ABSTRACT

Introduction: Emotional issues are often reported among individuals with myotonic dystrophy type 1 (DM1) and some studies have suggested that deficits in ability to quickly encode emotions may contribute to these problems. However, poor performance on emotion encoding tasks could also be explained by a more general cognitive deficit (Full Scale IQ [FSIQ]), rather than a specific deficit in emotional processing. Since individuals with DM1 are known to exhibit difficulties in general cognitive abilities, it is important to account for FSIQ when evaluating emotion encoding. The aim of this study was to compare emotion encoding abilities between individuals with and without DM1, while adjusting for the impact of general cognitive abilities (FSIQ).

Methods: The sample included 35 individuals with adult-onset DM1 and 54 unaffected adults who completed assessments of emotion encoding abilities (Ekman faces test) and general cognitive abilities (Wechsler Adult Intelligence Scale-IV). Performance on the emotion encoding task was operationalized as proportion correct and response time. Group differences in proportion correct were evaluated with generalized linear regression, while linear regression models were used to determine the effect of group on response time. Models were adjusted for age, sex, and FSIQ. The false discovery rate (FDR) was applied to control false positives due to multiple comparisons (p_fdr ).

Results: No significant group differences were observed for emotion encoding abilities (all p_fdr > 0.13). FSIQ was significantly associated with proportion correct and with response time (all p_fdr < 0.05).

Conclusions: Emotion encoding appears intact in individuals with DM1 and variation in the ability to encode facial expressions was associated with FSIQ. Further research is required to address the relationship between general cognitive abilities and emotion encoding abilities among DM1 patients.

摘要

简介：在患有 1 型强直性营养不良 (DM1) 的个体中经常报告情绪问题，一些研究表明，快速编码情绪的能力缺陷可能会导致这些问题。然而，情绪编码任务的糟糕表现也可以用更普遍的认知缺陷（全量程智商 [FSIQ]）来解释，而不是情绪处理中的特定缺陷。由于已知患有 DM1 的个体在一般认知能力方面表现出困难，因此在评估情绪编码时考虑 FSIQ 很重要。本研究的目的是比较有和没有 DM1 的个体之间的情绪编码能力，同时调整一般认知能力 (FSIQ) 的影响。

方法：样本包括 35 名成人发病 DM1 和 54 名未受影响的成年人，他们完成了情绪编码能力（Ekman 面部测试）和一般认知能力（韦克斯勒成人智力量表-IV）的评估。情感编码任务的性能被操作为比例正确和响应时间。用广义线性回归评估正确比例的组差异，而线性回归模型用于确定组对响应时间的影响。模型根据年龄、性别和 FSIQ 进行了调整。错误发现率 (FDR) 用于控制由于多重比较 ( p _fdr )引起的误报。

结果：在情绪编码能力方面没有观察到显着的组间差异（所有p _fdr > 0.13）。FSIQ 与正确比例和响应时间显着相关（所有p _fdr < 0.05）。

结论：情感编码在 DM1 个体中完好无损，并且面部表情编码能力的变化与 FSIQ 相关。需要进一步研究来解决 DM1 患者的一般认知能力与情绪编码能力之间的关系。