal in the right hemipons and hemicerebellar peduncle. Magnetic resonance angiography findings were unremarkable. She was treated with oral prednisolone, and her recurrent gait ataxia diminished within 2 months of the follow-up period. To the best of our knowledge, this is only the second case of PRS presenting with an abnormal involvement of the ipsilateral hemipons.

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참고문헌

  1. Stone J, Franks AJ, Guthrie JA, Johnson MH. Scleroderma "en coup de sabre": pathological evidence of intracerebral inflammation. J Neurol Neurosurg Psychiatry 2001;70:382-5. https://doi.org/10.1136/jnnp.70.3.382
  2. Duymaz A, Karabekmez FE, Keskin M, Tosun Z. Parry-Romberg syndrome: facial atrophy and its relationship with other regions of the body. Ann Plast Surg 2009;63:457-61. https://doi.org/10.1097/SAP.0b013e31818bed6d
  3. Carreno M, Donaire A, Barcelo MI, Rumia J, Falip M, Agudo R, et al. Parry Romberg syndrome and linear scleroderma in coup de sabre mimicking Rasmussen encephalitis. Neurology 2007;68: 1308-10. https://doi.org/10.1212/01.wnl.0000259523.09001.7a
  4. Stone J. Parry-Romberg syndrome: a global survey of 205 patients using the Internet. Neurology 2003;61:674-6. https://doi.org/10.1212/WNL.61.5.674
  5. Kister I, Inglese M, Laxer RM, Herbert J. Neurologic manifestations of localized scleroderma: a case report and literature review. Neurology 2008;71:1538-45. https://doi.org/10.1212/01.wnl.0000334474.88923.e3
  6. Chiu YE, Vora S, Kwon EK, Maheshwari M. A significant proportion of children with morphea en coup de sabre and Parry-Romberg syndrome have neuroimaging findings. Pediatr Dermatol 2012;29:738-48. https://doi.org/10.1111/pde.12001
  7. Cory RC, Clayman DA, Faillace WJ, McKee SW, Gama CH. Clinical and radiologic findings in progressive facial hemiatrophy (Parry-Romberg syndrome). AJNR Am J Neuroradiol 1997;18:751-7.
  8. Moko SB, Mistry Y, Blandin de Chalain TM. Parry-Romberg syndrome: intracranial MRI appearances. J Craniomaxillofac Surg 2003;31:321-4. https://doi.org/10.1016/S1010-5182(03)00028-3
  9. Qureshi UA, Wani NA, Altaf U. Parry-Romberg syndrome associated with unusual intracranial vascular malformations and Phthisis bulbi. J Neurol Sci 2010;291:107-9. https://doi.org/10.1016/j.jns.2010.01.003
  10. El-Kehdy J, Abbas O, Rubeiz N. A review of Parry-Romberg syndrome. J Am Acad Dermatol 2012;67:769-84. https://doi.org/10.1016/j.jaad.2012.01.019
  11. Gambichler T, Kreuter A, Hoffmann K, Bechara FG, Altmeyer P, Jansen T. Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications. BMC Dermatol 2001;1:9. https://doi.org/10.1186/1471-5945-1-9
  12. Drummond PD, Hassard S, Finch PM. Trigeminal neuralgia, migraine and sympathetic hyperactivity in a patient with Parry-Romberg syndrome. Cephalalgia 2006;26:1146-9. https://doi.org/10.1111/j.1468-2982.2006.01161.x
  13. Sathornsumetee S, Schanberg L, Rabinovich E, Lewis D Jr, Weisleder P. Parry-Romberg syndrome with fatal brain stem involvement. J Pediatr 2005;146:429-31. https://doi.org/10.1016/j.jpeds.2004.10.026
  14. Lonchampt P, Emile J, Pelier-Cady MC, Cadou B, Barthelaix A. Central sympathetic dysregulation and immunological abnormalities in a case of progressive facial hemiatrophy (Parry-Romberg disease). Clin Auton Res 1995;5:199-204. https://doi.org/10.1007/BF01824007
  15. Gonul M, Dogan B, Izci Y, Varol G. Parry-Romberg syndrome in association with anti-dsDNA antibodies: a case report. J Eur Acad Dermatol Venereol 2005;19:740-2. https://doi.org/10.1111/j.1468-3083.2005.01290.x

피인용 문헌

  1. Parry–Romberg syndrome affecting one half of the body vol.6, pp.4, 2016, https://doi.org/10.4103/2231-0762.186792