Department of Neurology and Neurological Science, Graduate School of Medical and Dental Science, Tokyo Medical and Dental University, Bunkyo-Ku, Tokyo, Japan.
Laboratory of Clinical Cognitive Neuroscience, Institute of Liberal Arts and Science, Kanazawa University, Kanazawa, Ishikawa, Japan.
J Neurol. 2024 Jul;271(7):4473-4484. doi: 10.1007/s00415-024-12391-4. Epub 2024 May 3.
Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disease, and sometimes shows idiopathic normal pressure hydrocephalus (iNPH)-like presentations. We aimed to evaluate spinal tap responsiveness in patients with PSP, including the effect of sham spinal tap.
Eleven patients with PSP, ten with probable/definite iNPH, and eight control patients were prospectively enrolled. All participants underwent sham spinal tap and spinal tap procedures. Gait was evaluated using wearable inertial sensors. We defined "tap responders" as individuals with a 10% or more improvement from baseline in any of the gait parameters (timed up-and-go test total time, stride length, and velocity during straight walking under single-task and cognitive dual-task conditions). We compared the ratio of responders in patients with PSP to patients with iNPH and controls.
The ratio of tap responders and the ratio of sham tap responders in patients with PSP were significantly higher than those in control patients, and not different from those in patients with iNPH. PSP patients with iNPH-like MRI features tended to respond to the spinal tap compared to those without such imaging features. Notably, one patient with PSP, who responded to the spinal tap beyond the effect of sham spinal tap, was treated by the shunt operation.
This is the first prospective study to demonstrate tap and shunt responsiveness in patients with PSP while highlighting the placebo effects of the spinal tap in patients with PSP or iNPH. Our findings suggest that some PSP patients have impaired cerebrospinal fluid circulation, contributing to a distinct component of the clinical spectrum.
进行性核上性麻痹（PSP）是一种进行性神经退行性疾病，有时表现为特发性正常压力脑积水（iNPH）样表现。我们旨在评估 PSP 患者的腰椎穿刺反应性，包括假腰椎穿刺的效果。
前瞻性纳入 11 例 PSP 患者、10 例可能/确诊 iNPH 患者和 8 例对照患者。所有参与者均接受了假腰椎穿刺和腰椎穿刺程序。使用可穿戴惯性传感器评估步态。我们将“腰椎穿刺反应者”定义为任何步态参数（计时起立行走测试总时间、步长和直走时在单任务和认知双重任务条件下的速度）基线提高 10%或以上的个体。我们比较了 PSP 患者与 iNPH 患者和对照组的反应者比例。
PSP 患者的腰椎穿刺反应者比例和假腰椎穿刺反应者比例明显高于对照组，与 iNPH 患者无差异。具有 iNPH 样 MRI 特征的 PSP 患者与不具有此类影像学特征的患者相比，更倾向于对腰椎穿刺产生反应。值得注意的是，1 例 PSP 患者对腰椎穿刺的反应超出了假腰椎穿刺的效果，接受了分流手术治疗。
这是第一项前瞻性研究，证明了 PSP 患者的腰椎穿刺和分流反应性，同时强调了 PSP 或 iNPH 患者腰椎穿刺的安慰剂效应。我们的研究结果表明，一些 PSP 患者的脑脊液循环受损，这是其临床谱的一个独特组成部分。
