Department of Clinical Neurological Sciences, London Health Sciences Centre, Lawson Health Research Institute, 339 Windermere Road, A10-026, London, ON, N6A 5A5, Canada.
Schulich School of Medicine and Dentistry, University of Western, 1151 Richmond Street, London, ON, N6A 3K7, Canada.
J Neurol. 2021 Mar;268(3):989-996. doi: 10.1007/s00415-020-10233-7. Epub 2020 Oct 3.
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.
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.
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.
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.
目前对于进行性核上性麻痹（PSP），尚无有效的对症治疗方法。最近的研究报告称脊髓刺激（SCS）对帕金森病和非典型帕金森病患者的冻结步态（FOG）和步态障碍有治疗作用。这是第一项关于 SCS 对 Richardson 综合征 PSP（PSP-RS）患者的治疗效果的研究。
对 3 名 PSP-RS 女性参与者（发病 3.2±1.3 年）进行硬膜外 SCS 植入。在不同日子，以超阈值强度随机测试 6 个程序（300-400µs/30-130Hz）。每位参与者在研究中每天使用最能改善步态/FOG 的设置。Protokinetics 步道采集时空步态测量和 FOG 发作（原地和行走时）以及临床量表，包括 FOG 问卷、UPDRS-III（OFF-/ON-左旋多巴）和参与者感知的整体变化印象（GISC），在 SCS 前、3、6 和 12 个月时进行收集。
参与者 #1 表现出最高的 GISC 评分（6.5/10），FOG 减少 43.8%，UPDRS-III 评分下降（-5 分），步长和步速提高（33.6%），同时在 12 个月内保持约 12%的左旋多巴反应。参与者 #2，行走 FOG 频率和转弯持续时间减少 39.0%（OFF-左旋多巴），ON-左旋多巴 UPDRS-III 评分在 12 个月时恶化（+5 分）。参与者 #3，FOG 频率在 6 个月时减少了 75%，评分 3/10，但在 12 个月时疾病严重程度恶化。所有参与者的步行步态参数普遍提高了 29.6%。
结果支持 SCS 对 PSP-RS 的 FOG 和步态症状的益处，并表明应考虑早期对多巴胺能抵抗性步态进行 SCS 干预。
