| 549 | 26 | 1878 |
| 下载次数 | 被引频次 | 阅读次数 |
目的探讨Sturge-Weber综合征的临床特点及CT、MRI表现及诊断。方法归纳8例Sturge-Weber综合征患者的临床表现、影像学特点,进行回顾性分析,并复习相关文献。结果 8例均有脑内软脑膜血管瘤,其中3例伴有颜面部血管瘤,均位于三叉神经眼支分布区(均累及单侧);7例表现为癫痫发作,其中5例伴有头痛,1例伴有头晕及一侧肢体乏力;1例表现为单纯反复头痛;均无智力低下或青光眼。影像学检查可清晰显示脑萎缩、脑回样钙化、软脑膜广泛强化伴静脉畸形。结论 Sturge-Weber综合征是一种少见疾病,根据典型临床特点及CT、MRI等影像学检查有助于确诊。
Abstract:Objective To determine the clinical and imaging features of Sturge-Weber syndrome(SWS).Methods The clinical and imaging features of 8 patients with Sturge-Weber syndrome were reviewed.Results Leptomeningeal angiomatosis was found in all patients,accompanied by unilateral facial hemangioma(3) in the distribution of the ophthalmic division of the trigeminal nerve.Of 7 patients with epilepsy,5 were accompanied by headache,1 by dizziness and unilateral muscle weakness,1 by recurring simple headache.There was no mental retardation or glaucoma in any of the patients...CT and MRI clearly showed brain atrophy,gyriform calcification,diffuse enhancement of pia mater accompanied by cerebral venous malformation.Conclusion Sturge-Weber syndrome has typical clinical and imaging features on CT and MRI for diagnosis.
[1]Yallapragada AV,Cure JK,Holden KR.Sturge-Weber syndrome variant with atypical intracranial findings:case report.J Child Neurol,2006,21:155-157.
[2]Schrimer R.Ein fall von telangiektasie.Albrecht von Graefes.Arch Ophtalmol,1860,7:119-121.
[3]Sturge WA.A case of partial epilepsy,apparently due to a lesion of one of the vaso-motor centers of the brain,Arch Neurol,1969,21:555-556.
[4]Kalischer S,.Demonstration des Gehirns eines kindes mit teleangiektasie der linksseitigen gesichts-kopfhaut und hirnoberflache.Berl Klin Wochenschr,1897,34:1059-1067.
[5]Cushing H,Cases of spontaneous int racranial hemorrhage associated with trigeminal nevi.JAMA,1906,47:178-183.
[6]Maiuri F,Gangemi M,Iaconetta G,et al.Sturge-Weber disease without facial nevus.J Neurosurg Sci,1989,33:215-218.
[7]Stehbens WE.Pathology of cerebral blood vessels.Saint Louis:the CV Mosby Company,1972:519-520.
[8]Toole JF,Cerebrovascular disorders,5th ed,Philadelphia:Lippiocott Williams&Wilkins,1999:459-461.
[9]Comi AM,Hunt P,Vawter MP,et al.Increased fibronectin expression in Sturge-weber syndrome fibroblasts and brain tissue.Pediatr Res,2003,53:762-769.
[10]刘焯霖,梁秀龄.神经遗传病学.北京:人民卫生出版社,1988:175-178.
[11]杜传书,刘祖洞,主编.医学遗传学.北京:人民卫生出版社,1983:633.
[12]陈雪芹,陈铌,王晓洁,等.Sturge-Weber综合征一例.中华病理学杂志,2006,35:503-504.
[13]Greene AK,Taber SF,Ball KL,et al.Sturge-Weber syndrome:soft-tissue and skeletal overgrowth.J Craniofac Surg,2009,20Suppl 1:617-721.
[14]Suskauer SJ,Trovato MK,Zabel TA,et al.Physiatric findings in individuals with Sturge-Weber syndrome,Am J Phys Med Rehabil,2010,89:323-330.
[15]Di Rocco C,Tamburrini G,Sturge-Weber syndrome,Childs Nerv Syst,2006,22:909-921.
[16]Thomas-Sohl KA,Vaslow DF,Maria BL.Sturge-Weber syndrome:a review.Pediatr Neurol,2004,30:303-310.
[17]Roach ES.Neurocutaneous syndromes.Pediatr Clin North Am,1992,39:591-620.
[18]Aydin A,Cakmakci H,Kovanlikaya A,et al.Sturge-Weber syndrome without facial nevus.Pediatr Neurol,2000,22:400-402.
[19]黎军强,刘彪,王丽娜,等.Sturge-Weber综合征的CT和MRI诊断价值(附6例报告及文献复习).实用放射学杂志,2007,23:427-429.
[20]吴荣鑫,徐光炎.Sturge-Weber综合征的CT诊断(附9例报告).影像诊断与介入放射学,2008,17:247-249.
[21]Atlas SW,Magnetic resonance imaging of the brain and spine.4th ed.Philadelphia:Lippiocott Williams&Wilkins,2009:294-300.
[22]Boukobza M,Enjolras O,Cambra M,et al,Sturge-Weber syndrome.The current neuroradiologic data.J Radiol,2000,81:765-771.
[23]杜彦,任翠萍,李彩霞,等.Sturge-Weber综合征9例临床分析及CT和MRI诊断.郑州大学学报(医学版),2008,43:1048-1049.
[24]桂绍高,王川红,李明智,等.Sturge-Weber综合征的综合影像诊断.实用临床医学,2007,8:107-111.
[25]Hu J,Yu Y,Juhasz C,et al,MR susceptibility weighted imaging(SWI)complements conventional contrast enhanced T1weighted MRI in characterizing brain abnormalities of SturgeWeber Syndrome.J Magn Reson Imaging,2008,28:300-307.
[26]田树平,黄敏华,郑奎宏,等.磁敏感加权成像及增强T1WI FLAIR序列在脑-三叉神经血管瘤病MR诊断中的应用探讨.中国医学影像学杂志,2010,18:353-358.
[27]Kossoff EH,Buck C,Freeman JM,Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide,Neurology,2002,59:1735-1738.
[28]Bourgeois M,Crimmins DW,de Oliveira RS,et al.Surgical treatment of epilepsy in Sturge-Weber syndrome in children.J Neurosurg,2007,106 Suppl 1:20-28.
[29]Arzimanoglou AA,Andermann F,Aicardi J,et al.SturgeWeber syndrome:.indications and results of surgery in 20patients.Neurology,2000,55:1472-1479.
[30]Hoffman HJ,Hendrick EB,Dennis M,et al.Hemispherectomy for Sturge-Weber syndrome,Childs Brain,1979,5:233-248.
[31]Tuxhom IE,Pannek HW.Epilepsy surgery in bilateral SturgeWeber syndrome.Pediatr Neurol,2002,26:394-397.
基本信息:
中图分类号:R743.4;R445.2;R816.1
引用信息:
[1]黄晓星,王志龙,肖学红,等.Sturge-Weber综合征临床分析及影像诊断[J],2015,24(01):68-73.