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Reversal of status dystonicus after relocation of pallidal electrodes in DYT6 generalized dystonia

Oterdoom, D. L.Marinus ; Van Egmond, Martje E. ; Ascencao, Luisa Cassini ; Van Dijk, J. Marc C. ; Saryyeva, Assel ; Beudel, Martijn ; Runge, Joachim ; de Koning, Tom J. LU ; Abdallat, Mahmoud and Eggink, Hendriekje , et al. (2018) In Tremor and Other Hyperkinetic Movements 8.
Abstract

Background: DYT6 dystonia can have an unpredictable clinical course and the result of deep brain stimulation(DBS)of the internal part of the globus pallidus(GPi)is known to be less robust than in other forms of autosomal dominant dystonia. Patients who had previous stereotactic surgery with insufficient clinical benefit form a particular challenge with very limited other treatment options available. Case Report: A pediatric DYT6 patient unexpectedly deteriorated to status dystonicus 1 year after GPi DBS implantation with good initial clinical response. After repositioning the DBS electrodes the status dystonicus resolved. Discussion: This case study demonstrates that medication-resistant status dystonicus in DYT6 dystonia can be... (More)

Background: DYT6 dystonia can have an unpredictable clinical course and the result of deep brain stimulation(DBS)of the internal part of the globus pallidus(GPi)is known to be less robust than in other forms of autosomal dominant dystonia. Patients who had previous stereotactic surgery with insufficient clinical benefit form a particular challenge with very limited other treatment options available. Case Report: A pediatric DYT6 patient unexpectedly deteriorated to status dystonicus 1 year after GPi DBS implantation with good initial clinical response. After repositioning the DBS electrodes the status dystonicus resolved. Discussion: This case study demonstrates that medication-resistant status dystonicus in DYT6 dystonia can be reversed by relocation of pallidal electrodes. This case highlights that repositioning of DBS electrodes may be considered in patients with status dystonicus, especially when the electrode position is not optimal, even after an initial clinical response to DBS.

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type
Contribution to journal
publication status
published
keywords
deep brain stimulation, DYT6, Status dystonicus
in
Tremor and Other Hyperkinetic Movements
volume
8
publisher
Columbia University Libraries
external identifiers
  • pmid:29520331
  • scopus:85051759877
DOI
10.7916/D82F90DX
language
English
LU publication?
no
id
54191f8a-0279-42e1-addb-fcf51d863f5e
date added to LUP
2020-02-11 12:39:34
date last changed
2020-07-01 05:29:29
@article{54191f8a-0279-42e1-addb-fcf51d863f5e,
  abstract     = {<p>Background: DYT6 dystonia can have an unpredictable clinical course and the result of deep brain stimulation(DBS)of the internal part of the globus pallidus(GPi)is known to be less robust than in other forms of autosomal dominant dystonia. Patients who had previous stereotactic surgery with insufficient clinical benefit form a particular challenge with very limited other treatment options available. Case Report: A pediatric DYT6 patient unexpectedly deteriorated to status dystonicus 1 year after GPi DBS implantation with good initial clinical response. After repositioning the DBS electrodes the status dystonicus resolved. Discussion: This case study demonstrates that medication-resistant status dystonicus in DYT6 dystonia can be reversed by relocation of pallidal electrodes. This case highlights that repositioning of DBS electrodes may be considered in patients with status dystonicus, especially when the electrode position is not optimal, even after an initial clinical response to DBS.</p>},
  author       = {Oterdoom, D. L.Marinus and Van Egmond, Martje E. and Ascencao, Luisa Cassini and Van Dijk, J. Marc C. and Saryyeva, Assel and Beudel, Martijn and Runge, Joachim and de Koning, Tom J. and Abdallat, Mahmoud and Eggink, Hendriekje and Tijssen, Marina A.J. and Krauss, Joachim K.},
  language     = {eng},
  month        = {01},
  publisher    = {Columbia University Libraries},
  series       = {Tremor and Other Hyperkinetic Movements},
  title        = {Reversal of status dystonicus after relocation of pallidal electrodes in DYT6 generalized dystonia},
  url          = {http://dx.doi.org/10.7916/D82F90DX},
  doi          = {10.7916/D82F90DX},
  volume       = {8},
  year         = {2018},
}