Background

There are no effective symptomatic treatments for progressive supranuclear palsy (PSP). Recent studies report benefits of spinal cord stimulation (SCS) for freezing of gait (FOG) and gait disorders in Parkinson’s disease and atypical Parkinsonism patients. This is the first study to report therapeutic effects of SCS in Richardson’s syndrome PSP (PSP-RS) patients.

Methods

Epidural SCS was implanted in three female PSP-RS participants (3.2 ± 1.3 years with disease). Six programs (300–400 µs/30–130 Hz) were randomly tested at suprathreshold intensity on separate days. The setting that best improved gait/FOG was used daily by each participant in the study. Protokinetics walkway captured spatiotemporal gait measures and FOG episodes (turning on the spot and while walking) and clinical scales including FOG questionnaire, UPDRS-III (OFF-/ON-l-dopa), and participant-perceived global impression of change (GISC) were collected at pre-SCS, and 3, 6, 12 months post-SCS.

Results

Participant #1 demonstrated the highest GISC score (6.5/10) with a consistent reduction of FOGs by 43.8%, UPDRS-III score (− 5 points), and improved step length and stride velocity (33.6%) while maintaining a l-dopa response of ~ 12% over the 12 months. Participant #2, walking FOG frequency and turning duration was reduced by 39.0% (OFF-l-dopa), and ON-l-dopa UPDRS-III score worsened (+ 5 points) at 12 months. Participant #3, FOG frequency reduced by 75% up to 6 months rating a GISC 3/10 score, however disease severity worsened at 12 months. Ambulatory gait parameters universally improved by 29.6% in all participants.

Conclusion

The results support the benefit of SCS for FOG and gait symptoms in PSP-RS and suggests early SCS intervention for dopaminergic-resistant gait should be considered.

中文翻译：

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脊髓刺激治疗进行性核上性麻痹患者步态功能障碍

背景

进行性核上性麻痹 (PSP) 没有有效的对症治疗。最近的研究报告了脊髓刺激 (SCS) 对帕金森病和非典型帕金森病患者步态冻结 (FOG) 和步态障碍的益处。这是第一项报告 SCS 在理查森综合征 PSP (PSP-RS) 患者中的治疗效果的研究。

方法

硬膜外 SCS 植入了三名女性 PSP-RS 参与者（患病 3.2 ± 1.3 年）。六个程序（300–400 µs/30–130 Hz）在不同的日子以超阈值强度随机测试。研究中的每个参与者每天都使用最能改善步态/FOG 的设置。Protokinetics walkway 捕获时空步态测量和 FOG 发作（当场转动和行走时）和临床量表，包括 FOG 问卷、UPDRS-III（OFF-/ON- l - dopa）和参与者感知的全球变化印象 (GISC)在 SCS 前和 SCS 后 3、6、12 个月收集。

结果

参与者 #1 表现出最高的 GISC 得分 (6.5/10)，FOG 持续减少 43.8%，UPDRS-III 得分（- 5 分），在保持左旋多巴的同时，步长和步速得到改善（33.6 %）在 12 个月内反应约 12%。参与者 #2，步行 FOG 频率和转动持续时间减少了 39.0%（OFF - l - dopa），ON- l -dopa UPDRS-III 评分在 12 个月时恶化（+ 5 分）。参与者 #3，FOG 频率在 6 个月内减少了 75%，评分为 GISC 3/10，但疾病严重程度在 12 个月时恶化。所有参与者的步行步态参数普遍提高了 29.6%。

结论

结果支持 SCS 对 PSP-RS 中 FOG 和步态症状的益处，并建议应考虑对多巴胺能抵抗步态进行早期 SCS 干预。