| GM_2神经节苷脂沉积症(附2例报告) |
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| 引用本文： | 许念桂,彭隆祥,卢伟,谭利明,胡治平,肖波,蒋波.GM_2神经节苷脂沉积症(附2例报告)[J].临床神经病学杂志,2001(6). |
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| 作者姓名： | 许念桂 彭隆祥 卢伟 谭利明 胡治平 肖波 蒋波 |
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| 作者单位： | 长沙中南大学湘雅二医院神经内科，中南大学湘雅医学院电镜室，长沙中南大学湘雅二医院神经内科，长沙中南大学湘雅二医院神经内科，长沙中南大学湘雅二医院神经内科，长沙中南大学湘雅二医院神经内科，中南大学湘雅医院神经内科 410011，410011，410011，410011，410011 |
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| 摘 要： | 目的 探讨 GM2 神经节苷脂沉积症的临床特点和病理改变。方法 报道 2例晚发型 GM2 神经节苷脂沉积症的临床表现 ,并取 2例患者右额叶脑组织进行病理观察。结果 晚发型 GM2 神经节苷脂沉积症的临床表现无特异性 ,光镜下神经元呈气球样肿胀 ,胞浆内有脂质沉积 ,电镜观察见沉积物为膜性胞浆体和斑马体。结论 对临床上疑为 GM2 神经节苷脂沉积症的患者 ,脑活检光镜加电镜观察有助于明确诊断
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| 关 键 词： | 神经节苷脂沉积症 临床表现 超微结构 |
GM_2 gangliosidosis |
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| Xu Niangui,Peng Longxiang,Lu Wei,et al..GM_2 gangliosidosis[J].Journal of Clinical Neurology,2001(6). |
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| Authors: | Xu Niangui Peng Longxiang Lu Wei |
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| Institution: | Xu Niangui,Peng Longxiang,Lu Wei,et al.Department of Neurology,the Se cond Xiangya Hospital,Zhongnan University,Changsha 410011 |
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| Abstract: | Objective To probe into the clinical features and pathological change of GM 2 gangliosidosis. Methods The clinical manifestations of 2 patients with late onset GM 2 gangliosidosis were reported, a biopsy of the right frontal lobe was performed for each case.Results The clinical manifestations of the late onset GM 2 gangliosidosis were nonspecific, the ballooned neurons with cytoplasmic deposits were found under the light microscopy.The deposits were membranous cytoplasmic bodies(MCB) together with zebra bodies.Conclusion The result suggested that light microscopy with electron microscopy in taking brain biopsy was very important for the diagnosis of GM 2 gangliosidosis. |
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| Keywords: | Gangliosidosis Clinical manifestation Ultrastructure |
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