脑型肝豆状核变性患者的智能、记忆障碍研究

目的了解脑型肝豆状核变性(HLD)患者发生智能、记忆障碍的状况。方法采用龚氏修定的韦氏成人智力量表中国版、记忆量表中国版,对40例脑型HLD患者及相匹配的40例正常对照组进行检查研究。结果脑型HLD患者组智商、记忆商测验值与正常对照组比较差异有高度显著性(P<0.01),26例智力低于正常,其中10例出现智能障碍;32例记忆力低于正常,其中记忆障碍者17例。40例患者中全部病例脑部MRI、CT均有异常,病变累及范围广泛且较对称,发病部位主要在基底节区,部分病例波及丘脑,其中有30例伴有程度不等的广泛性大脑皮层萎缩,16例以额颞叶萎缩为重,伴有脑室轻度扩大。结论脑型HLD患者存在明显智能、记忆障碍,广泛性大脑皮层萎缩,尤其是额颞叶萎缩是主要原因之一。

Intellectual and memory disorders in patients with cerebral hepatolenticular degeneration

Objective To summarize the memory and intellectual disorders in patients with cerebral hepatolenticular degeneration.Methods 40 subjects with cerebral hepatolentcular degeneration and 40 healthy subjects were involved in theis study.All the subjects were examined by using the Chinese revision of the Wechsler Adults Intelligence Scales and the memory scale.The data were statistically analyzed.Results In comparison to normal subjects,the evaluation values of intelligence quotient,memory quotient in patients with cerebral hepatolentcular degeneration were higher,there were statistical significant differences(P<0.01),26 cases had lower intelligence quotient,of 10 had intelligence disorder;32 had lower memory quotient,of 17 cases had memory disorder.40 patients all had abnormality of MRI,CT screening,the lesions were extensive and symmetrical.The lesions mainly located in basal ganglia,some extended to thalamus,of 30 cases complicated with different degrees of extensive cerebral cortex atrophy,and of 16 had serious frontotemporal lobe atrophy associated with slight ventricular enlargement.Conclusion Patients with cerebral hepatolenticular degeneration had intelligence and memory disorders,the main cause was extensive cerebral cortex atrophy,especially frontotemporal lobe atrophy.