Ghanchi Hammad, Bernstein Jacob E, Taka Taha M, Patchana Tye, Kashyap Samir, Hariri Omid R, Jamshidi Ali O, Ananda Ajay K
Neurosurgery, Riverside University Health System Medical Center, Moreno Valley, USA.
Neurosurgery, University of California Riverside, Riverside, USA.
Cureus. 2020 Oct 16;12(10):e10992. doi: 10.7759/cureus.10992.
Introduction Dystonia can cause severe disability when left untreated. Once a patient has exhausted medical management, surgical intervention may be the only treatment option. Although not curative, deep brain stimulation has been shown to be beneficial for patients affected by this condition. Our study sought to review patients undergoing deep brain stimulation for medically refractory dystonia to assess outcomes. Methods Our institution's operative database was reviewed retrospectively for all patients undergoing deep brain stimulator placement over the last six years. These medical records were reviewed for the severity of dystonia preoperatively and followed postoperatively for 24 months, focusing on the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). Patients with less than two-year postoperative follow-up were excluded from the study. The patients were further stratified by age into Group A, consisting of patients less than 40 years old, and Group B, patients greater than or equal to 40 years old. Other attributes such as age, sex, age of disease onset, disease duration at the time of surgery, genetic tests for dystonia-related genes, and any complication associated with surgery were also reviewed. Results Four hundred fifty-five operative cases for deep brain stimulator placement were reviewed, and 16 patients met inclusion criteria for the study. The mean age for our patient cohort was 43.75 years, with four males and 12 females. The average time from the age of disease onset to time of surgery was 9.7 years for Group A and 10.8 years for Group B; the overall average was 10.3 years. All patients had globus pallidus interna (GPi) as their surgical target. The first incidence of a statistically significant decrease in BFMDRS score was noted at three months postoperatively (p<0.001) when compared to preoperative values. Fourteen patients in our cohort underwent preoperative genetic testing for DYT gene mutations, out of which four were found to have a mutation. Conclusion Our review of outcomes for primary generalized dystonia at our institution found that deep brain stimulator targeting the GPi is safe and effective. We found an overall 88% response rate with younger patients (< 40-year-old) showing a better response at two years than older patients.
引言
肌张力障碍若不治疗会导致严重残疾。一旦患者用尽药物治疗手段，手术干预可能是唯一的治疗选择。虽然深部脑刺激术并非治愈性疗法，但已证明对受该病症影响的患者有益。我们的研究旨在回顾接受深部脑刺激术治疗药物难治性肌张力障碍的患者，以评估治疗效果。
方法
回顾了本机构手术数据库中过去六年内所有接受深部脑刺激器植入的患者。审查这些病历以了解术前肌张力障碍的严重程度，并在术后随访24个月，重点关注伯克 - 法恩 - 马斯登肌张力障碍评定量表（BFMDRS）。术后随访时间少于两年的患者被排除在研究之外。患者按年龄进一步分层为A组（年龄小于40岁的患者）和B组（年龄大于或等于40岁的患者）。还审查了其他属性，如年龄、性别、疾病发病年龄、手术时的疾病持续时间、肌张力障碍相关基因的基因检测以及与手术相关的任何并发症。
结果
审查了455例深部脑刺激器植入手术病例，16例患者符合研究纳入标准。我们患者队列的平均年龄为43.75岁，其中男性4例，女性12例。A组从疾病发病年龄到手术时间的平均时长为9.7年，B组为10.8年；总体平均时长为10.3年。所有患者均以内侧苍白球（GPi）为手术靶点。与术前值相比，术后三个月时首次观察到BFMDRS评分有统计学意义的显著下降（p<0.001）。我们队列中的14例患者接受了DYT基因突变的术前基因检测，其中4例被发现有突变。
结论
我们对本机构原发性全身性肌张力障碍治疗效果的回顾发现，以GPi为靶点的深部脑刺激术是安全有效的。我们发现总体有效率为88%，年轻患者（<40岁）在两年时的反应比老年患者更好。
