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肌萎缩侧索硬化后综合征(PPS)的非运动症状:疲劳、认知症状和影像学特征。

Extra-motor manifestations in post-polio syndrome (PPS): fatigue, cognitive symptoms and radiological features.

机构信息

Computational Neuroimaging Group, Trinity Biomedical Sciences Institute, Trinity College Dublin, Room 5.43, Pearse Street, Dublin 2, Ireland.

出版信息

Neurol Sci. 2021 Nov;42(11):4569-4581. doi: 10.1007/s10072-021-05130-4. Epub 2021 Feb 26.

DOI:10.1007/s10072-021-05130-4
PMID:33635429
Abstract

BACKGROUND

There is a paucity of cerebral neuroimaging studies in post-polio syndrome (PPS), despite the severity of neurological and neuropsychological sequelae associated with the condition. Fatigue, poor concentration, limited exercise tolerance, paraesthesia and progressive weakness are frequently reported, but the radiological underpinnings of these symptoms are poorly characterised.

OBJECTIVE

The aim of this study is to evaluate cortical and subcortical alterations in a cohort of adult polio survivors to explore the anatomical substrate of extra-motor manifestations.

METHODS

Thirty-six patients with post-polio syndrome, a disease-control group with amyotrophic lateral sclerosis patients and a cohort of healthy individuals were included in a prospective neuroimaging study with a standardised clinical and radiological protocol. Validated clinical instruments were utilised to assess mood, cognitive and behavioural domains and specific aspects of fatigue. Cortical thickness analyses, subcortical volumetry, brainstem segmentation and region-of-interest (ROI) white matter analyses were undertaken to assess regional grey and white matter alterations.

RESULTS

A high proportion of PPS patients exhibited apathy, verbal fluency deficits and reported self-perceived fatigue. On ROI analyses, cortical atrophy was limited to the cingulate gyrus, and the temporal pole and subcortical atrophy were only detected in the left nucleus accumbens. No FA reductions were noted to indicate white matter degeneration in any of the lobes.

CONCLUSIONS

Despite the high incidence of extra-motor manifestations in PPS, only limited cortical, subcortical and white matter degeneration was identified. Our findings suggest that non-structural causes, such as polypharmacy and poor sleep, may contribute to the complex symptomatology of post-polio syndrome.

摘要

背景

尽管与后灰质炎综合征(PPS)相关的神经和神经心理学后遗症严重,但针对该疾病的大脑神经影像学研究却很少。疲劳、注意力不集中、运动耐量有限、感觉异常和进行性无力等症状经常被报道,但这些症状的放射学基础尚未得到很好的描述。

目的

本研究旨在评估一组成年脊髓灰质炎幸存者的皮质和皮质下改变,以探索运动外表现的解剖学基础。

方法

将 36 名患有后灰质炎综合征的患者、一组肌萎缩侧索硬化症患者和一组健康个体纳入一项前瞻性神经影像学研究,采用标准化的临床和放射学方案。使用经过验证的临床仪器评估情绪、认知和行为领域以及特定方面的疲劳。进行皮质厚度分析、皮质下体积测量、脑干分割和感兴趣区域(ROI)白质分析,以评估区域灰质和白质改变。

结果

相当一部分 PPS 患者表现出冷漠、言语流畅性缺陷,并报告自我感知疲劳。在 ROI 分析中,皮质萎缩仅限于扣带回,颞极和皮质下萎缩仅在左侧伏隔核中检测到。在任何脑叶中都没有发现 FA 降低表明白质退化。

结论

尽管 PPS 中运动外表现的发生率很高,但仅发现有限的皮质、皮质下和白质退化。我们的研究结果表明,非结构性原因,如多药治疗和睡眠不佳,可能导致后灰质炎综合征的复杂症状。

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