Valálik István, Jobbágy Akos, Bognár László, Csókay András
Department of Neurosurgery, St. John's Hospital, Budapest, Hungary.
Stereotact Funct Neurosurg. 2011;89(3):157-61. doi: 10.1159/000323341. Epub 2011 Apr 13.
We report the case of a 64-year-old woman with bilateral manifestation of Meige syndrome (MS) successfully treated with left-side unilateral ventroposterolateral pallidotomy.
Symptoms were evaluated according to the Burke-Fahn-Marsden dystonia rating scale. Head tremor, blepharospasm and orofacial dyskinesia were measured with an infrared, video-based, computerized, real-time passive marker-based analyzer of motions (RTPAM).
The Burke-Fahn-Marsden score showed a 90.2% reduction (from 25.5 to 2.5) at 6 months, and an 88.2% long-lasting benefit (to 3.0) at the 3-year follow-up with good bilateral control of the blepharospasm and orofacial movements. The RTPAM showed a substantial regression of acceleration for all markers, and abolishment of the 4.8-Hz head tremor. The correlation between symmetrical markers, and between markers within the right and left sides, was significantly decreased.
Pallidotomy with staged procedure is recommended for the treatment of MS in patients on whom deep brain stimulation could not be performed. In case of good bilateral benefits from the unilateral procedure, contralateral surgery is not needed. The RTPAM is a useful tool for the mapping of facial involuntary movements.
我们报告了一例64岁双侧梅杰综合征(MS)患者,经左侧单侧腹后外侧苍白球切开术成功治疗。
根据伯克-法恩-马斯登肌张力障碍评定量表评估症状。使用基于视频的红外计算机化实时被动标记运动分析仪(RTPAM)测量头部震颤、眼睑痉挛和口面部运动障碍。
伯克-法恩-马斯登评分在6个月时降低了90.2%(从25.5降至2.5),在3年随访时长期获益率为88.2%(降至3.0),双侧眼睑痉挛和口面部运动得到良好控制。RTPAM显示所有标记物的加速度大幅下降,4.8赫兹的头部震颤消失。对称标记物之间以及左右两侧标记物之间的相关性显著降低。
对于无法进行脑深部刺激的MS患者,建议采用分期苍白球切开术治疗。如果单侧手术能带来良好的双侧获益,则无需进行对侧手术。RTPAM是绘制面部不自主运动的有用工具。