肝豆状核变性与锥体束征

Hepatolenticular degeneration and pyramidal tract sign

为了探讨肝豆状核变性的锥体束损害,作者对确诊的200例肝豆状核变性患者锥体束受累的情况进行了调查,结果发现,82例(41％)患者具有锥体束征,与无锥体束征的肝豆状核变性患者比较,具有锥体束征患者的脑电图异常率明显高于无锥体束征组,锥体束损害与肝豆状核变性病程无关,但与临床类型明显相关,尤以脑—内脏型及肝型肝豆状核变性患者易出现锥体束征,驱铜治疗后,约半数患者锥体束征消失。作者认为,如同锥体外系受损一样,肝豆状核变性也可以出现锥体束征损害。

To explore pyramidal tract damage of hepatolenticular degeneration (HLD), the arthors hadve undertaken a clinical analysis of 200 patients with HLD. Pyramidal tract sign was elicited in 82 patients with HLD. The rate of abnormal electroencepealograhic display in patients with pyramidal tract sige was obvioualy higher than that in patients without. The damage of the pyramidal tract was related not to the HLD course, but to the clinical type. Typically, the brain - viscera - type and liver -type patients of HLD were liable to develop pyramidal tract lesions. After copperdispelling treatment, The pyramidal tract sign disappeared in about half of the patients. The authors hold that When the extrapyramidal system of HLD patients is damaged, the pyramidal tract is affected at the same time.