特发性正常压力脑积水的诊断与治疗

特发性正常压力脑积水是一种原因不明的脑室扩大而脑脊液压力政党的脑积水,病理生理改变复杂,目前对其诊断与治疗还存在着较多争议.临床上主要根据病人症状、影像学表现、脑脊液动力学检查结果及颅内压临测进行诊断,治疗上脑脊液分流术为该疾病最有效的方法,然而还存在手术效果不确切,对可疑患者选择病例进行分流的困难.     

特发性正常压力脑积水36例诊治体会

特发性正常压力脑积水（INPH）是指无明确诱因的正常压力脑积水。有脑室扩大但脑脊液压力正常。以步态障碍、精神障碍、尿失禁为临床特征。其临床表现隐匿,好发于老年人,又与老年病相似,容易漏诊、误诊。一旦确诊,脑脊液分流手术效果良好。目前国际上对其诊断、     

特发性正常压力脑积水的临床治疗进展

正常压力性脑积水(normal pressure hydrocephalus,NPH)是一种特殊类型的交通性脑积水,主要表现为步态障碍、认知障碍和尿失禁"三联征";影像学检查主要表现为脑室扩大,Evan’s指数>0.3,侧裂池增宽,部分病人脑室旁白质可见低密度影;脑脊液压力测定在70~200 mmH2O的一组临床综合征。NPH临床分为两类:一类是继发性NPH(secondary normal pressure hydrocephalus,sNPH),常继发于有明确发病原因的颅脑损伤、高血压性脑出血、蛛网膜下腔出血、颅内感染、脑炎等。     

特发性正常压力脑积水的诊断及治疗进展

正特发性正常压力脑积水(idiopathic normal pressure hydrocephalus,iNPH)是一种发病原因未知,以步态不稳、认知障碍和/或小便失禁为主要表现,脑脊液压力正常,影像学检查见脑室扩大的疾病,在老年人发生率较高,且伴随年龄增大患病率升高,有研究发现发病率在0.2%~5.9%~([1-3])。其病理生理机制不完全明确,给治疗带来了一定困难。本文期望通过对近年iNPH的相关研究进行综述,加深对其认识。     

特发性正常压力脑积水影像学特征

正常压力脑积水(normal pressure hydrocephalus,NPH)这一概念最早由Hakim于1964年提出,之后Adams和Hakim等又详细阐述了该疾病的特征性临床表现,即可以通过治疗而改善的"步态障碍、认知障碍、尿失禁"三联征[1].特发性正常压力脑积水(idiopathic normal pressure hydrocephalus,iNPH)是病因不明的正常颅压下脑室扩大,临床表现为步态异常和/或痴呆及括约肌功能障碍的疾病,大多发生于老年人.iNPH是极少数可以通过分流来改善痴呆症状的疾病之一,所以对于该疾病的正确诊断和有效治疗无论对于家庭还是社会都有着重要意义.然而目前临床上对于该病的诊断方法相对简单,准确性不高,且难以与其他的老年性疾病,如其他伴随脑室扩大的痴呆性疾病、颈椎或腰椎狭窄、外周神经性疾病、关节炎或前列腺肥大鉴别.幸运的是,伴随着近些年来影像学技术的迅猛发展,各国学者的对iNPH影像学特征研究得以不断深化,针对于该疾病影像学检查手段的阐述也层出不穷,并于2005年由Relkin等[2]通过循证医学方法制定出iNPH诊断标准,其目的就在于提高iNPH的诊断准确性并预测分流手术的效果.     

老年人特发性正常压力脑积水的临床研究(附35例报道)

目的 探讨脑室腹腔分流术在老年人特发性正常压力脑积水治疗中的临床疗效.方法 回顾性总结沧州市中心医院神经外科自2006年5月至2010年5月收治的35例老年人特发性正常压力脑积水患者的病史、CT和(或)MR影像资料、腰椎穿刺放液试验等临床资料,分析以上因素对脑室腹腔分流手术效果的影响. 结果 本组35例患者术后均得到6～12月随访,其中显效16例,有效12例,无效7例;智能障碍患者的术后有效率为58.0％,步态不稳患者的术后有效率为85.0％,尿失禁患者的术后有效率为81.5％.33例患者影像学复查见脑室系统均有不同程度缩小. 结论 脑室腹腔分流术是目前治疗老年人特发性正常压力脑积水的最有效方法.     

特发性正常颅压脑积水的诊断及治疗现状

特发性正常颅压脑积水（Idiopathic normal pressure hydrocephalus,INPH）系正常颅压脑积水的类型之一,病因不明,但有别于有明确病因的继发性正常颅压脑积水,如果及时得到正确的治疗,可以阻止智能的减退,并明显改善患者的生活质量.     

