Abstract：
The cognitive functioning of individuals with spinal muscular atrophy (SMA) is not well understood, prompting a call for more research to better grasp cognitive involvement in SMA. This study aims to explore recent findings regarding cognitive outcomes in SMA patients, including correlations between clinical features and cognitive abilities. The investigation seeks to identify commonly used measures for assessing cognitive function in this patient population. A scoping review following the Joanna Briggs Institute methodology examined literature until December 2023. Two databases were searched along with relevant article references using specific terms such as \"spinal muscular atrophy,\" \"SMA,\" \"cognitive,\" \"abilities,\" \"functions,\" \"intellective,\" or \"intellectual.\" Screening focused on titles and abstracts from English language peer-reviewed journals. After the initial research, 1452 articles were identified. Subsequent screening and selection led to the inclusion of 13 articles in the review. Among these studies, four indicated a cognitive trend within the normal range for SMA patients. In four other studies, the majority of patients fell within the normal range. However, smaller proportions were observed to be either above or below the norm compared to the controls. Three studies reported noted cognitive performance below the average, while two showed above-average scores. The scoping review suggests that most SMA patients have cognitive abilities similar to the general population, with types II and III showing even lesser impact. However, certain cognitive domains may be affected in type I patients, highlighting the need for further research to fully understand cognitive involvement in SMA.
摘要：
脊髓性肌萎缩症（SMA）患者的认知功能尚未得到很好的理解，促使人们呼吁进行更多的研究，以更好地掌握SMA中的认知参与。这项研究旨在探索SMA患者认知结果的最新发现，包括临床特征和认知能力之间的相关性。调查旨在确定该患者人群中评估认知功能的常用措施。根据乔安娜·布里格斯研究所的方法进行的范围审查审查了直到2023年12月的文献。使用诸如“脊髓性肌萎缩症”之类的特定术语搜索了两个数据库以及相关的文章参考文献，\"\"SMA,“\”认知，\"\"能力，\"\"函数,\"\"智力，\"或\"知识分子。“筛选的重点是英语同行评审期刊的标题和摘要。经过初步研究,共识别1452篇文章。随后的筛选和选择导致在审查中纳入了13篇文章。在这些研究中,4例显示SMA患者的认知趋势在正常范围内.在另外四项研究中，大多数患者落在正常范围内.然而,与对照组相比，观察到较小的比例高于或低于常模。三项研究报告指出认知表现低于平均水平，而两人的得分高于平均水平。范围审查表明，大多数SMA患者的认知能力与一般人群相似，II型和III型的影响更小。然而,I型患者的某些认知领域可能会受到影响，强调需要进一步研究以充分理解SMA中的认知参与。
