| 伴皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病的临床、影像和病理学一例分析 |
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| 引用本文： | 蔡桂兰,许春伶,王佳伟,王长英,王得新. 伴皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病的临床、影像和病理学一例分析[J]. 脑与神经疾病杂志, 2010, 18(2): 115-118 |
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| 作者姓名： | 蔡桂兰 许春伶 王佳伟 王长英 王得新 |
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| 作者单位： | 首都医科大学附属北京友谊医院神经内科,北京,100050 |
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| 摘 要： | 目的探讨伴皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病(CADASIL)的临床特点、影像和病理学特征,提高对CADASIL的认识。方法对一例CADASIL患者的临床表现、影像学表现及皮肤活检进行总结。结果患者妊娠晚期发病,表现为反复的缺血性卒中发作,头颅MRI见皮质下梗死和白质脑病的改变,皮肤活检提示微小动脉的平滑肌细胞表面出现颗粒样嗜锇物质(GOM)。结论有阳性家族史,无常见脑血管病危险因素且发病年龄较早的缺血性脑血管病患者要考虑到CADASIL的可能,同时妊娠可能会对促进本病的发展有一定的影响。
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| 关 键 词： | 伴皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病 嗜锇物质 妊娠 |
Clinical features,imaging menifestations and pathology of a case of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy |
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| CAI Gui-lan,XU Chun-ling,WANG Jia-wei,WANG Chang-ying,WANG De-xin. Clinical features,imaging menifestations and pathology of a case of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy[J]. Journal of Brain and Nervous Diseases, 2010, 18(2): 115-118 |
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| Authors: | CAI Gui-lan XU Chun-ling WANG Jia-wei WANG Chang-ying WANG De-xin |
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| Affiliation: | CAI Gui-lan,XU Chun-ling,WANG Jia-wei,WANG Chang-ying,WANG De-xin.Department of Neurology,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China |
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| Abstract: | Objective To study the clinical features,the imaging menifestations and pathology of cerebral autosomal dominant arteriopathy with subcortical infarcts and leckoencephalopahty(CADASIL),and to improve the recognition about it.Methods One case of CADASIL was diagnosised after biopsy of skin,family history,clinical menifestations and brain MRI data were recorded and investigated.Results The patient was a 29-year-old female who had recurrent ischemic stroke during her late trimester of pregnancy.The brain MRI d... |
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| Keywords: | Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy Granular osmiophilic material Pregnancy |
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