Larsson Maria U, Almkvist Ove, Luszcz Mary A, Wahlin Tarja-Brita Robins
Department of Neurobiology, Care Sciences and Society, Karolinska Institutet, Stoskholm, Sweden.
Neuropsychology. 2008 Sep;22(5):596-605. doi: 10.1037/0894-4105.22.5.596.
The aim of the present study was to investigate verbal fluency in preclinical Huntington's disease (HD). Phonemic and semantic fluency and the rate of word production over time were assessed for 29 asymptomatic gene carriers and 34 noncarriers of HD. The relationship between fluency tasks and other cognitive domains was investigated. Compared to noncarriers, carriers produced fewer words and produced them more slowly in the phonemic fluency task but not in the semantic fluency task. When the carrier group was divided on the basis of Predicted-Years-To-Onset (PYTO), only carriers with <12 PYTO performed worse than noncarriers on both fluency tasks. Correlational analyses revealed that phonemic fluency was associated with cognitive speed and working memory, while semantic fluency was linked with crystallized abilities. The difference between carriers and noncarriers in phonemic fluency and a difference between the two carrier groups (<12 PYTO and >or=12 PYTO) in semantic fluency, but not in phonemic fluency, suggest that frontostriatal deficits may precede temporal involvement in preclinical HD.
本研究的目的是调查临床前亨廷顿舞蹈症（HD）患者的言语流畅性。对29名无症状基因携带者和34名非HD携带者进行了音素流畅性、语义流畅性以及随时间的单词生成速率评估。研究了流畅性任务与其他认知领域之间的关系。与非携带者相比，携带者在音素流畅性任务中生成的单词更少且生成速度更慢，但在语义流畅性任务中并非如此。当根据预测发病年限（PYTO）对携带者组进行划分时，只有预测发病年限小于12年的携带者在两项流畅性任务中的表现比非携带者差。相关性分析表明，音素流畅性与认知速度和工作记忆相关，而语义流畅性与晶体能力相关。携带者和非携带者在音素流畅性上的差异，以及两个携带者组（预测发病年限小于12年和大于或等于12年）在语义流畅性而非音素流畅性上的差异，表明在临床前HD中，额纹状体缺陷可能先于颞叶受累。
