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난치성 간질을 동반한 Parry-Romberg 증후군 1례

Other Titles
 A Case of Parry-Romberg Syndrome with Intractable Seizure 
Authors
 허윤정  ;  김성우  ;  정희정  ;  송동우 
Citation
 Journal of the Korean Child Neurology Society, Vol.11(1) : 144-149, 2003 
Journal Title
Journal of the Korean Child Neurology Society(대한소아신경학회지)
ISSN
 1226-6884 
Issue Date
2003
MeSH
Progressive hemifacial atrophy ; Parry-Romberg syndrome ; Intractable seizure
Keywords
Progressive hemifacial atrophy ; Parry-Romberg syndrome ; Intractable seizure
Abstract
Parry-Romberg syndrome, first described in 1825 by Parry and in 1846 by Romberg, is a rare disorder characterized by a progressive hemifacial atrophy of the skin and adipose tissue and atrophy of muscle, cartilage, and underlying bony structures. It is sometimes accompanied with such complications as ophthalmologic abnormality, localized alopecia and neurologic symptoms, for example, contralateral Jacksonian epilepsy, trigeminal neuralgia, migrane and hemiplegia. The onset is slow and progressive, starting at the first or second decade of life and lasting for 2-10 years, ending with a face being 'burned out'. It is often associated with epilepsy but the link between these two conditions is poorly understood. In patients with progressive hemifacial atrophy, a high incidence of abnormal neuroradiologic findings in the brain has been reported. Brain MRI findings include cerebral hemiatrophy, cortical calcification, unilateral focal infarction in the corpus callosum, diffuse deep and subcortical white matter signal changes and mild cortical thickening. We report a case of Parry-Romberg syndrome in a 5-year-old boy who had a progressive Rt. facial hemiatrophy with intractable epilepsy and basal ganglia calcification from brain MRI.
Full Text
http://scholar.dkyobobook.co.kr/searchDetail.laf?barcode=4010022737848
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Psychiatry (정신과학교실) > 1. Journal Papers
Yonsei Authors
Song, Dong Ho(송동호) ORCID logo https://orcid.org/0000-0002-9647-3130
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/113756
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