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

Research output: Contribution to journalArticle


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.


  • D. L.Marinus Oterdoom
  • Martje E. Van Egmond
  • Luisa Cassini Ascencao
  • J. Marc C. Van Dijk
  • Assel Saryyeva
  • Martijn Beudel
  • Joachim Runge
  • Tom J. de Koning
  • Mahmoud Abdallat
  • Hendriekje Eggink
  • Marina A.J. Tijssen
  • Joachim K. Krauss
External organisations
  • University Medical Center Groningen
  • Ommelander Ziekenhuis Groningen
  • Hannover Medical School
  • Isala Klinieken
Research areas and keywords


  • deep brain stimulation, DYT6, Status dystonicus
Original languageEnglish
Article number530
Pages (from-to)1-4
Number of pages4
JournalTremor and Other Hyperkinetic Movements
Publication statusPublished - 2018
Publication categoryResearch
Externally publishedYes