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MELAS 综合征患者的认知障碍、临床严重程度和 MRI 改变。

Cognitive impairment, clinical severity and MRI changes in MELAS syndrome.

机构信息

Department of Neurology, University of Halle (Saale), Germany.

Department of Neurology, University of Halle (Saale), Germany.

出版信息

Mitochondrion. 2019 Jan;44:53-57. doi: 10.1016/j.mito.2017.12.012. Epub 2017 Dec 29.

Abstract

OBJECTIVE

To examine clinical severity, cognitive impairment, and MRI changes in patients with MELAS syndrome.

METHODS

Cognitive-mnestic functions, brain MRI (lesion load, cella media index) and clinical severity of ten patients with MELAS syndrome were examined. All patients carried the m.3243A>G mutation.

RESULTS

The detailed neuropsychological assessment revealed cognitive deficits in attention, executive function, visuoperception, and -construction. There were significant correlations between these cognitive changes, lesion load in MRI, disturbances in everyday life (clinical scale), and high scores in NMDAS.

CONCLUSION

Patients with MELAS syndrome showed no global neuropsychological deficit, but rather distinct cognitive deficits.

摘要

目的

探讨 MELAS 综合征患者的临床严重程度、认知障碍和 MRI 改变。

方法

对 10 例 MELAS 综合征患者的认知功能、脑 MRI(病灶负荷、中线指数)和临床严重程度进行了检查。所有患者均携带 m.3243A>G 突变。

结果

详细的神经心理学评估显示,注意力、执行功能、视知觉和构建方面存在认知缺陷。这些认知变化与 MRI 病灶负荷、日常生活障碍(临床量表)和 NMDAS 高分之间存在显著相关性。

结论

MELAS 综合征患者无总体神经心理学缺陷,但存在明显的认知缺陷。

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