Parry-Romberg syndrome with ipsilateral hemipons involvement presenting as monoplegic ataxia

Yun Jin Lee, Kee Yang Chung, Hoon Chul Kang, Heung Dong Kim, Joon Soo Lee

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)354-357
Number of pages4
JournalKorean Journal of Pediatrics
Volume58
Issue number9
DOIs
Publication statusPublished - 2015 Sep 1

Fingerprint

Facial Hemiatrophy
Ataxia
Gait Ataxia
Localized Scleroderma
Skin
Parietal Lobe
Cheek
Magnetic Resonance Angiography
Skull Base
Brain
Prednisolone
Scalp
Atrophy
Leg
Magnetic Resonance Imaging
Muscles

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Pediatrics

Cite this

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abstract = "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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Parry-Romberg syndrome with ipsilateral hemipons involvement presenting as monoplegic ataxia. / Lee, Yun Jin; Chung, Kee Yang; Kang, Hoon Chul; Kim, Heung Dong; Lee, Joon Soo.

In: Korean Journal of Pediatrics, Vol. 58, No. 9, 01.09.2015, p. 354-357.

Research output: Contribution to journalArticle

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