The differential diagnosis of chorea.

The differential diagnosis of chorea.
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DOI:
10.1136/pn.2007.134585
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发表时间:
2007-11-01
影响因子:
2.8
通讯作者:
Tabrizi, S J
Tabrizi, S J
中科院分区:
其他
文献类型:
--
作者:
Wild, E J;Tabrizi, S J

文献摘要

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舞蹈病是一种多动性运动障碍,其特征是时间不规则、随机分布和突然的过度自发运动。在这篇文章中,作者讨论了舞蹈病的原因,特别是亨廷顿病和可能类似于它的遗传综合征,包括HDL 1 -3,遗传性朊病毒病,脊髓小脑共济失调1,3和17,神经棘红细胞增多症,齿状红核苍白球路易体萎缩(DRPLA),脑铁积累障碍,威尔逊病,良性遗传性舞蹈病,弗里德赖希共济失调和线粒体疾病。获得性舞蹈病的病因包括血管疾病、感染后自身免疫性中枢神经系统疾病(PANDAS)、药物、系统性红斑狼疮、抗磷脂综合征、甲状腺毒症、艾滋病、妊娠舞蹈病和真性红细胞增多症。作者提出了一种舞蹈病临床评估的方法,研究的价值,包括基因检测(他们提供了一个结构化的框架,强调事先咨询的重要性),最后简要讨论了舞蹈病的对症药物治疗。
Chorea is a hyperkinetic movement disorder characterised by excessive spontaneous movements that are irregularly timed, randomly distributed and abrupt. In this article, the authors discuss the causes of chorea, particularly Huntington's disease and the genetic syndromes that may resemble it, including HDL1-3, inherited prion disease, spinocerebellar ataxias 1, 3 and 17, neuroacanthocytosis, dentatorubro-pallidoluysian atrophy (DRPLA), brain iron accumulation disorders, Wilson's disease, benign hereditary chorea, Friedreich's ataxia and mitochondrial disease. Acquired causes of chorea include vascular disease, post-infective autoimmune central nervous system disorders (PANDAS), drugs, systemic lupus erythematosus, antiphospholipid syndrome, thyrotoxicosis, AIDS, chorea gravidarum, and polycythaemia rubra vera. The authors suggest an approach to the clinical assessment of chorea, the value of investigations, including genetic tests (for which they offer a structured framework highlighting the importance of prior counselling), and finally briefly discuss symptomatic drug treatment of chorea.