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Published in: Journal of Neurology 11/2018

01-11-2018 | Review

Deep brain stimulation in uncommon tremor disorders: indications, targets, and programming

Authors: Carlo Alberto Artusi, Ashar Farooqi, Alberto Romagnolo, Luca Marsili, Roberta Balestrino, Leonard L. Sokol, Lily L. Wang, Maurizio Zibetti, Andrew P. Duker, George T. Mandybur, Leonardo Lopiano, Aristide Merola

Published in: Journal of Neurology | Issue 11/2018

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Abstract

Background

In uncommon tremor disorders, clinical efficacy and optimal anatomical targets for deep brain stimulation (DBS) remain inadequately studied and insufficiently quantified.

Methods

We performed a systematic review of PubMed.gov and ClinicalTrials.gov. Relevant articles were identified using the following keywords: “tremor”, “Holmes tremor”, “orthostatic tremor”, “multiple sclerosis”, “multiple sclerosis tremor”, “neuropathy”, “neuropathic tremor”, “fragile X-associated tremor/ataxia syndrome”, and “fragile X.”

Results

We identified a total of 263 cases treated with DBS for uncommon tremor disorders. Of these, 44 had Holmes tremor (HT), 18 orthostatic tremor (OT), 177 multiple sclerosis (MS)-associated tremor, 14 neuropathy-associated tremor, and 10 fragile X-associated tremor/ataxia syndrome (FXTAS). DBS resulted in favorable, albeit partial, clinical improvements in HT cases receiving Vim-DBS alone or in combination with additional targets. A sustained improvement was reported in OT cases treated with bilateral Vim-DBS, while the two cases treated with unilateral Vim-DBS demonstrated only a transient effect. MS-associated tremor responded to dual-target Vim-/VO-DBS, but the inability to account for the progression of MS-associated disability impeded the assessment of its long-term clinical efficacy. Neuropathy-associated tremor substantially improved with Vim-DBS. In FXTAS patients, while Vim-DBS was effective in improving tremor, equivocal results were observed in those with ataxia.

Conclusions

DBS of select targets may represent an effective therapeutic strategy for uncommon tremor disorders, although the level of evidence is currently in its incipient form and based on single cases or limited case series. An international registry is, therefore, warranted to clarify selection criteria, long-term results, and optimal surgical targets.
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Metadata
Title
Deep brain stimulation in uncommon tremor disorders: indications, targets, and programming
Authors
Carlo Alberto Artusi
Ashar Farooqi
Alberto Romagnolo
Luca Marsili
Roberta Balestrino
Leonard L. Sokol
Lily L. Wang
Maurizio Zibetti
Andrew P. Duker
George T. Mandybur
Leonardo Lopiano
Aristide Merola
Publication date
01-11-2018
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 11/2018
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-018-8823-x

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