TY - JOUR
T1 - Reversal of status dystonicus after relocation of pallidal electrodes in DYT6 generalized dystonia
AU - Oterdoom, D. L. Marinus
AU - van Egmond, Martje E.
AU - Ascencao, Luisa Cassini
AU - van Dijk, J. Marc C.
AU - Saryyeva, Assel
AU - Beudel, Martijn
AU - Runge, Joachim
AU - de Koning, Tom J.
AU - Abdallat, Mahmoud
AU - Eggink, Hendriekje
AU - Tijssen, Marina A. J.
AU - Krauss, Joachim K.
PY - 2018
Y1 - 2018
N2 - 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.
AB - 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.
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85069503067&origin=inward
U2 - https://doi.org/10.5334/TOHM.409
DO - https://doi.org/10.5334/TOHM.409
M3 - Article
SN - 2160-8288
VL - 8
SP - 1
EP - 4
JO - Tremor and Other Hyperkinetic Movements
JF - Tremor and Other Hyperkinetic Movements
ER -