Reference : Long-term outcome of deep brain stimulation in fragile X-associated tremor/ataxia syndrome

	Document type :	Scientific journals : Article
	Discipline(s) :	Human health sciences : Neurology
	To cite this reference:	http://hdl.handle.net/10993/22634

Title :	Long-term outcome of deep brain stimulation in fragile X-associated tremor/ataxia syndrome
Language :	English
Author, co-author :	Weiss, D. [> >]
	Mielke, C. [> >]
	Wachter, T. [> >]
	Bender, B. [> >]
	Liscic, R. M. [> >]
	Scholten, M. [> >]
	Naros, G. [> >]
	Plewnia, C. [> >]
	Gharabaghi, A. [> >]
	Krüger, Rejko [University of Luxembourg > Faculty of Science, Technology and Communication (FSTC) > Life Science Research Unit]
Publication date :	2015
Journal title :	Parkinsonism & related disorders
Publisher :	Elsevier
Volume :	21
Issue/season :	3
Pages :	310-313
Peer reviewed :	Yes (verified by ORBilu)
ISSN :	1353-8020
e-ISSN :	1873-5126
Keywords :	[en] Fragile X-associated tremor/ataxia ; syndrome ; Deep brain stimulation (DBS) ; Ventral intermedial thalamus (Vim) ; Gait ; Kinematic analysis
Abstract :	[en] INTRODUCTION: Fragile X-associated tremor/ataxia syndrome (FXTAS) presents as complex movement disorder including tremor and cerebellar ataxia. The efficacy and safety of deep brain stimulation of the nucleus ventralis intermedius of the thalamus in atypical tremor syndromes like FXTAS remains to be determined. METHODS: Here, we report the long-term outcome of three male genetically confirmed FXTAS patients treated with bilateral neurostimulation of the nucleus ventralis intermedius for up to four years. RESULTS: All patients demonstrated sustained improvement of both tremor and ataxia - the latter included improvement of intention tremor and axial tremor. Kinematic gait analyses further demonstrated a regularization of the gait cycle. Initial improvements of hand functional disability were not sustained and reached the preoperative level of impairment within one to two years from surgery. CONCLUSION: Our data on patients with a genetic cause of tremor show favorable outcome and may contribute to improved patient stratification for neurostimulation therapy in the future.
Research centres :	Luxembourg Centre for Systems Biomedicine (LCSB): Clinical & Experimental Neuroscience (Krüger Group)
Permalink :	http://hdl.handle.net/10993/22634
DOI :	10.1016/j.parkreldis.2014.12.015

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