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共济失调-神经棘红细胞病(附3例报道) 被引量:2

Ataxia-Neuroacanthocytosis (3 Cases Report)
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摘要 目的:报道3例散发性神经棘红细胞病患者的临床表现。方法:对经电镜证实的3例外周血红细胞中棘红细胞〉5%患者的临床表现和分型,结合复习相关文献予以分析。结果:神经棘红细胞病3例,其中男2例、女1例;年龄35~79岁,平均年龄(56.6±22.0)岁;平均病程(2.6±1.0)年(7个月至5年)。均为隐匿起病,缓慢进展,表现为明显的小脑共济失调体征和步态。MRI示小脑萎缩。1例伴智能减退和异动症,1例高密度脂蛋白高于正常。扫描电镜发现3例患者周围血红细胞中,棘红细胞均〉5%。结论:散发性小脑性共济失调的成人患者应考虑神经棘红细胞病可能。 Aim: To report 3 cases with neuroacanthocytosis.Methods: The percentage of acanthocyte was found more than 5% in peripheral blood in 4 cases by scanning electron microscope.Results: The four cases consisted of 2 males and 1 female.The mean age of onset was 56.6 yrs(35 to 79).The course of clinical development at first examination was near 2.6 yrs(7 months to 5 yrs) and all of the four cases were progressively sporadic.On examination,all of the cases had dysarthria and cerebellar ataxia.One of them had the dyskinesia and dementia that MMSE scale showed 23.Another had the plantar responses.Percentage of acanthocytes in total red blood cells was more than 10%(10%~28%).MRI of all the patients showed cerebellar atrophy.The levels of high density lipoprotein cholesterol were increased in two cases.Conclusion: Neuroacanthocytosis might present a variety of neurological manifestation,including cerebellar ataxia,dysarthria,and etc.
出处 《中国临床神经科学》 2012年第1期10-15,共6页 Chinese Journal of Clinical Neurosciences
关键词 神经棘红细胞病 共济失调 运动障碍 neuroacanthocytosis ataxia movement disorder
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参考文献6

  • 1Feinberg TE,Cianci CD,Morrow JS,et al.Diagnostic tests forchoreoacanthocytosis[J].Neurology,1991,41:1000-1006. 被引量:1
  • 2Hardie RJ,Pullon HW,Harding AE,et al.Neuroacanthocytosis.A clinical haematological and pathological study of 19 cases[J].Brain,1991,114:13-19. 被引量:1
  • 3Danek A,Juang HH,Melone MA,et al.Neuroacanthocytosis:newdevelopments in a neglected group of dementing disorders[J].JNeurol Sci,2005,229-230:171-186. 被引量:1
  • 4蒋雨平.晚发性小脑性共济失调病例讨论[J].中国临床神经科学,2010,18(2):223-224. 被引量:2
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二级参考文献4

  • 1Rampoldi L,Danek A,Monaco AP.Clinical featwres and molecular bases of neuroaconthocytosis[J].J Mol Med,2002,80:475-491. 被引量:1
  • 2Walker RH,Jung HH.Dobson-stone C,et a1.Neurologic phenotypes associated with aconthocytosis[J]. Neurology,2007,68:92-98. 被引量:1
  • 3王新德.神经病学:梁秀龄.神经系统遗传性疾病[M].第19卷.北京:人民卫生出版社,2001:308. 被引量:1
  • 4蒋雨平,周叶.儿童全身性肌张力障碍病例讨论[J].中国临床神经科学,2008,16(5):562-564. 被引量:1

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