Rafee Shameer, Diepman Madeleine, McCormack Derval, Monaghan Ruth, Fearon Conor, Hutchinson Michael, O'Keeffe Fiadhnait
Department of Neurology, St Vincent's University Hospital, Ireland.
School of Medicine and Medical Sciences, University College Dublin, Ireland.
Clin Park Relat Disord. 2023 Oct 16;9:100226. doi: 10.1016/j.prdoa.2023.100226. eCollection 2023.
Cervical dystonia (CD) presents as a motor disorder but has a number of non-motor features. Studies have demonstrated diverse changes in cognition in patients with CD. The rarity of this disorder, phenotypic heterogeneity, and, in particular, a lack of consistency in cognitive testing measures limits clear definition of cognitive changes in this disorder. The relationship between cognition, motor symptoms and quality of life has not been well defined. We undertook a comprehensive analysis of cognition in CD.
Patients with adult onset idiopathic isolated CD (AOICD) who had completed a battery of cognitive assessments- general intellectual functioning, verbal and visual memory, executive functions and social cognition measures, were included. Participants were assessed for mood symptoms, motor severity and quality of life.
13 patients (8 women) with AOICD were included covering 40 cognitive subtests. Mean age was 59.9 years and mean TWSTRS-2 severity was 11. Mean estimated premorbid function was in the normal range. Overall performance on most measures were within normal limits. The lowest mean z-score was observed in Florida Affect Battery (social cognition) subtests, z = -1.75 and -0.81. and in verbal recall, z = -0.82. The majority of patients (75%) scored below population mean on spatial working memory and (62%) performed below population mean on word retrieval and working memory.
We provide detailed cognitive results across a wide range of measures. Although patients tended towards average outcomes on the majority of tests, poorer performance than expected averages were noted in measures of social cognition, word retrieval, spatial working memory and, processing speed.
颈部肌张力障碍(CD)表现为一种运动障碍,但具有多种非运动特征。研究表明,CD患者存在多种认知变化。这种疾病的罕见性、表型异质性,尤其是认知测试方法缺乏一致性,限制了对该疾病认知变化的明确定义。认知、运动症状和生活质量之间的关系尚未明确界定。我们对CD患者的认知进行了全面分析。
纳入成年起病的特发性孤立性CD(AOICD)患者,这些患者完成了一系列认知评估,包括一般智力功能、言语和视觉记忆、执行功能以及社会认知测量。对参与者进行情绪症状、运动严重程度和生活质量评估。
纳入13例(8例女性)AOICD患者,涵盖40项认知子测试。平均年龄为59.9岁,平均TWSTRS - 2严重程度为11。平均估计病前功能在正常范围内。大多数测量指标的总体表现均在正常范围内。在佛罗里达情感量表(社会认知)子测试中观察到最低平均z分数,z = -1.75和 -0.81,在言语回忆中,z = -0.82。大多数患者(75%)在空间工作记忆方面得分低于总体平均水平,(62%)在单词检索和工作记忆方面表现低于总体平均水平。
我们提供了广泛测量指标的详细认知结果。尽管大多数测试中患者倾向于平均结果,但在社会认知、单词检索、空间工作记忆和处理速度测量中,发现其表现比预期平均水平差。