DYT6型全身性肌张力障碍患者苍白球电极重新定位后扭转性肌张力障碍状态的逆转
Reversal of Status Dystonicus after Relocation of Pallidal Electrodes in DYT6 Generalized Dystonia.
作者信息
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, Abdallat Mahmoud, Eggink Hendriekje, Tijssen Marina A J, Krauss Joachim K
机构信息
Department of Neurosurgery, University of Groningen, University Medical Center Groningen, the Netherlands.
Department of Neurology, University of Groningen, University Medical Center Groningen, the Netherlands.
出版信息
Tremor Other Hyperkinet Mov (N Y). 2018 Feb 13;8:530. doi: 10.7916/D82F90DX. eCollection 2018.
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.
背景
DYT6肌张力障碍的临床病程可能不可预测，已知苍白球内侧部（GPi）的深部脑刺激（DBS）效果不如其他常染色体显性肌张力障碍形式显著。既往接受立体定向手术但临床获益不足的患者面临特殊挑战，因为可用的其他治疗选择非常有限。
病例报告
一名儿科DYT6患者在GPi DBS植入后最初临床反应良好，但1年后意外恶化为张力障碍状态。重新调整DBS电极位置后，张力障碍状态得以缓解。
讨论
本病例研究表明，DYT6肌张力障碍中对药物治疗耐药的张力障碍状态可通过重新定位苍白球电极得到逆转。该病例突出表明，对于处于张力障碍状态的患者，尤其是电极位置不理想的患者，即使在对DBS有初始临床反应后，也可考虑重新定位DBS电极。
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