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 language | English |
|---|---|
| Pages (from-to) | 354-357 |
| Number of pages | 4 |
| Journal | Korean Journal of Pediatrics |
| Volume | 58 |
| Issue number | 9 |
| DOIs | |
| Publication status | Published - 2015 Sep 1 |
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All Science Journal Classification (ASJC) codes
- Pediatrics, Perinatology, and Child Health
- Pediatrics
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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 journal › Article
TY - JOUR
T1 - Parry-Romberg syndrome with ipsilateral hemipons involvement presenting as monoplegic ataxia
AU - Lee, Yun Jin
AU - Chung, Kee Yang
AU - Kang, Hoon Chul
AU - Kim, Heung Dong
AU - Lee, Joon Soo
PY - 2015/9/1
Y1 - 2015/9/1
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=84941553355&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84941553355&partnerID=8YFLogxK
U2 - 10.3345/kjp.2015.58.9.354
DO - 10.3345/kjp.2015.58.9.354
M3 - Article
AN - SCOPUS:84941553355
VL - 58
SP - 354
EP - 357
JO - Korean Journal of Pediatrics
JF - Korean Journal of Pediatrics
SN - 1783-1061
IS - 9
ER -