Goh Eng Tah, Lock Christine, Tan Audrey Jia Luan, Tan Bee Ling, Liang Sai, Pillay Robin, Kumar Sumeet, Ahmad-Annuar Azlina, Narayanan Vairavan, Kwok Janell, Tan Yi Jayne, Ng Adeline Sl, Tan Eng King, Czosnyka Zofia, Czosnyka Marek, Pickard John D, Keong Nicole C
Department of Neurosurgery, National Neuroscience Institute, Singapore, Singapore.
Department of Neuroradiology, National Neuroscience Institute, Singapore, Singapore.
Front Neurol. 2022 Jul 12;13:868000. doi: 10.3389/fneur.2022.868000. eCollection 2022.
Normal pressure hydrocephalus (NPH) is a neurological condition characterized by a clinical triad of gait disturbance, cognitive impairment, and urinary incontinence in conjunction with ventriculomegaly. Other neurodegenerative diseases, such as Alzheimer's disease, Parkinson's disease, and vascular dementia share some overlapping clinical features. However, there is evidence that patients with comorbid NPH and Alzheimer's or Parkinson's disease may still exhibit good clinical response after CSF diversion. This study aims to evaluate clinical responses after ventriculo-peritoneal shunt (VPS) in a cohort of patients with coexisting NPH and neurodegenerative disease.
The study has two components; (i) a pilot study was performed that specifically focused upon patients with Complex NPH and following the inclusion of the Complex NPH subtype into consideration for the clinical NPH programme, (ii) a retrospective snapshot study was performed to confirm and characterize differences between Classic and Complex NPH patients being seen consecutively over the course of 1 year within a working subspecialist NPH clinic. We studied the characteristics of patients with Complex NPH, utilizing clinical risk stratification and multimodal biomarkers.
There was no significant difference between responders and non-responders to CSF diversion on comorbidity scales. After VPS insertion, significantly more Classic NPH patients had improved cognition compared to Complex NPH patients ( = 0.005). Improvement in gait and urinary symptoms did not differ between the groups. 26% of the Classic NPH group showed global improvement of the triad, and 42% improved in two domains. Although only 8% showed global improvement of the triad, all Complex NPH patients improved in gait.
Our study has demonstrated that the presence of neurodegenerative disorders co-existing with NPH should not be the sole barrier to the consideration of high-volume tap test or lumbar drainage a specialist NPH programme. Further characterization of distinct cohorts of NPH with differing degrees of CSF responsiveness due to overlay from neurodegenerative or comorbidity risk burden may aid toward more precise prognostication and treatment strategies. We propose a simplistic conceptual framework to describe NPH by its Classic vs. Complex subtypes to promote the clinical paradigm shift toward subspecialist geriatric neurosurgery by addressing needs for rapid screening tools at the clinical-research interface.
正常压力脑积水(NPH)是一种神经系统疾病,其特征为步态障碍、认知障碍和尿失禁三联征,同时伴有脑室扩大。其他神经退行性疾病,如阿尔茨海默病、帕金森病和血管性痴呆,具有一些重叠的临床特征。然而,有证据表明,合并NPH和阿尔茨海默病或帕金森病的患者在脑脊液分流术后仍可能表现出良好的临床反应。本研究旨在评估一组同时患有NPH和神经退行性疾病的患者在脑室-腹腔分流术(VPS)后的临床反应。
本研究有两个部分;(i)进行了一项试点研究,专门针对复杂型NPH患者,并在将复杂型NPH亚型纳入临床NPH项目的考虑范围后进行,(ii)进行了一项回顾性快照研究,以确认和描述在一个专业的NPH诊所中,在1年期间连续就诊的经典型和复杂型NPH患者之间的差异。我们利用临床风险分层和多模式生物标志物研究了复杂型NPH患者的特征。
在合并症量表上,脑脊液分流术的反应者和无反应者之间没有显著差异。VPS植入后,与复杂型NPH患者相比,经典型NPH患者认知改善的比例显著更高(=0.005)。两组之间步态和泌尿系统症状的改善情况没有差异。经典型NPH组中有26%的患者三联征整体改善,42%的患者在两个方面有所改善。虽然只有8%的患者三联征整体改善,但所有复杂型NPH患者的步态都有所改善。
我们的研究表明,与NPH并存的神经退行性疾病的存在不应成为在专业NPH项目中考虑进行大容量抽液试验或腰椎引流的唯一障碍。由于神经退行性疾病或合并症风险负担的叠加,对具有不同程度脑脊液反应性的不同NPH队列进行进一步特征化,可能有助于更精确的预后评估和治疗策略制定。我们提出了一个简单的概念框架,通过经典型与复杂型亚型来描述NPH,以通过满足临床研究界面快速筛查工具的需求,推动向专业老年神经外科的临床范式转变。