Progressive supranuclear palsy with marked ventricular dilatation mimicking normal pressure hydrocephalus
Progressive supranuclear palsy with marked ventricular dilatation mimicking normal pressure hydrocephalus
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DOI：
10.1007/s10072-021-05594-4
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发表时间：
2021-09-09
影响因子：
3.3
通讯作者：
Quattrone, Aldo
中科院分区：
文献类型：
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作者：
Quattrone, Andrea;Sarica, Alessia;Quattrone, Aldo
背景 进行性核上性麻痹 (PSP) 患者可表现出类似于正常压力脑积水 (NPH) 的脑室扩大。本研究的目的是将具有明显心室扩张（PSP-vd）的 PSP 患者与心室系统正常的患者区分开，并评估 PSP-vd 患者中 NPH 的共存情况。方法 103 名疑似 PSP 患者、18 名确诊 NPH 患者和 41 名对照受试者纳入本研究。埃文斯指数 (EI) > 0.32 且胼胝角 (CA) < 100 度用于识别 PSP-vd 患者。对 T1 加权 MR 图像进行自动心室容量 (AVV) 和磁共振脑积水指数 (MRHI) 评估 PSP-vd 患者是否存在 NPH。结果 103 例 PSP 患者中有 12 例（11.6%）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。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 我们的研究提供的证据表明，EI 和 CA 生物标志物的组合可以识别具有明显心室扩张（模仿 NPH）的 PSP 患者。这些患者中只有少数患有 PSP + NPH。识别这些心室扩大的 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从分流手术中受益的 PSP + NPH 患者，并避免对心室扩大但无 NPH 的患者进行手术。
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 degrees 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.