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[鉴别特发性正常压力脑积水与进行性核上性麻痹的挑战]

[Challenges to Distinguish Idiopathic Normal Pressure Hydrocephalus from Progressive Supranuclear Palsy].

作者信息

Ohara Masahiro

机构信息

Department of Neurology and Neurological Science, Graduate School of Medical and Dental Science, Tokyo Medical and Dental University.

出版信息

Brain Nerve. 2024 Feb;76(2):127-134. doi: 10.11477/mf.1416202574.

Abstract

Idiopathic normal pressure hydrocephalus (iNPH) is a clinical condition characterized by symptoms of gait disturbance, cognitive dysfunction, and urinary disturbance. In contrast, progressive supranuclear palsy (PSP) is a progressive neurodegenerative disease characterized by supranuclear gaze palsy, akinetic rigidity, gait disturbance, and dementia. PSP manifests various clinical phenotypes that mimic other diseases and occasionally present iNPH-like presentations. Our previous publication showed that PSP develops iNPH-like magnetic resonance imaging (MRI) features more frequently than other neurodegenerative diseases. It is thus sometimes challenging to distinguish iNPH from PSP. Recently, we showed that patients with PSP, particularly those with iNPH-like MRI findings, often demonstrate amelioration of their gait disturbance following a spinal tap or shunt operation. Moreover, our study revealed that both patients with iNPH and PSP often manifest a placebo effect that can be evaluated by implementing a sham spinal tap. Therefore, although a positive response to a spinal tap has been thought of as a distinct feature of iNPH, it may not be useful in differentiating iNPH and PSP. However, in clinical practice, comparing the response to a spinal tap with that of a sham spinal tap may help accurately specify patients with iNPH or PSP who definitively respond to the shunt operation.

摘要

特发性正常压力脑积水(iNPH)是一种临床病症,其特征为步态障碍、认知功能障碍和排尿障碍症状。相比之下,进行性核上性麻痹(PSP)是一种进行性神经退行性疾病,其特征为核上性凝视麻痹、运动不能性强直、步态障碍和痴呆。PSP表现出各种模仿其他疾病的临床表型,偶尔会出现类似iNPH的表现。我们之前的出版物表明,PSP比其他神经退行性疾病更频繁地出现类似iNPH的磁共振成像(MRI)特征。因此,有时将iNPH与PSP区分开来具有挑战性。最近,我们表明,PSP患者,尤其是那些具有类似iNPH的MRI表现的患者,在进行腰椎穿刺或分流手术后,其步态障碍往往会有所改善。此外,我们的研究表明,iNPH和PSP患者通常都表现出安慰剂效应,这可以通过实施假腰椎穿刺来评估。因此,尽管对腰椎穿刺的阳性反应一直被认为是iNPH的一个独特特征,但它可能无助于区分iNPH和PSP。然而,在临床实践中,将对腰椎穿刺的反应与假腰椎穿刺的反应进行比较,可能有助于准确确定对分流手术有明确反应的iNPH或PSP患者。

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