Cognitive impairment in Chinese adult patients with type III spinal muscular atrophy without disease-modifying treatment
ObjectiveSpinal muscular atrophy (SMA) is a neurodegenerative disorder characterized by the degeneration of motor neurons in the spinal cord. It remains uncertain whether the cognitive performance of adult patients with SMA is impaired. The objective of this study was to assess the cognitive profile...
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| Format: | Article |
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Frontiers Media S.A.
2023-11-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2023.1226043/full |
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| author | Ying Hu Ying Hu Ying Hu Ling Wei Ling Wei Ling Wei Aonan Li Aonan Li Aonan Li Tingting Liu Tingting Liu Tingting Liu Yubao Jiang Yubao Jiang Yubao Jiang Chengjuan Xie Chengjuan Xie Chengjuan Xie Kai Wang Kai Wang Kai Wang |
| author_facet | Ying Hu Ying Hu Ying Hu Ling Wei Ling Wei Ling Wei Aonan Li Aonan Li Aonan Li Tingting Liu Tingting Liu Tingting Liu Yubao Jiang Yubao Jiang Yubao Jiang Chengjuan Xie Chengjuan Xie Chengjuan Xie Kai Wang Kai Wang Kai Wang |
| author_sort | Ying Hu |
| collection | DOAJ |
| description | ObjectiveSpinal muscular atrophy (SMA) is a neurodegenerative disorder characterized by the degeneration of motor neurons in the spinal cord. It remains uncertain whether the cognitive performance of adult patients with SMA is impaired. The objective of this study was to assess the cognitive profile of adult Chinese patients with SMA and the association between clinical features and cognitive ability, particularly executive function.MethodsThis cross-sectional study included 22 untreated adult patients with type III SMA and 20 healthy subjects. The following variables were assessed: general intelligence, memory, attention, language, executive function, depression, anxiety, and other demographic and clinical parameters. In addition, physical function was evaluated using the Hammersmith Functional Motor Scale Expanded (HFMSE), the Revised Upper Limb Module (RULM), and the 6-Minute Walk Test (6MWT).ResultsSMA patients had lower scores than healthy subjects in the Verbal Fluency Test, Stroop effect, Total Errors, Perseverative Responses, Perseverative Errors, and Non-perseverative Errors in the Wisconsin Card Sorting Test, showing impaired abilities of SMA patients in executive function. In the Attention Network Test (ANT), the results indicated that the SMA patients also had selective deficits in their executive control networks. Ambulant patients had better executive function test performance than non-ambulant ones. Compromised executive abilities in patients with SMA were correlated with a younger age at onset, poorer motor function, and higher levels of anxiety and depression.ConclusionOur study presented the distribution of cognitive impairment in a Chinese cohort with SMA. Patients with type III SMA showed selective deficits in executive function, which may be associated with disease severity, physical impairment, depression and anxiety. Future cognitive studies, accounting for motor and emotional impairment, are needed to evaluate if executive impairment is driven by specific brain changes or by those confounding factors. |
| first_indexed | 2024-03-11T13:20:14Z |
| format | Article |
| id | doaj.art-1acb6dd507e34ab2a2461b02873ecbc5 |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-03-11T13:20:14Z |
| publishDate | 2023-11-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neurology |
| spelling | doaj.art-1acb6dd507e34ab2a2461b02873ecbc52023-11-03T10:29:38ZengFrontiers Media S.A.Frontiers in Neurology1664-22952023-11-011410.3389/fneur.2023.12260431226043Cognitive impairment in Chinese adult patients with type III spinal muscular atrophy without disease-modifying treatmentYing Hu0Ying Hu1Ying Hu2Ling Wei3Ling Wei4Ling Wei5Aonan Li6Aonan Li7Aonan Li8Tingting Liu9Tingting Liu10Tingting Liu11Yubao Jiang12Yubao Jiang13Yubao Jiang14Chengjuan Xie15Chengjuan Xie16Chengjuan Xie17Kai Wang18Kai Wang19Kai Wang20Department of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei, ChinaThe School of Mental Health and Psychological Sciences, Anhui Medical University, Hefei, ChinaAnhui Province Key Laboratory of Cognition and Neuropsychiatric Disorders, Hefei, ChinaDepartment of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei, ChinaThe School of Mental Health and Psychological Sciences, Anhui Medical University, Hefei, ChinaAnhui Province Key Laboratory of Cognition and Neuropsychiatric Disorders, Hefei, ChinaDepartment of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei, ChinaThe School of Mental Health and Psychological Sciences, Anhui Medical University, Hefei, ChinaAnhui Province Key Laboratory