目的通过观察脑型肝豆状核变性患者中文书写特点,探讨基底神经节病变与中文书写的关系。方法采用汉语失写检查法对35例脑型肝豆状核变性患者进行中文书写检查。结果①22例患者出现书写障碍(62.9%),以看图书写成绩最差,其他依次为主动书写、听写、自动书写和抄写。②14例患者出现语言性书写障碍(40%),11例患者出现运动性书写障碍(31.4%)。其中存在多种书写障碍类型并存的现象。③在语言性书写障碍的患者中:构字障碍12例,字词错写4例,语法错误2例。④在运动性书写障碍的患者中:小写症5例,震颤性书写4例,重复性书写2例。结论书写障碍是脑型肝豆状核变性的常见症状,以构字障碍最多见,提示基底神经节参与汉字书写过程。
Objective To study the relationship between the basal ganglia disorder and Chinese writing in patients with Wilson's disease. Methods Chinese agraphia battcl7 ( CAB ) , and the Xn and AgQ scores were used to identify agraphia in 35 patients with Wilson's disease. Results ①Agraphia was identified in 22 cases ( 62. 9% ) and the patients had difficulty in writing by picture, positive writing, dictation, automatic writing and copying ( in descending order of sever- ity). ② Language agraphia and movement agraphia or mixed agraphia were detected in 14 (40%) and 11 cases (31.4%), respectively. ③Among patients with language agraphia, 12 cases had character formation disorder, 4 cases, wrong-writing characters; and 2 cases, grammar disorder.④ Among patients with movement agraphia, 5 cases had micrographia; 4 cases, tremor agraphia; and 2 cases, reiterative agraphia. Conclusions Agraphia, especially character formation disorder, is a common symptom in patients with Wilson's disease, suggesting the involvement of the basal ganglia in Chinese writing.