【摘要】 目的分析常染色体显性遗传性皮层下梗死和白质脑病(cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy, CADASIL)患者语言记忆和视空间记忆功能损害及与脑萎缩的相关性。方法采用华山版听觉词语学习测验(auditory verbal learning test-Huashan version, AVLT-H)和ReyOsterrieth复杂图形测试(Rey-Osterrieth complex figure test, RCFT)评测20例CADASIL患者和与之性别、年龄、受教育程度匹配的对照组的语言记忆和视空间记忆功能。全脑皮层萎缩量表(global cortical atrophy, GCA)评价脑萎缩程度。采用线性回归分析语言记忆和视空间记忆指标与GCA分值的相关性。结果 CADASIL患者听觉词语学习(r=-0.572,P=0.008)和复杂图形临摹表现(r=-0.538,P=0.017)与GCA分值呈负相关;语言回忆和视空间回忆评测项中除听觉词语短延迟回忆遗忘率与GCA分值呈正相关外,听觉词语长延迟回忆、线索回忆、复杂图形回忆与脑萎缩均无统计学相关。结论 CADASIL患者听觉词语学习、短延迟回忆和复杂图形临摹功能损害与脑萎缩严重程度相关,语言和视空间长延迟回忆与脑萎缩严重程度无显著相关。更多还原

【Abstract】 Objective Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy(CADASIL) is the most common and best known monogenic small vessel disease. Cognitive impairment is the common symptom of CADASIL. Memory impairment in patients with CADASIL is still inconclusive. The study was conducted to investigate the impairment of verbal and visuospatial memory in patients with CADASIL and the association of verbal and visuospatial memory impairment with cerebral atrophy. Methods Twenty CADASIL patients and 20 controls matched by age, gender and years of education were recruited. All participants underwent memory and visuospatial measurement with the Auditory Verbal Learning Test-Huashan version(AVLT-H) and Rey-Osterrieth Complex Figure Test(RCFT). The performances of verbal learning, figure copy and delayed recall were recorded and the rates of forgetfulness of delayed recall were calculated. The Global Cortical Atrophy(GCA)scale was used to evaluate the cerebral atrophy by a neuroimaging specialist. Linear regression analysis was used to analyze the association of verbal memory and visuospatial memory with GCA scores. Results The performance of verbal learning(r=-0.572,P=0.008) and RCFT copy(r=-0.538,P=0.017) were negatively associated with the GCA scores. In terms of verbal memory, only the rate of forgetfulness of the listed words in the short delayed recall test was positively associated with the GCA scores, r=0.50,P=0.025. Performances of the delayed and cued recall of the listed words in the AVLT-H test and the delayed recall of complex figure tended to be worse with the increase of the GCA scores,however, the associations were not significant. Conclusion The verbal and visuospatial memory are impaired in patients with CADASIL. The performances of verbal leaning and short delayed recall, complex figure copy are significantly associated with the degree of cerebral atrophy, while no significant association is found between the performances of the long delayed recall of words as well as complex figure and the cerebral atrophy.更多还原