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华西医学  2011 

无面部血管瘤的Sturge-Weber综合征一例

, PP. 671-673

Keywords: Sturge-Weber综合征,面部血管瘤,癫痫

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Abstract:

【】 目的 分析无面部血管瘤的Sturge-Weber综合征(SWS)的临床特点。 方法 2008年10月收治1例女性患儿,8岁,因发作性四肢强直入院,患儿无面部血管瘤及眼部异常,仅表现为癫痫。患儿接受丙戊酸钠抗癫痫治疗。 结果 患儿经头部CT、MRI确诊为无面部血管瘤的SWS。抗癫痫治疗后随访12个月,未再发癫痫,智力发育无减退,未出现瘫痪、蛛网膜下腔出血、脑出血、脑卒中样发作等。 结论 无面部血管瘤的SWS确诊依靠头部CT及MRI,治疗方法为药物抗癫痫治疗。复习文献得出无面部血管瘤的SWS患者临床表现不同于有面部血管瘤者,多仅表现出癫痫发作,且药物治疗有效,无需手术切除病灶,预后较好。【Abstract】 Objective ToanalyzetheclinicalfeaturesofSturge-Webersyndromewithoutfacialhemangioma. Methods Oneeight-year-oldgirlwasadmittedtothehospitalfortonicseizureinOctober2008.Thephysicalexaminationresultswerenormalwithneitherfacialhemangiomanorocularabnormalities.Theonlymanifestationofthepatientwasepilepsy.Thepatientwastreatedwithsodiumvalproate. Results AccordingtocranialCTandMRIresults,thepatientwasconsideredtohaveSturge-Webersyndromewithoutfacialhemangioma.Thepatientwastreatedwithanticonvulsantdrugsandexperiencednorecurrenceoftheseizuresoranymanifestationsofmentalretardation,hemiplegia,subarachnoidhemorrhage,cerebralhemorrhage,stroke-likeepisodesormigraineduringthefollowing12monthsoffollow-up. Conclusion Intheabsenceoffacialhemangioma,thediagnosiscanbebasedoncranialCTandMRI.Sturge-Webersyndromepatientswithoutfacialhemangiomaarehelpedbyanticonvulsantdrugs.Accordingtothiscaseandthereportsofliterature,mostpatientswithoutfacialhemangiomaonlymanifestepilepsywhichcanbetreatedeffectivelywithantiepilepticdrugsandsuchpatientshaveagoodprognosiswithoutoperation,whichisdifferentfromthosewithfacialhemangioma.

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