Labuschagne Izelle, Cassidy Amy Mulick, Scahill Rachael I, Johnson Eileanoir B, Rees Elin, O'Regan Alison, Queller Sarah, Frost Chris, Leavitt Blair R, Dürr Alexandra, Roos Raymond, Owen Gail, Borowsky Beth, Tabrizi Sarah J, Stout Julie C
1School of Psychological Sciences,Monash University,Clayton,Victoria,Australia.
3Department of Medical Statistics,London School of Hygiene & Tropical Medicine,London,United Kingdom.
J Int Neuropsychol Soc. 2016 Jul;22(6):595-608. doi: 10.1017/S1355617716000321. Epub 2016 May 23.
Visuospatial processing deficits have been reported in Huntington's disease (HD). To date, no study has examined associations between visuospatial cognition and posterior brain findings in HD.
We compared 119 premanifest (55> and 64<10.8 years to expected disease onset) and 104 early symptomatic (59 stage-1 and 45 stage-2) gene carriers, with 110 controls on visual search and mental rotation performance at baseline and 12 months. In the disease groups, we also examined associations between task performance and disease severity, functional capacity and structural brain measures.
Cross-sectionally, there were strong differences between all disease groups and controls on visual search, and between diagnosed groups and controls on mental rotation accuracy. Only the premanifest participants close to onset took longer than controls to respond correctly to mental rotation. Visual search negatively correlated with disease burden and motor symptoms in diagnosed individuals, and positively correlated with functional capacity. Mental rotation ("same") was negatively correlated with motor symptoms in stage-2 individuals, and positively correlated with functional capacity. Visual search and mental rotation were associated with parieto-occipital (pre-/cuneus, calcarine, lingual) and temporal (posterior fusiform) volume and cortical thickness. Longitudinally, visual search deteriorated over 12 months in stage-2 individuals, with no evidence of declines in mental rotation.
Our findings provide evidence linking early visuospatial deficits to functioning and posterior cortical dysfunction in HD. The findings are important since large research efforts have focused on fronto-striatal mediated cognitive changes, with little attention given to aspects of cognition outside of these areas. (JINS, 2016, 22, 595-608).
已有研究报道亨廷顿舞蹈症(HD)存在视觉空间处理缺陷。迄今为止,尚无研究探讨HD患者视觉空间认知与后脑结构之间的关联。
我们比较了119名临床前期患者(55名年龄大于预期发病年龄10.8岁,64名年龄小于预期发病年龄10.8岁)和104名早期症状期患者(59名处于1期,45名处于2期)基因携带者,以及110名对照者在基线和12个月时的视觉搜索和心理旋转表现。在疾病组中,我们还研究了任务表现与疾病严重程度、功能能力和脑结构测量之间的关联。
横断面分析显示,所有疾病组与对照组在视觉搜索方面存在显著差异,确诊组与对照组在心理旋转准确性方面存在显著差异。只有接近发病的临床前期参与者在心理旋转任务中正确反应的时间比对照组更长。在确诊个体中,视觉搜索与疾病负担和运动症状呈负相关,与功能能力呈正相关。在2期个体中,心理旋转(“相同”)与运动症状呈负相关,与功能能力呈正相关。视觉搜索和心理旋转与顶枕叶(中央前回/楔叶、距状裂、舌回)和颞叶(后梭状回)的体积和皮质厚度相关。纵向分析显示,2期个体的视觉搜索在12个月内有所恶化,而心理旋转没有下降的迹象。
我们的研究结果提供了证据,表明HD患者早期的视觉空间缺陷与功能和后皮质功能障碍有关。这些发现很重要,因为大量研究工作集中在额叶-纹状体介导的认知变化上,而对这些区域之外的认知方面关注较少。(《神经心理学杂志》,2016年,第22卷,第595 - 608页)