Background

Progressive supranuclear palsy (PSP) patients can show ventricular enlargement mimicking normal pressure hydrocephalus (NPH). The aim of this study was to distinguish PSP patients with marked ventricular dilatation (PSP-vd) from those with normal ventricular system and to evaluate the coexistence of NPH in PSP-vd patients.

Methods

One hundred three probable PSP patients, 18 definite NPH patients, and 41 control subjects were enrolled in the study. Evans index (EI) > 0.32 associated with callosal angle (CA) < 100° was used to identify PSP-vd patients. Automated ventricular volumetry (AVV) and Magnetic Resonance Hydrocephalic Index (MRHI) were performed on T1-weighted MR images to evaluate the presence of NPH in PSP-vd patients.

Results

Twelve (11.6%) out of 103 PSP patients had both abnormal EI and CA values (PSP-vd). In two of these 12 patients, AVV and MRHI values suggested PSP + NPH. In the remaining 10 PSP-vd patients, AVV and MRHI values were higher than PSP patients with normal ventricular system and controls, but lower than PSP + NPH and NPH patients, suggesting a non-hydrocephalic ventricular enlargement.

Discussion

Our study provides evidence that the combination of EI and CA biomarkers allowed to identify PSP patients with marked ventricular dilatation mimicking NPH. Only a few of these patients had PSP + NPH. Recognition of these PSP patients with enlarged ventricles can positively impact the care of this disease, helping clinicians to identify patients with PSP + NPH who could benefit from shunt procedure and avoid surgery in those with enlarged ventricles without NPH.

中文翻译：

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进行性核上性麻痹伴明显的心室扩张，类似于正常压力脑积水

背景

进行性核上性麻痹 (PSP) 患者可表现出类似正常压力脑积水 (NPH) 的心室扩大。本研究的目的是区分具有明显心室扩张 (PSP-vd) 的 PSP 患者与心室系统正常的患者，并评估 PSP-vd 患者中 NPH 的共存情况。

方法

一百三名可能的 PSP 患者、18 名确定的 NPH 患者和 41 名对照受试者参加了研究。Evans 指数 (EI) > 0.32 与胼胝体角 (CA) < 100° 相关，用于识别 PSP-vd 患者。在 T1 加权 MR 图像上进行自动心室容积测定 (AVV) 和磁共振脑积水指数 (MRHI)，以评估 PSP-vd 患者中 NPH 的存在。

结果

103 名 PSP 患者中有 12 名 (11.6%) 的 EI 和 CA 值 (PSP-vd) 均异常。在这 12 名患者中，有两名患者的 AVV 和 MRHI 值提示 PSP + NPH。在其余 10 例 PSP-vd 患者中，AVV 和 MRHI 值高于脑室系统正常的 PSP 患者和对照组，但低于 PSP + NPH 和 NPH 患者，提示非脑积水脑室扩大。

讨论

我们的研究提供了证据，表明 EI 和 CA 生物标志物的组合允许识别具有明显心室扩张模拟 NPH 的 PSP 患者。这些患者中只有少数患有 PSP + NPH。识别这些脑室扩大的 PSP 患者可以对该疾病的治疗产生积极影响，帮助临床医生识别可能从分流手术中受益的 PSP + NPH 患者，并避免在没有 NPH 的脑室扩大的患者中进行手术。