School of Medicine, Cheeloo College of Medicine, Shandong University, Jinan, China.
Research Institute of Neuromuscular and Neurodegenerative Disease, Department of Neurology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, West Wenhua street No.107, Jinan, 250012, China.
J Neurol. 2022 Jun;269(6):2980-2988. doi: 10.1007/s00415-021-10900-3. Epub 2021 Nov 15.
To investigate atrophy patterns in hypothalamic subunits at different stages of ALS and examine correlations between hypothalamic subunit volume and clinical information.
We used the King's clinical staging system to divide 91 consecutive ALS patients into the different disease stages. We investigated patterns of hypothalamic atrophy using a recently published automated segmentation method in ALS patients and in 97 healthy controls. We recorded all subjects' demographic and clinical information.
Compared with healthy controls, we found significant atrophy in the bilateral anterior-superior subunit and the superior tubular subunit, as well as a reduction in global hypothalamic volume in ALS patients. When we used the King's clinical staging system to divide patients into the different disease stages, we found neither global nor specific subunit atrophy until King's stage 3 in the hypothalamus. Moreover, specific subunit volumes were significantly associated with body mass index.
In a relatively large sample of Chinese patients with ALS, using a recently published automated segmentation method for the hypothalamus, we found the pattern of hypothalamic atrophy in ALS patients differed greatly across King's clinical disease stages. Moreover, specific hypothalamic subunit atrophy may play an important role in energy metabolism in ALS patients. Thus, our findings suggest that hypothalamic atrophy may have potential phenotypic associations, and improved energy metabolism may become an important component of individualised therapy for ALS.
研究不同阶段肌萎缩侧索硬化(ALS)患者下丘脑亚区的萎缩模式,并探讨下丘脑亚区体积与临床信息之间的相关性。
我们使用 King 临床分期系统将 91 例连续 ALS 患者分为不同的疾病阶段。我们使用最近发表的一种自动分割方法对 ALS 患者和 97 例健康对照者的下丘脑萎缩模式进行了研究。我们记录了所有受试者的人口统计学和临床信息。
与健康对照组相比,我们发现 ALS 患者双侧前上亚区和上管亚区明显萎缩,以及整个下丘脑体积减少。当我们使用 King 临床分期系统将患者分为不同的疾病阶段时,直到 King 3 期我们才在脑下垂体中发现整体或特定亚区萎缩。此外,特定亚区体积与体重指数显著相关。
在一项相对较大的中国 ALS 患者样本中,使用最近发表的自动分割方法对下丘脑进行研究,我们发现 ALS 患者的下丘脑萎缩模式在 King 临床疾病阶段之间存在显著差异。此外,特定的下丘脑亚区萎缩可能在 ALS 患者的能量代谢中发挥重要作用。因此,我们的研究结果表明,下丘脑萎缩可能具有潜在的表型关联,改善能量代谢可能成为 ALS 个体化治疗的一个重要组成部分。