Movement Disorders (revue)

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Neurosurgical treatment of tremor in mitochondrial encephalopathy

Identifieur interne : 003102 ( Istex/Corpus ); précédent : 003101; suivant : 003103

Neurosurgical treatment of tremor in mitochondrial encephalopathy

Auteurs : Norbert Kovacs ; Endre Pal ; Istvan Balas ; Jozsef Janszky ; Ferenc Nagy ; Hajnalka Merkli

Source :

RBID : ISTEX:C797028743EB2151E9AFC862F6C4C458A5B0070B

English descriptors

Abstract

A 53‐year‐old woman underwent several ischemic stroke‐like episodes and later developed incomplete, bilateral ophthalmoplegia, left vision deterioration, and bilateral tremor. The clinical course, laboratory data, and muscle histology led to a diagnosis of mitochondrial encephalomyopathy. No other etiology could be identified in the background of her disabling bilateral postural–kinetic tremor. As this tremor did not respond to pharmacological therapy, left thalamotomy and subsequently right thalamic deep brain stimulator (DBS) implantation were performed, which resulted in an excellent clinical outcome. The Fahn–Tolosa–Marin Tremor Rating Scale improved from 110 to 11 points. This case suggests that the rare tremor caused by mitochondrial encephalopathy may be treated long‐term with either thalamotomy or thalamic DBS implantation. © 2006 Movement Disorder Society

Url:
DOI: 10.1002/mds.21128

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ISTEX:C797028743EB2151E9AFC862F6C4C458A5B0070B

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<caption> Segment 1 . Before the left thalamotomy. Segment 2 . Two days before right thalamic deep brain stimulator implantation. Segment 3 .Three years after the implantation, deep brain stimulation turned off. Segment 4 .Three years after the implantation, deep brain stimulation turned on. </caption>
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<p>A 53‐year‐old woman underwent several ischemic stroke‐like episodes and later developed incomplete, bilateral ophthalmoplegia, left vision deterioration, and bilateral tremor. The clinical course, laboratory data, and muscle histology led to a diagnosis of mitochondrial encephalomyopathy. No other etiology could be identified in the background of her disabling bilateral postural–kinetic tremor. As this tremor did not respond to pharmacological therapy, left thalamotomy and subsequently right thalamic deep brain stimulator (DBS) implantation were performed, which resulted in an excellent clinical outcome. The Fahn–Tolosa–Marin Tremor Rating Scale improved from 110 to 11 points. This case suggests that the rare tremor caused by mitochondrial encephalopathy may be treated long‐term with either thalamotomy or thalamic DBS implantation. © 2006 Movement Disorder Society</p>
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<affiliation>Department of Neurology, University of Pecs, Pecs, Hungary</affiliation>
<description>Correspondence: Department of Neurology, University of Pecs, H‐7623, Pecs, Ret utca 2, Hungary</description>
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<abstract lang="en">A 53‐year‐old woman underwent several ischemic stroke‐like episodes and later developed incomplete, bilateral ophthalmoplegia, left vision deterioration, and bilateral tremor. The clinical course, laboratory data, and muscle histology led to a diagnosis of mitochondrial encephalomyopathy. No other etiology could be identified in the background of her disabling bilateral postural–kinetic tremor. As this tremor did not respond to pharmacological therapy, left thalamotomy and subsequently right thalamic deep brain stimulator (DBS) implantation were performed, which resulted in an excellent clinical outcome. The Fahn–Tolosa–Marin Tremor Rating Scale improved from 110 to 11 points. This case suggests that the rare tremor caused by mitochondrial encephalopathy may be treated long‐term with either thalamotomy or thalamic DBS implantation. © 2006 Movement Disorder Society</abstract>
<note type="funding">OTKA - No. T043005; </note>
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<topic>mitochondrial encephalopathy</topic>
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<note type="content"> This article includes Supplementary Video, available online at http://www.interscience.wiley.com/jpages/0885‐3185/suppmatSupporting Info Item: Segment 1 . Before the left thalamotomy. Segment 2 . Two days before right thalamic deep brain stimulator implantation. Segment 3 .Three years after the implantation, deep brain stimulation turned off. Segment 4 .Three years after the implantation, deep brain stimulation turned on. - </note>
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