Department of Neurology, University Hospital "12 de Octubre", Madrid, Spain.
Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Madrid, Spain.
Ann Clin Transl Neurol. 2019 Nov;6(11):2212-2222. doi: 10.1002/acn3.50916. Epub 2019 Oct 6.
The pathogenesis of orthostatic tremor (OT) is unknown. We investigated OT-related white matter changes and their correlations with scores from a neuropsychological testing battery.
Diffusion tensor imaging measures were compared between 14 OT patients and 14 age- and education-matched healthy controls, using whole-brain tract-based spatial statistics analysis. Correlations between altered diffusion metrics and cognitive performance in OT group were assessed.
In all cognitive domains (attention, executive function, visuospatial ability, verbal memory, visual memory, and language), OT patients' cognitive performance was significantly worse than that of healthy controls. OT patients demonstrated altered diffusivity metrics not only in the posterior lobe of the cerebellum (left cerebellar lobule VI) and in its efferent cerebellar fibers (left superior cerebellar peduncle), but also in medial lemniscus bilaterally (pontine tegmentum), anterior limb of the internal capsule bilaterally, right posterior limb of the internal capsule, left anterior corona radiata, right insula, and the splenium of corpus callosum. No relationship was found between diffusion measures and disease duration in OT patients. Diffusion white matter changes, mainly those located in right anterior limb of the internal capsule, were correlated with poor performance on tests of executive function, visuospatial ability, verbal memory, and visual memory in OT patients.
White matter changes were preferentially located in the cerebellum, its efferent pathways, as well as in the pontine tegmentum and key components of the frontal-thalamic-cerebellar circuit. Further work needs to be done to understand the evolution of these white matter changes and their functional consequences.
直立性震颤(OT)的发病机制尚不清楚。我们研究了与 OT 相关的白质变化及其与神经心理学测试结果的相关性。
采用全脑基于束流的空间统计学分析方法,比较了 14 例 OT 患者和 14 例年龄和教育程度匹配的健康对照者的弥散张量成像测量值。评估 OT 组中改变的扩散指标与认知表现之间的相关性。
在所有认知领域(注意力、执行功能、视空间能力、言语记忆、视觉记忆和语言)中,OT 患者的认知表现均明显差于健康对照组。OT 患者不仅在小脑后叶(左侧小脑 VI 叶)及其传出小脑纤维(左侧上小脑脚),而且在双侧内侧丘系(脑桥被盖部)、双侧内囊前肢、右侧内囊后肢、左侧前放射冠、右侧岛叶和胼胝体压部均表现出弥散性指标改变。OT 患者的弥散指标与疾病持续时间之间没有关系。弥散白质变化主要位于右侧内囊前肢,与 OT 患者执行功能、视空间能力、言语记忆和视觉记忆测试成绩差有关。
白质变化主要位于小脑及其传出通路、脑桥被盖部以及内囊前肢和丘脑-小脑回路的关键组成部分。需要进一步研究以了解这些白质变化的演变及其功能后果。