Parkinson's Disease and Dystonia Research Center, Tokushima University Hospital, Tokushima 770-8503, Japan.
Parkinsonism Relat Disord. 2011 Feb;17(2):123-5. doi: 10.1016/j.parkreldis.2010.11.013. Epub 2010 Dec 13.
Primary Meige syndrome is an idiopathic movement disorder that manifests as craniofacial and often cervical dystonias. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) has emerged as a powerful surgical option in the treatment of primary generalized or segmental dystonia. However, the experience with GPi-DBS in Meige syndrome is limited. We followed 5 patients with disabling Meige syndrome treated by bilateral GPi-DBS for 49 ± 43.7 (mean ± SD) months. All patients were assessed before surgery and at the last follow-up after surgery using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) which includes both the movement and disability scales. Bilateral GPi-DBS produced a sustained and long-lasting improvement in dystonia symptoms associated with Meige syndrome. At the last follow-up, the mean scores of BFMDRS movement and disability scales improved significantly by 84 ± 6.8% (range, 75-94%) and 89 ± 8.1% (range, 80-100%), respectively. Bilateral pallidal stimulation is a beneficial therapeutic option for long-term relief of the disabling dystonia symptoms in Meige syndrome.
原发性梅杰综合征是一种特发性运动障碍，表现为颅面部和颈部的张力障碍。深部脑刺激（DBS）的苍白球 internus（GPi）已经成为治疗原发性全身性或节段性张力障碍的一种强大的手术选择。然而，在梅杰综合征中使用 GPi-DBS 的经验有限。我们随访了 5 例因致残性梅杰综合征接受双侧 GPi-DBS 治疗的患者，随访时间为 49±43.7（平均值±标准差）个月。所有患者在术前和术后最后一次随访时均采用 Burke-Fahn-Marsden 肌张力障碍评定量表（BFMDRS）进行评估，该量表包括运动和残疾量表。双侧 GPi-DBS 可显著改善与梅杰综合征相关的肌张力障碍症状，并持续且长期有效。在最后一次随访时，BFMDRS 运动和残疾量表的平均评分分别显著改善了 84±6.8%（范围为 75-94%）和 89±8.1%（范围为 80-100%）。双侧苍白球刺激是一种长期缓解梅杰综合征致残性肌张力障碍症状的有益治疗选择。
