Neuroradiology Unit, University Hospital "G. Martino", Messina, Italy.
Neurosurgery Unit, University Hospital "G. Martino", Messina, Italy.
World Neurosurg. 2024 Jan;181:171-177. doi: 10.1016/j.wneu.2023.10.110. Epub 2023 Oct 26.
Idiopatic normal pressure hydrocephalus (iNPH) is a progressive neurologic syndrome featured by the triad of gait disturbance, mental deterioration and urinary incontinence, associated with ventriculomegaly and normal cerebrospinal fluid (CSF) pressure. The clinical presentation may be atypical or incomplete, or mimicked by other diseases, so conventional neuroradiologic imaging plays an important role in defining this pathology. iNPH pathophysiologic mechanisms have not yet been fully elucidated, although several studies have demonstrated the involvement of the glymphatic system, a highly organized fluid transport system, the malfunction of which is involved in the pathogenesis of several disorders including normotensive hydrocephalus.
Recent studies have shown how crucial in the diagnosis of this pathology is the definition of morphologic biomarkers, such as ventricular enlargement disproportionate to cerebral atrophy and associated ballooning of frontal horns; periventricular hyperintensities; and corpus callosum thinning and elevation, with callosal angle <90 degrees.
Another interesting feature that is becoming a well-recognized factor to look for and useful for the diagnosis of iNPH is disproportionately enlarged subarachnoid space hydrocephalus, which includes enlarged ventricles, tight high-convexity and medial surface subarachnoid spaces, and expanded Sylvian fissures. A correct choice of MRI sequences is important for a proper characterization identification of others diseases that may underlie this pathology. Magnetic resonance imaging allows us to evaluate CSF flow, enabling us to define qualitative and quantitative parameters necessary for the purpose of accurate iNPH diagnosis.
iNPH can represent a real diagnostic challenge; a proper correlation among clinical features, traditional MRI, and CSF dynamics analysis can lead to a correct diagnosis.
特发性正常压力脑积水(iNPH)是一种进行性神经系统综合征,其特征为步态障碍、智力下降和尿失禁三联征,伴有脑室扩大和正常脑脊液(CSF)压力。临床表现可能不典型或不完整,或被其他疾病模仿,因此常规神经影像学在定义这种病理方面起着重要作用。iNPH 的病理生理机制尚未完全阐明,尽管有几项研究表明糖质系统(一种高度组织化的液体转运系统)参与其中,该系统的功能障碍与包括正常压力性脑积水在内的几种疾病的发病机制有关。
最近的研究表明,在诊断这种病理时,定义形态学生物标志物(如脑室扩大与脑萎缩不成比例,伴额角球囊样扩张;脑室周围高信号;胼胝体变薄和抬高,胼胝体角<90 度)是多么关键。
另一个有趣的特征是不成比例扩大的蛛网膜下腔脑积水,这是一种越来越被认为是寻找和有助于 iNPH 诊断的因素,它包括扩大的脑室、紧高凸面和内侧蛛网膜下腔空间以及扩大的大脑外侧裂。正确选择 MRI 序列对于正确识别可能导致这种病理的其他疾病非常重要。磁共振成像(MRI)允许我们评估 CSF 流动,使我们能够定义定性和定量参数,以实现准确的 iNPH 诊断。
iNPH 可能是一个真正的诊断挑战;临床特征、传统 MRI 和 CSF 动力学分析之间的正确关联可以导致正确的诊断。