of Cognition and Neuropsychiatric Disorders, Hefei, ChinaDepartment of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei, ChinaThe School of Mental Health and Psychological Sciences, Anhui Medical University, Hefei, ChinaAnhui Province Key Laboratory of Cognition and Neuropsychiatric Disorders, Hefei, ChinaDepartment of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei, ChinaThe School of Mental Health and Psychological Sciences, Anhui Medical University, Hefei, ChinaAnhui Province Key Laboratory of Cognition and Neuropsychiatric Disorders, Hefei, ChinaDepartment of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei, ChinaThe School of Mental Health and Psychological Sciences, Anhui Medical University, Hefei, ChinaAnhui Province Key Laboratory of Cognition and Neuropsychiatric Disorders, Hefei, ChinaDepartment of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei, ChinaThe School of Mental Health and Psychological Sciences, Anhui Medical University, Hefei, ChinaAnhui Province Key Laboratory of Cognition and Neuropsychiatric Disorders, Hefei, ChinaObjectiveSpinal muscular atrophy (SMA) is a neurodegenerative disorder characterized by the degeneration of motor neurons in the spinal cord. It remains uncertain whether the cognitive performance of adult patients with SMA is impaired. The objective of this study was to assess the cognitive profile of adult Chinese patients with SMA and the association between clinical features and cognitive ability, particularly executive function.MethodsThis cross-sectional study included 22 untreated adult patients with type III SMA and 20 healthy subjects. The following variables were assessed: general intelligence, memory, attention, language, executive function, depression, anxiety, and other demographic and clinical parameters. In addition, physical function was evaluated using the Hammersmith Functional Motor Scale Expanded (HFMSE), the Revised Upper Limb Module (RULM), and the 6-Minute Walk Test (6MWT).ResultsSMA patients had lower scores than healthy subjects in the Verbal Fluency Test, Stroop effect, Total Errors, Perseverative Responses, Perseverative Errors, and Non-perseverative Errors in the Wisconsin Card Sorting Test, showing impaired abilities of SMA patients in executive function. In the Attention Network Test (ANT), the results indicated that the SMA patients also had selective deficits in their executive control networks. Ambulant patients had better executive function test performance than non-ambulant ones. Compromised executive abilities in patients with SMA were correlated with a younger age at onset, poorer motor function, and higher levels of anxiety and depression.ConclusionOur study presented the distribution of cognitive impairment in a Chinese cohort with SMA. Patients with type III SMA showed selective deficits in executive function, which may be associated with disease severity, physical impairment, depression and anxiety. Future cognitive studies, accounting for motor and emotional impairment, are needed to evaluate if executive impairment is driven by specific brain changes or by those confounding factors.https://www.frontiersin.org/articles/10.3389/fneur.2023.1226043/fullneuromuscular diseasesspinal muscular atrophycognitionexecutive functionemotion |
| spellingShingle | Ying Hu Ying Hu Ying Hu Ling Wei Ling Wei Ling Wei Aonan Li Aonan Li Aonan Li Tingting Liu Tingting Liu Tingting Liu Yubao Jiang Yubao Jiang Yubao Jiang Chengjuan Xie Chengjuan Xie Chengjuan Xie Kai Wang Kai Wang Kai Wang Cognitive impairment in Chinese adult patients with type III spinal muscular atrophy without disease-modifying treatment Frontiers in Neurology neuromuscular diseases spinal muscular atrophy cognition executive function emotion |
| title | Cognitive impairment in Chinese adult patients with type III spinal muscular atrophy without disease-modifying treatment |
| title_full | Cognitive impairment in Chinese adult patients with type III spinal muscular atrophy without disease-modifying treatment |
| title_fullStr | Cognitive impairment in Chinese adult patients with type III spinal muscular atrophy without disease-modifying treatment |
| title_full_unstemmed | Cognitive impairment in Chinese adult patients with type III spinal muscular atrophy without disease-modifying treatment |
| title_short | Cognitive impairment in Chinese adult patients with type III spinal muscular atrophy without disease-modifying treatment |
| title_sort | cognitive impairment in chinese adult patients with type iii spinal muscular atrophy without disease modifying treatment |
| topic | neuromuscular diseases spinal muscular atrophy cognition executive function emotion |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2023.1226043/full |
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