“虎眼征”对诊断Hallervorden-Spartz病的意义
王诗男;孙斌;
摘要(Abstract)：
Hallervorden-Spatz病(HSD)是由于铁代谢障碍导致Fe3+在苍白球、黑质、红核等部位内沉积所引起的一种罕见中枢神经系统变性病,具有很强的临床和遗传异质性。病理特异性的表现为含铁色素聚集,神经轴索肿胀,神经元脱失。临床表现以锥体外系受累为主,以姿势和步态异常、肌张力增高和不自主运动为起始症状,常见舞蹈、手足徐动或震颤;晚期出现构音、吞咽困难、锥体束征和智能障碍。根据临床表现的不同可以分为早发型和晚发型两种类型。头颅MRIT2加权像可见苍白球和黑质呈低信号,并在双侧苍白球前内侧低信号的背景上对称出现高信号,即"虎眼征"。目前尚无明确的生化指标可以帮助诊断该病,脑MRIT2加权像出现"虎眼征"对HSD的生前诊断具有重要意义。
关键词(KeyWords)： Hallervorden-Spatz病;T2加权像;虎眼征
基金项目(Foundation):
作者(Authors): 王诗男;孙斌;
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