Abstract

Introduction: Essential tremor (ET) is a tremor syndrome characterized by bilateral, upper limb action tremor. Essential tremor-plus (ET-plus) describes ET patients with additional neurologic signs. It is unknown whether there is a difference in response to treatment with ventralis intermedius nucleus deep brain stimulation (VIM DBS) in patients with ET and ET-plus. Due to potential variability in underlying etiology in ET-plus, there is a concern that ET-plus patients may have worse outcomes. The aim of this study was to identify whether patients with ET-plus have worse tremor outcomes after VIM DBS than patients with ET.Methods: This is a retrospective chart and video review evaluating VIM DBS outcomes by comparing changes from baseline in the Fahn-Tolosa-Marin Tremor Rating Scale Part B (FTM-B) for the treated limb between patients with ET and ET-plus at follow-up examinations. Patients were re-classified as having ET or ET-plus using pre-operative examination videos by two independent movement disorders neurologists blinded to patient characteristics. As a secondary outcome, we evaluated for correlations and potential predictors of treatment response.Results: Twenty-six patients were included: 13 with ET, 13 with ET-plus. There were no significant differences in the change in FTM-B scores between the ET and ET-plus patients at each follow-up examination. None of the included patients developed new symptoms compatible with dystonia, parkinsonism or gait disturbances.Conclusions: Patients with ET-plus had tremor improvement from VIM DBS, with no differences when compared to those with ET, without emergence of postoperative neurological issues. Patients with ET-plus should still be considered good candidates for VIM DBS for treatment of tremor.

Highlights

  • Essential tremor (ET) is a tremor syndrome characterized by bilateral, upper limb action tremor

  • The primary objective of this study was to identify whether patients with essential tremor-plus (ET-plus) have worse long-term tremor outcomes after ventralis intermedius nucleus deep brain stimulation (VIM deep brain stimulation (DBS)) than patients with ET

  • ET was defined based on the tremor classification as “isolated tremor syndrome of bilateral upper limb action tremor, at least 3 years’ duration, with or without tremor in other locations, and the absence of other neurological signs, such as dystonia, ataxia or parkinsonism” [3]

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Summary

Introduction

Essential tremor (ET) is a tremor syndrome characterized by bilateral, upper limb action tremor. Essential tremor-plus (ET-plus) describes ET patients with additional neurologic signs. It is unknown whether there is a difference in response to treatment with ventralis intermedius nucleus deep brain stimulation (VIM DBS) in patients with ET and ET-plus. In 2018, a new classification for tremor syndromes was introduced which addressed the phenotypic heterogeneity of ET and other tremor syndromes [3] In this classification, patients with tremor consistent with ET, but with additional neurologic signs of uncertain significance such as impaired tandem gait or dystonia, are classified as essential tremor-plus (ET-plus). There exist several invasive treatments, including lesioning or stimulating the ventralis intermedius nucleus (VIM) of the thalamus, effectively reducing upper extremity tremor [8, 9]. There are some risks associated with DBS, which must be considered when determining whether a patient may benefit from it, including paresthesias, headache, gait disorder, dysarthria, stroke, and death [7]

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