Department of Neurology, Zhongshan Hospital, Fudan University, Shanghai, China.
Department of Neurosugery, Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
CNS Neurosci Ther. 2020 Dec;26(12):1230-1240. doi: 10.1111/cns.13526. Epub 2020 Nov 26.
Idiopathic normal pressure hydrocephalus (iNPH), the most common type of adult-onset hydrocephalus, is a potentially reversible neuropsychiatric entity characterized by dilated ventricles, cognitive deficit, gait apraxia, and urinary incontinence. Despite its relatively typical imaging features and clinical symptoms, the pathogenesis and pathophysiology of iNPH remain unclear. In this review, we summarize current pathogenetic conceptions of iNPH and its pathophysiological features that lead to neurological deficits. The common consensus is that ventriculomegaly resulting from cerebrospinal fluid (CSF) dynamics could initiate a vicious cycle of neurological damages in iNPH. Pathophysiological factors including hypoperfusion, glymphatic impairment, disturbance of metabolism, astrogliosis, neuroinflammation, and blood-brain barrier disruption jointly cause white matter and gray matter lesions, and eventually lead to various iNPH symptoms. Also, we review the current treatment options and discuss the prospective treatment strategies for iNPH. CSF diversion with ventriculoperitoneal or lumboperitonealshunts remains as the standard therapy, while its complications prompt attempts to refine shunt insertion and develop new therapeutic procedures. Recent progress on advanced biomaterials and improved understanding of pathogenesis offers new avenues to treat iNPH.
特发性正常压力脑积水（iNPH）是最常见的成人脑积水类型，是一种潜在可逆转的神经精神疾病实体，其特征为脑室扩张、认知障碍、步态失用和尿失禁。尽管其具有相对典型的影像学特征和临床症状，但 iNPH 的发病机制和病理生理学仍不清楚。在这篇综述中，我们总结了目前关于 iNPH 的发病机制概念及其导致神经功能缺损的病理生理学特征。目前的共识是，脑脊液（CSF）动力学引起的脑室扩大可能引发 iNPH 中的神经损伤恶性循环。包括低灌注、神经淋巴功能障碍、代谢紊乱、星形胶质细胞增生、神经炎症和血脑屏障破坏等病理生理因素共同导致白质和灰质损伤，最终导致各种 iNPH 症状。我们还回顾了目前的治疗选择，并讨论了 iNPH 的潜在治疗策略。CSF 分流术（脑室-腹腔或腰-腹腔分流术）仍然是标准治疗方法，但其并发症促使人们尝试改进分流术的插入，并开发新的治疗程序。最近在先进生物材料方面的进展和对发病机制的深入了解为治疗 iNPH 提供了新的途径。
