Objective: Our study aims to examine structural alterations in brain grey and white matter of patients with essential tremor (ET) plus dystonic soft signs compared to ET patients without soft signs, patients with tremulous cervical dystonia (DT) and age- and gender-matched healthy controls (HC).

Background: Questionable signs of dystonia are a common finding in patients with ET and from a clinical point of view ET plus represents a borderline entity between ET and dystonic tremor. It has not yet been examined whether patients with ET plus dystonic soft signs show brain structural alterations compared to patients with ET, DT and HC.

Method: 42 patients with an essential, 18 patients with a dystonic and 18 patients with a borderline tremor syndrome as well as 42 HC were included into this study. Tremor severity was rated according to the Essential Tremor Rating Assessment Scale (TETRAS). Dystonic soft signs were assessed by two independent raters using a “standardized tremor elements assessment” (STEA). The MRI protocol included a T1 MPRAGE and a diffusion weighted sequence. Grey matter alterations were analysed by voxel-based morphometry and fractional anisotropy (FA) and mean diffusivity (MD) were analyzed voxel-wise as markers of white matter integrity

Results: VBM showed a significant increase of grey matter in the right putamen in ET plus and DT patients compared to HC. Further, an increase of cortical grey matter including Brodmann areas 8 and 9 in ET plus was shown. Compared to ET, bilateral putamen, pallidum and thalamus were significantly increased in ET plus. Whole brain voxel-wise analysis of FA and MD values demonstrated alterations in the basal ganglia for ET plus and the cerebellum for DT in comparison to healthy controls, thereby, not reaching significance.

Conclusion: Patients with ET plus dystonic soft signs show grey matter brain structural alterations compared to ET and HC in regions that are abnormal in DT patients as well. These findings suggest a pathophysiological involvement of thebasal ganglia cortical loop not only in DT, but also in ET plus

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MDS Abstracts - https://www.mdsabstracts.org/abstract/grey-and-white-matter-brain-structural-alterations-in-patients-with-essential-tremor-plus-dystonic-soft-signs/