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Frontotemporal Pathology in Motor Neuron Disease Phenotypes: Insights From Neuroimaging.
Frontiers in Neurology ( IF 3.4 ) Pub Date : 2021-08-16 , DOI: 10.3389/fneur.2021.723450
Mary Clare McKenna 1 , Philippe Corcia 2, 3, 4 , Philippe Couratier 4, 5 , We Fong Siah 1 , Pierre-Francois Pradat 6 , Peter Bede 1, 6
Affiliation  

Frontotemporal involvement has been extensively investigated in amyotrophic lateral sclerosis (ALS) but remains relatively poorly characterized in other motor neuron disease (MND) phenotypes such as primary lateral sclerosis (PLS), progressive muscular atrophy (PMA), spinal muscular atrophy (SMA), spinal bulbar muscular atrophy (SBMA), post poliomyelitis syndrome (PPS), and hereditary spastic paraplegia (HSP). This review focuses on insights from structural, metabolic, and functional neuroimaging studies that have advanced our understanding of extra-motor disease burden in these phenotypes. The imaging literature is limited in the majority of these conditions and frontotemporal involvement has been primarily evaluated by neuropsychology and post mortem studies. Existing imaging studies reveal that frontotemporal degeneration can be readily detected in ALS and PLS, varying degree of frontotemporal pathology may be captured in PMA, SBMA, and HSP, SMA exhibits cerebral involvement without regional predilection, and there is limited evidence for cerebral changes in PPS. Our review confirms the heterogeneity extra-motor pathology across the spectrum of MNDs and highlights the role of neuroimaging in characterizing anatomical patterns of disease burden in vivo. Despite the contribution of neuroimaging to MND research, sample size limitations, inclusion bias, attrition rates in longitudinal studies, and methodological constraints need to be carefully considered. Frontotemporal involvement is a quintessential clinical facet of MND which has important implications for screening practices, individualized management strategies, participation in clinical trials, caregiver burden, and resource allocation. The academic relevance of imaging frontotemporal pathology in MND spans from the identification of genetic variants, through the ascertainment of presymptomatic changes to the design of future epidemiology studies.

中文翻译:

运动神经元疾病表型的额颞叶病理学:来自神经影像学的见解。

额颞叶受累已在肌萎缩侧索硬化 (ALS) 中得到广泛研究,但在其他运动神经元疾病 (MND) 表型如原发性侧索硬化 (PLS)、进行性肌萎缩 (PMA)、脊髓性肌萎缩 (SMA)、脊髓延髓肌萎缩症 (SBMA)、脊髓灰质炎后综合征 (PPS) 和遗传性痉挛性截瘫 (HSP)。本综述重点关注结构、代谢和功能神经影像学研究的见解,这些研究促进了我们对这些表型中运动外疾病负担的理解。大多数这些疾病的影像学文献有限,额颞叶受累主要通过神经心理学和验尸研究进行评估。现有的影像学研究表明,在 ALS 和 PLS 中可以很容易地检测到额颞叶变性,在 PMA、SBMA 和 HSP 中可以捕捉到不同程度的额颞叶病变,SMA 表现出脑部受累而没有区域偏好,并且 PPS 脑部变化的证据有限. 我们的审查证实了整个 MND 范围内的运动外病理的异质性,并强调了神经影像学在表征体内疾病负担的解剖模式中的作用。尽管神经影像学对 MND 研究有贡献,但需要仔细考虑样本量限制、纳入偏差、纵向研究中的流失率和方法学限制。额颞叶受累是 MND 的典型临床方面,对筛查实践具有重要意义,个性化管理策略、参与临床试验、照顾者负担和资源分配。MND 中额颞叶病理学成像的学术相关性从遗传变异的识别到症状前变化的确定,再到未来流行病学研究的设计。
更新日期:2021-08-16
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