Department of Neurosurgery, Postgraduate Medical Center, Marymoncka 99/103 Street, 01-813 Warsaw, Poland.
Chair of Rehabilitation, Department of Rehabilitation, Academy of Physical Education in Warsaw, Marymoncka 34 Street, 00-968 Warsaw, Poland.
J Neurol Sci. 2017 Dec 15;383:153-157. doi: 10.1016/j.jns.2017.10.017. Epub 2017 Oct 12.
The aim of the present study was to report the short-term as well long-term results of bilateral pallidal stimulation in 6 consecutive patients for severe debilitating craniocervical dystonia (Meige syndrome) using Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS).
We evaluated 6 consecutive patients with the diagnosis of intractable long-standing craniocervical dystonia. The formal objective assessment included the motor and disability BFMDRS scores. The BFMDRS assessment was performed before and after it roughly annually up to 60months when bilateral pallidal stimulation was switched on and compared to baseline BFMDRS scores. We present short-term (3months postoperatively) follow-up as well long-term (from 6 to 60months) results. Baseline BFMDRS scores and subsequent follow-up BFMDRS scores were compared with the use of a Wilcoxon signed-rank test for matched pairs. A two-tailed probability level of 5% (p<0.05) was considered significant.
Bilateral GPi DBS improved the BFMDRS total movement score by 65% at short-term follow-up and by 53% at long-term follow-up when compared to baseline scores. Subscores for eyes at short-term follow-up were reduced by 78%, for mouth by 58%, and for speech/swallowing by 49%. This improvements for individual subscores were maintained at long-term follow-up and were as follows for eyes by 67%, mouth by 50% and speech/swallowing by 39%. The BFMDRS disability score was reduced by 48% at short-term follow-up and by 55% at long-term follow-up when compared to baseline scores.
Our results showed that bilateral GPi DBS in craniocervical dystonia is effective and safe. Phasic dystonic movements like blepharospasm or oromandibular dystonia responded very fast and favorable to pallidal stimulation reducing disabilities.
本研究旨在报告 6 例严重致残性颅颈肌张力障碍（Meige 综合征）患者双侧苍白球刺激的短期和长期结果，采用 Burke-Fahn-Marsden 肌张力障碍评定量表（BFMDRS）。
我们评估了 6 例诊断为难治性长期颅颈肌张力障碍的连续患者。正式的客观评估包括运动和残疾 BFMDRS 评分。BFMDRS 评估在双侧苍白球刺激开启前后进行，大致每年一次，持续 60 个月，并与基线 BFMDRS 评分进行比较。我们报告了短期（术后 3 个月）随访和长期（6 至 60 个月）结果。使用配对符号秩检验比较基线 BFMDRS 评分和随后的随访 BFMDRS 评分。双侧 GPi DBS 可使 BFMDRS 总运动评分在短期随访时提高 65%，在长期随访时提高 53%，与基线评分相比。短期随访时，眼的亚评分降低了 78%，口的亚评分降低了 58%，言语/吞咽的亚评分降低了 49%。这些个别亚评分的改善在长期随访时得以维持，分别为眼的亚评分 67%、口的亚评分 50%和言语/吞咽的亚评分 39%。BFMDRS 残疾评分在短期随访时降低了 48%，在长期随访时降低了 55%，与基线评分相比。
我们的结果表明，双侧苍白球刺激在颅颈肌张力障碍中是有效和安全的。阵挛性运动障碍，如眼睑痉挛或口下颌肌张力障碍，对苍白球刺激反应迅速且有利，可降低残疾程度。
