Robins Wahlin Tarja-Brita, Luszcz Mary A, Wahlin Åke, Byrne Gerard J
Division of Clinical Geriatrics, Department of Neurobiology, Care Sciences and Society, Karolinska Institutet, Stockholm, Sweden; School of Medicine, University of Queensland, Brisbane, Qld., Australia.
School of Psychology, Flinders University, Adelaide, S.A., Australia.
Dement Geriatr Cogn Dis Extra. 2015 Dec 18;5(3):517-29. doi: 10.1159/000441942. eCollection 2015 Sep-Dec.
This study examines non-verbal (design) and verbal (phonemic and semantic) fluency in prodromal Huntington's disease (HD). An accumulating body of research indicates subtle deficits in cognitive functioning among prodromal mutation carriers for HD.
Performance was compared between 32 mutation carriers and 38 non-carriers in order to examine the magnitude of impairment across fluency tasks. The predicted years to onset (PYTO) in mutation carriers was calculated by a regression equation and used to divide the group according to whether onset was predicted as less than 12.75 years (HD+CLOSE; n = 16) or greater than 12.75 years (HD+DISTANT; n = 16).
The results indicate that both non-verbal and verbal fluency is sensitive to subtle impairment in prodromal HD. HD+CLOSE group produced fewer items in all assessed fluency tasks compared to non-carriers. HD+DISTANT produced fewer drawings than non-carriers in the non-verbal task. PYTO correlated significantly with all measures of non-verbal and verbal fluency.
The pattern of results indicates that subtle cognitive deficits exist in prodromal HD, and that less structured tasks with high executive demands are the most sensitive in detecting divergence from the normal range of functioning. These selective impairments can be attributed to the early involvement of frontostriatal circuitry and frontal lobes.
本研究考察前驱期亨廷顿舞蹈症(HD)患者的非言语(设计)及言语(音素和语义)流畅性。越来越多的研究表明,HD前驱期突变携带者存在细微的认知功能缺陷。
比较32名突变携带者和38名非携带者的表现,以检验各项流畅性任务中的损伤程度。通过回归方程计算突变携带者的预测发病年限(PYTO),并根据预测发病年限是否小于12.75年(HD+CLOSE组;n = 16)或大于12.75年(HD+DISTANT组;n = 16)将该组进行划分。
结果表明,非言语和言语流畅性对前驱期HD的细微损伤均敏感。与非携带者相比,HD+CLOSE组在所有评估的流畅性任务中生成的项目较少。在非言语任务中,HD+DISTANT组绘制的图画比非携带者少。PYTO与非言语和言语流畅性的所有测量指标均显著相关。
结果模式表明,前驱期HD存在细微的认知缺陷,且执行要求高、结构较松散的任务在检测与正常功能范围的差异时最为敏感。这些选择性损伤可归因于额纹状体回路和额叶的早期受累。