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特发性正常压力脑积水合并进行性核上性麻痹。

Idiopathic normal pressure hydrocephalus concomitant with progressive supranuclear palsy.

作者信息

Shimada Tomoyo, Sakurai Anri, Niiyama Shunichi, Kawamura Kaito, Nakajima Madoka, Okuzumi Ayami, Hatano Taku, Miyajima Masakazu, Hattori Nobutaka, Tsunemi Taiji

机构信息

Department of Neurology, Juntendo University School of Medicine, Tokyo, Japan.

Department of Neurosurgery, Juntendo University School of Medicine, Tokyo, Japan.

出版信息

Parkinsonism Relat Disord. 2025 Mar;132:107273. doi: 10.1016/j.parkreldis.2025.107273. Epub 2025 Jan 10.

Abstract

INTRODUCTION

Recent studies by us and others have unveiled a frequent coexistence of idiopathic normal pressure hydrocephalus (iNPH) with neurodegenerative movement disorders, including progressive supranuclear palsy (PSP). This study aims to explore the clinical and radiological characteristics of patients with iNPH who also had comorbid with PSP, referred to as iNPHc + PSP.

METHODS

We retrospectively analyzed patients with iNPH admitted to our department between 2009 and 2024. We adhered to the established clinical criteria for iNPH and the Movement Disorder Society (MDS)-PSP criteria for probable or possible PSP with minor modifications.

RESULTS

Among 85 iNPH patients, 18 were categorized as having iNPHc + PSP (21.2 %). Clinically, comorbid PSP led to impaired vertical eye movement, axial-dominant parkinsonism, and a propensity to fall backward in iNPH (p = 0.022, p = 0.002, and p < 0.005, respectively). Radiologically, comorbid PSP demonstrated a shortened mesencephalic tegmentum length in the magnetic resonance imaging and an asymmetrical deficit of dopamine transporter activities in the DaTscan (p = 0.0005, p = 0.0129, respectively). Lumboperitoneal shunt (LPS) surgery improved the modified Rankin scale (p = 0.038) and the iNPH grading scale (total score, p = 0.003; gait disturbance, p = 0.020; urinary incontinence, p = 0.026) one-year after surgery.

CONCLUSIONS

PSP can coexist with iNPH, and detailed clinical and radiological assessment can aid in detecting this comorbidity. Importantly, LPS surgery can improve outcomes of patients with iNPHc + PSP.

摘要

引言

我们和其他研究团队最近的研究发现,特发性正常压力脑积水(iNPH)常与神经退行性运动障碍共存,包括进行性核上性麻痹(PSP)。本研究旨在探讨合并PSP的iNPH患者(称为iNPHc + PSP)的临床和影像学特征。

方法

我们回顾性分析了2009年至2024年期间在我科住院的iNPH患者。我们遵循既定的iNPH临床标准和运动障碍协会(MDS)-PSP可能或疑似PSP的标准,并做了一些小的修改。

结果

在85例iNPH患者中,18例被归类为iNPHc + PSP(21.2%)。临床上,合并PSP导致iNPH患者垂直眼球运动障碍、轴性为主的帕金森症以及向后跌倒倾向(分别为p = 0.022、p = 0.002和p < 0.005)。影像学上,合并PSP在磁共振成像中表现为中脑被盖长度缩短,在DaTscan中表现为多巴胺转运体活性不对称降低(分别为p = 0.0005、p = 0.0129)。腰大池腹腔分流(LPS)手术在术后一年改善了改良Rankin量表评分(p = 0.038)和iNPH分级量表评分(总分,p = 0.003;步态障碍,p = 0.020;尿失禁,p = 0.026)。

结论

PSP可与iNPH共存,详细的临床和影像学评估有助于发现这种合并症。重要的是,LPS手术可改善iNPHc + PSP患者的预后。

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