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Hallervorden-Spatz综合征的临床和影像学特点——附2例报告及文献复习 被引量：2

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摘要 目的分析Hallervorden-Spatz综合征的临床和影像学特点。方法回顾性分析了2例Hallervorden-Spatz综合征患者的临床表现和影像学特点。1例患者进行了MRI检查,另1例进行MRI及磁共振波谱(magnetic resonance spectroscopy,MRS)检查。结果2例患者均表现出锥体外系症状和典型的"虎眼征"。1例患者起病年龄早,存在智能障碍,视力障碍和锥体束受累。另1例起病年龄较晚,构音障碍明显,病程进展快,MRS显示出NAA值和NAA/Cr比值下降。结论影像学特点("虎眼征")和临床症状是临床实践中明确诊断HSS的重要依据。NAA值和NAA/Cr比值可以帮助预测HSS患者病灶局部神经元受损情况。
作者 肖力 袁强 方登富 何俐
机构地区 四川大学华西医院神经内科
出处 《中国神经精神疾病杂志》 CAS CSCD 北大核心 2009年第10期618-620,共3页 Chinese Journal of Nervous and Mental Diseases
关键词 HALLERVORDEN-SPATZ综合征 磁共振成像 磁共振波谱学
分类号 R445.2 [医药卫生—影像医学与核医学] R741 [医药卫生—神经病学与精神病学]
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参考文献10

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同被引文献30

  • 1Bala s I, Kovacs N, Holl6dy K. Staged bilateral stereotactic pallidothalamotomy for life-threatening dystonia in a child with Hallervorden Spatz disease. Mov Disord, 2006, 21:82-85.
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  • 7Gregory A, Polster BJ, Hayflick SJ. Clinical and genetic delineation of neurodegeneration with brain iron accumulation. J Med Genet, 2009, 46:73-80.
  • 8Morgan NV, Westaway SK, Morton JE, et al. PLA2G6, encoding a phospholipase A2, is mutated in neurodegenerative disorders with high brain iron. Nat Genet, 2006, 38:752-754.
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  • 10Koeppen AH, Diekson AC. Iron in the Hallervorden- Spatz syndrome. Pediatr Neurol, 2001, 25:148-155.

引证文献2

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中国神经精神疾病杂志
2009年 第10期

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