Linear Scleroderma “En Coup De Sabre”: Initial Presentation as Intractable Partial Seizures in a Child
Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. The “en coup de sabre” subtype of linear scleroderma is more often associated with systemic morbidity, including ocular, oral, and neurological abnormalities. Here, we...
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Elsevier
2009-12-01
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| Series: | Pediatrics and Neonatology |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S1875957209600814 |
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| author | Kuo-Liang Chiang Kai-Ping Chang Tai-Tong Wong Ting-Rong Hsu |
| author_facet | Kuo-Liang Chiang Kai-Ping Chang Tai-Tong Wong Ting-Rong Hsu |
| author_sort | Kuo-Liang Chiang |
| collection | DOAJ |
| description | Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. The “en coup de sabre” subtype of linear scleroderma is more often associated with systemic morbidity, including ocular, oral, and neurological abnormalities. Here, we report one patient with typical linear scleroderma “en coup de sabre” (LSCS). Initially, he presented with refractory partial seizures before the characteristic skins lesion on his head developed. This was a rare case with obvious brain parenchyma involvement. We did not prescribe medication but performed serial brain magnetic resonance imaging follow-up for the intraparenchymal lesion. The atrophic changes of the skin, face and brain remained the same, and his seizures had not worsened at the most recent follow-up. Parry-Romberg syndrome, a very similar condition, should be differentiated from LSCS. |
| format | Article |
| id | doaj-art-431e1883b65e458282efb9b9e5dddafd |
| institution | OA Journals |
| issn | 1875-9572 |
| language | English |
| publishDate | 2009-12-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Pediatrics and Neonatology |
| spelling | doaj-art-431e1883b65e458282efb9b9e5dddafd2025-08-20T01:56:56ZengElsevierPediatrics and Neonatology1875-95722009-12-0150629429810.1016/S1875-9572(09)60081-4Linear Scleroderma “En Coup De Sabre”: Initial Presentation as Intractable Partial Seizures in a ChildKuo-Liang Chiang0Kai-Ping Chang1Tai-Tong Wong2Ting-Rong Hsu3Department of Pediatrics, Neurological Institute, Taipei Veterans General Hospital, Taipei, TaiwanDepartment of Pediatrics, Neurological Institute, Taipei Veterans General Hospital, Taipei, TaiwanDepartment of Pediatrics, Kuang-Tien General Hospital, Taichung, TaiwanDepartment of Pediatrics, Neurological Institute, Taipei Veterans General Hospital, Taipei, TaiwanLinear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. The “en coup de sabre” subtype of linear scleroderma is more often associated with systemic morbidity, including ocular, oral, and neurological abnormalities. Here, we report one patient with typical linear scleroderma “en coup de sabre” (LSCS). Initially, he presented with refractory partial seizures before the characteristic skins lesion on his head developed. This was a rare case with obvious brain parenchyma involvement. We did not prescribe medication but performed serial brain magnetic resonance imaging follow-up for the intraparenchymal lesion. The atrophic changes of the skin, face and brain remained the same, and his seizures had not worsened at the most recent follow-up. Parry-Romberg syndrome, a very similar condition, should be differentiated from LSCS.http://www.sciencedirect.com/science/article/pii/S1875957209600814intractable seizurelinear scleroderma en coup de sabreprogressive brain atrophy |
| spellingShingle | Kuo-Liang Chiang Kai-Ping Chang Tai-Tong Wong Ting-Rong Hsu Linear Scleroderma “En Coup De Sabre”: Initial Presentation as Intractable Partial Seizures in a Child Pediatrics and Neonatology intractable seizure linear scleroderma en coup de sabre progressive brain atrophy |
| title | Linear Scleroderma “En Coup De Sabre”: Initial Presentation as Intractable Partial Seizures in a Child |
| title_full | Linear Scleroderma “En Coup De Sabre”: Initial Presentation as Intractable Partial Seizures in a Child |
| title_fullStr | Linear Scleroderma “En Coup De Sabre”: Initial Presentation as Intractable Partial Seizures in a Child |
| title_full_unstemmed | Linear Scleroderma “En Coup De Sabre”: Initial Presentation as Intractable Partial Seizures in a Child |
| title_short | Linear Scleroderma “En Coup De Sabre”: Initial Presentation as Intractable Partial Seizures in a Child |
| title_sort | linear scleroderma en coup de sabre initial presentation as intractable partial seizures in a child |
| topic | intractable seizure linear scleroderma en coup de sabre progressive brain atrophy |
| url | http://www.sciencedirect.com/science/article/pii/S1875957209600814 |
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