Athinoula A. Martinos Center for Biomedical Imaging, Massachusetts General Hospital, Charlestown, MA, 02129, USA; Department of Radiology, Harvard Medical School, Boston, MA, 02115, USA; Department of Neurology, Harvard Medical School, Boston, MA, 02115, USA; Center for Neuroimaging of Aging and Neurodegenerative Diseases, Massachusetts General Hospital, Charlestown, MA, 02129, USA.
Athinoula A. Martinos Center for Biomedical Imaging, Massachusetts General Hospital, Charlestown, MA, 02129, USA; Department of Radiology, Harvard Medical School, Boston, MA, 02115, USA; Department of Neurology, Harvard Medical School, Boston, MA, 02115, USA; Center for Neuroimaging of Aging and Neurodegenerative Diseases, Massachusetts General Hospital, Charlestown, MA, 02129, USA.
Parkinsonism Relat Disord. 2023 Apr;109:105289. doi: 10.1016/j.parkreldis.2023.105289. Epub 2023 Jan 20.
Early non-motor symptoms in Huntington's disease (HD), including visual perceptual difficulties, can have profound negative impacts on quality of life. In particular, deficits in emotion recognition may contribute to misinterpretation of social cues, and may adversely affect interpersonal relationships, work relationships and/or general well-being. This may be particularly salient during the pre-manifest period, a period prior to the onset of motor symptoms. We sought to evaluate impairments in emotion recognition in gene-positive individuals who did not meet criterial for a diagnosis of HD; we also sought to determine associations between emotion recognition processing and altered cortico-striatal circuitry.
We used a standardized battery to evaluate performance on a facial expression recognition task in a cohort of motor pre-manifest HD (Pre-HD) individuals (N = 21). Functional MRI (fMRI) was then used to assess the face processing network in a subset (N = 15).
We found significantly decreased response accuracy to certain facial expressions, particularly of negative emotions (p < 0.001) in Pre-HDs. When Pre-HDs viewed faces with different emotions, activation within the Superior Temporal Sulcus (fSTS) was reduced compared to controls; in contrast, the level of evoked response within other face-selective cortical regions was comparable.
Early deficits in emotion recognition in Pre-HD appear to be associated with alterations in the fSTS response, a distinctly different pathway from that involved in face perception and provide support for early cognitive and behavioral interventions.
亨廷顿病（HD）的早期非运动症状，包括视觉感知困难，可能对生活质量产生深远的负面影响。特别是，情绪识别缺陷可能导致对社会线索的误解，并可能对人际关系、工作关系和/或整体幸福感产生不利影响。这在发病前的时期（即运动症状出现前的时期）可能尤为明显。我们试图评估未达到亨廷顿病诊断标准的基因阳性个体的情绪识别障碍；我们还试图确定情绪识别处理与皮质纹状体回路改变之间的关系。
我们使用标准化的测试套件评估了一组运动前亨廷顿病（Pre-HD）个体（N=21）在面部表情识别任务中的表现。然后，使用功能磁共振成像（fMRI）对其中一部分个体（N=15）评估了面部处理网络。
我们发现 Pre-HD 患者对某些面部表情的反应准确性显著降低，特别是对负面情绪（p<0.001）。当 Pre-HD 患者观看不同情绪的面孔时，与对照组相比，Superior Temporal Sulcus（fSTS）内的激活减少；相比之下，其他面部选择性皮质区域的诱发反应水平相当。
Pre-HD 中的早期情绪识别缺陷似乎与 fSTS 反应的改变有关，这与涉及面部感知的途径明显不同，为早期认知和行为干预提供了支持。
