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Cognitive Profile of Patients With Mitochondrial Chronic Progressive External Ophthalmoplegia
Zhang, Guanyu1,2; Hou, Yue3; Wang, Zhaoxia3; Ye, Zheng4
First AuthorZhang, Guanyu
Correspondent Emailzhaoxia wang drwangzx@163.com ; zheng ye yez@ion.ac.cn
Contribution Rank1
Abstract

Mitochondrial chronic progressive external ophthalmoplegia (CPEO) is a major manifestation of human mitochondrial encephalomyopathies. Previous studies have shown cognitive deficits in patients with mitochondrial diseases. However, these studies often included patients with heterogeneous subtypes of mitochondrial diseases. Here, we aimed to provide a better cognitive profile of patients with CPEO by applying a comprehensive battery of neuropsychological assessments in a pure sample of patients with CPEO. We recruited 28 patients with CPEO (19 women, age 16-62 years) and 38 age- and education-matched healthy control subjects (25 women, age 16-60 years). The neuropsychological assessments covered global cognition and five cognitive domains (executive functions, language, working memory, memory, and visuospatial functions). We found that the patients were impaired in global cognition [Montreal Cognitive Assessment (MoCA)], executive functions [Trail Making Test Part B (TMT-B)], and language [Boston Naming Test (BNT)], but not in working memory, memory or visuospatial functions. Moreover, individual patients' performances in the TMT-B (completion time) were predicted by the severity of non-ophthalmoplegia mitochondrial symptoms/signs [Newcastle Mitochondrial Disease Adult Scale (NMDAS)] and duration of the mitochondrial disease (years). Namely, patients with more severe non-ophthalmoplegia mitochondrial symptoms/signs and a longer disease duration took a longer time to complete the TMT-B. No clinical measures predicted individual patients' performances in the BNT.

Keywordmitochondrial chronic progressive external ophthalmoplegia cognition executive functions language working memory memory visuospatial functions
2020-01-29
Language英语
DOI10.3389/fneur.2020.00036
Source PublicationFRONTIERS IN NEUROLOGY
ISSN1664-2295
Volume11Pages:7
Subtypearticle
Indexed BySCI
Funding ProjectNational Natural Science Foundation of China[31771216] ; Shanghai Municipal Science and Technology Major Project[2018SHZDZX05]
PublisherFRONTIERS MEDIA SA
WOS KeywordCHINESE VERSION ; ADULTS ; MELAS ; VALIDATION ; DISORDERS ; DEMENTIA ; DEFICITS ; DISEASE ; SCALE
WOS Research AreaNeurosciences & Neurology
WOS SubjectClinical Neurology ; Neurosciences
WOS IDWOS:000514398100001
Citation statistics
Document Type期刊论文
Identifierhttp://ir.psych.ac.cn/handle/311026/30951
Collection健康与遗传心理学研究室
Corresponding AuthorWang, Zhaoxia; Ye, Zheng
Affiliation1.Chinese Acad Sci, Inst Psychol, Beijing, Peoples R China
2.Univ Chinese Acad Sci, Dept Psychol, Beijing, Peoples R China
3.Peking Univ, Dept Neurol, Hosp 1, Beijing, Peoples R China
4.Chinese Acad Sci, Key Lab Primate Neurobiol, Inst Neurosci, Ctr Excellence Brain Sci & Intelligence Technol, Shanghai, Peoples R China
First Author AffilicationInstitute of Psychology, Chinese Academy of Sciences
Recommended Citation
GB/T 7714
Zhang, Guanyu,Hou, Yue,Wang, Zhaoxia,et al. Cognitive Profile of Patients With Mitochondrial Chronic Progressive External Ophthalmoplegia[J]. FRONTIERS IN NEUROLOGY,2020,11:7.
APA Zhang, Guanyu,Hou, Yue,Wang, Zhaoxia,&Ye, Zheng.(2020).Cognitive Profile of Patients With Mitochondrial Chronic Progressive External Ophthalmoplegia.FRONTIERS IN NEUROLOGY,11,7.
MLA Zhang, Guanyu,et al."Cognitive Profile of Patients With Mitochondrial Chronic Progressive External Ophthalmoplegia".FRONTIERS IN NEUROLOGY 11(2020):7.
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