Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, London, UK.
Icahn School of Medicine at Mount Sinai, New York, NY, USA.
J Huntingtons Dis. 2024;13(1):91-101. doi: 10.3233/JHD-231508.
Perivascular spaces (PVS) are fluid-filled cavities surrounding small cerebral blood vessels. There are limited reports of enlarged PVS across the grey matter in manifest Huntington's disease (HD). Little is known about how PVS morphometry in the white matter may contribute to HD. Enlarged PVS have the potential to both contribute to HD pathology and affect the distribution and success of intraparenchymal and intrathecally administered huntingtin-lowering therapies.
To investigate PVS morphometry in the global white matter across the spectrum of HD. Relationships between PVS morphometry and disease burden and severity measures were examined.
White matter PVS were segmented on 3T T2 W MRI brain scans of 33 healthy controls, 30 premanifest HD (pre-HD), and 32 early manifest HD (early-HD) participants from the Vancouver site of the TRACK-HD study. PVS count and total PVS volume were measured.
PVS total count slightly increased in pre-HD (p = 0.004), and early-HD groups (p = 0.005), compared to healthy controls. PVS volume, as a percentage of white matter volume, increased subtly in pre-HD compared to healthy controls (p = 0.044), but not in early-HD. No associations between PVS measures and HD disease burden or severity were found.
This study reveals relatively preserved PVS morphometry across the global white matter of pre-HD and early-HD. Subtle morphometric abnormalities are implied but require confirmation in a larger cohort. However, in conjunction with previous publications, further investigation of PVS in HD and its potential impact on future treatments, with a focus on subcortical grey matter, is warranted.
血管周围间隙(PVS)是围绕小脑血管的充满液体的腔隙。在明显亨廷顿病(HD)中,跨灰质存在广泛的 PVS 扩大的有限报道。关于白质中 PVS 形态测量如何有助于 HD 知之甚少。PVS 扩大有可能既有助于 HD 病理学,又影响脑室内和脑室内给予亨廷顿降低治疗的分布和成功。
研究整个 HD 谱中白质的 PVS 形态测量。检查了 PVS 形态测量与疾病负担和严重程度测量之间的关系。
在温哥华 TRACK-HD 研究的 33 名健康对照者、30 名前亨廷顿病(pre-HD)和 32 名早期亨廷顿病(early-HD)参与者的 3T T2W MRI 脑扫描上对 PVS 进行分割。测量 PVS 计数和总 PVS 体积。
与健康对照组相比,pre-HD(p=0.004)和 early-HD 组(p=0.005)的 PVS 总计数略有增加。与健康对照组相比,pre-HD 组 PVS 体积(占白质体积的百分比)略有增加(p=0.044),但在 early-HD 组中没有增加。未发现 PVS 测量值与 HD 疾病负担或严重程度之间存在相关性。
本研究揭示了 pre-HD 和 early-HD 整个白质的相对保留的 PVS 形态测量。暗示存在细微的形态异常,但需要在更大的队列中进行确认。然而,结合以前的出版物,需要进一步研究 HD 中的 PVS 及其对未来治疗的潜在影响,重点是皮质下灰质。