1949年Alexander尸检1例于发病后8个月内死亡的15个月婴儿,临床表现为智能、运动发育迟滞及脑积水,病理发现于大脑白质及软膜下广泛地被一种称做Rosenthal纤维分布在星状胶质细胞内,故命名为进行性白质变性症.

Objective To assess the characteristics of executive dysfunction in patients with hepatolenticular degeneration (HLD) through neuropsychological tests and explore its mechanism. Methods One hundred and two patients with HLD were chosen and divided into CT/MRI positive (n=70) and CT/MRI negative (n=32) groups in accordance with cerebral imaging and the other 30 healthy subjects were selected as normal control group. These three groups were recorded the results of such imaging data as Raven's standard progressive matrices (R'SPM) test, Stroop color-word test (CWT), trail making test (TMT). Results Imaging data indicated a statistical change of frontal-striatal loops in patients with HLD. Significant differences of the total scores, perception discrimination, serial relationship and abstract reasoning of R'SPM test were showed in the three groups and the word interference time of CWT, B items time-consuming and interference effects of TMT in these groups were statistically different (P<0.05). Conclusion Executive dysfunction appears commonly in patients with HLD. The CT/MRI positive group showed a more serious degree and area of the damage as compared to that in the CT/MRI negative group. Damages of frontal-striatal loops may be one of the important mechanisms of executive dysfunction in patients with HLD.