特发性正常压力脑积水脑脊液标志物的变化

目的 探讨特发性正常压力脑积水（iNPH）病人脑脊液（CSF）磷酸化tau蛋白（p-tau）、总tau蛋白（t-tau）、转化生长因子-β1（TGF-β1）的变化。方法 前瞻性收集2018年6月至2020年2月经分流术临床确诊的16例iNPH为确诊组,同期行脑脊液释放试验无效的16例可疑iNPH为可疑组。采用酶联免疫吸附试验检测CSF中p-tau、t-tau、TGF-β1水平。结果 确诊组CSF中p-tau水平显著低于可疑组（P<0.01）,TGF-β1水平显著高于可疑组（P<0.01）。两组CSF中t-tau水平无统计学差异（P>0.05）。8例确诊iNPH术后7 d的CSF中p-tau、t-tau水平较术前均明显降低（P<0.05）,而TGF-β1水平与术前无统计学差异（P>0.05）。结论 本文结果提示CSF中p-tau、t-tau、TGF-β1在iNPH诊断、分流术效果评估中具有一定的作用。     

重视特发性正常压力性脑积水的规范化诊治

随着社会进步和医疗水平的不断提高,人类寿命也在不断地延长。随着老龄化社会的发展,老年人相关疾病如老年痴呆症、帕金森病和特发性正常压力性脑积水(idiopathic normal pressure hydrocephalus,iNPH)日益成为医学界亟待解决的现实问题[1-2]。典型iNPH表现为步态不稳、痴呆和尿失禁     

侧脑室-腹腔分流术治疗正常压力脑积水

目的探讨正常压力脑积水(NPH)诊断和手术病例的选择方法,分析侧脑室-腹腔分流术治疗正常压力脑积水的效果。方法回顾性分析16例正常压力脑积水病人TAP试验前后步距、步速、转身步数,手术前后临床症状、KPS评分,腰穿测压和术中测压的结果。结果本组16例病人无手术死亡,无感染、脑内血肿,硬膜下积液1例。随访12个月,有效16例(100%),显效7例(43.75%)。结论正常压力脑积水的诊断和手术病例的选择需要综合分析临床资料;侧脑室-腹腔分流术能够改善病人临床症状,提高其生活质量。     

Pathogenesis and pathophysiology of idiopathic normal pressure hydrocephalus

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.     

Oxygen metabolism changes in patients with idiopathic normal pressure hydrocephalus before and after shunting operation

OBJECTIVE: The present study revealed the changes in cerebral oxygen metabolism before and after ventriculo-peritoneal shunt (VPS) using (15)O positron emission tomography ((15)O-PET). METHODS: Eight patients with idiopathic normal pressure hydrocephalus (i-NPH) underwent VPS. A (15)O-PET study was undertaken before and approximately 3 months after VPS. In five patients, the symptoms improved based on the classification by Krauss et al. [Neurosurgery 1996;39:292] (good responders) after VPS. In three patients, the symptoms improved subjectively following VPS (poor responders). The changes in oxygen metabolism before and after VPS were analyzed. RESULTS: The postoperative regional cerebral metabolic rate of oxygen (rCMRO(2)) of the good responders increased significantly. The postoperative regional oxygen extraction fraction (rOEF) is reduced in the poor responders. CONCLUSION: The improvement of rCMRO(2) correlated with the response to VPS. Significant changes in rOEF might predict poor response to VPS.     

Caudate structural abnormalities in idiopathic normal pressure hydrocephalus

BACKGROUND - Idiopathic normal pressure hydrocephalus (iNPH) is a reversible dementia in which fronto-striatal cognitive deficits and apathy may be present. OBJECTIVES - The study investigated structural volumetric changes in iNPH, apart from ventriculomegaly. MATERIALS AND METHODS - A full-brain voxel-based morphometric analysis between 11 iNPH patients and 14 healthy controls identified regions of interest (ROIs) for manual volumetric analyses. RESULTS - Caudate and corpus callosum ROI measurements revealed diminished caudate nuclei volume in the iNPH group. CONCLUSIONS - The role of the caudate nucleus in cognitive and affective changes in iNPH should now be explored.     

Loss of perivascular aquaporin-4 in idiopathic normal pressure hydrocephalus

Idiopathic normal pressure hydrocephalus (iNPH) is a subtype of dementia that may be successfully treated with cerebrospinal fluid (CSF) diversion. Recently, magnetic resonance imaging (MRI) using a MRI contrast agent as a CSF tracer revealed impaired clearance of the CSF tracer from various brain regions such as the entorhinal cortex of iNPH patients. Hampered clearance of waste solutes, for example, soluble amyloid-β, may underlie neurodegeneration and dementia in iNPH. The goal of the present study was to explore whether iNPH is associated with altered subcellular distribution of aquaporin-4 (AQP4) water channels, which is reported to facilitate CSF circulation and paravascular glymphatic drainage of metabolites from the brain parenchyma. Cortical brain biopsies of 30 iNPH patients and 12 reference individuals were subjected to AQP4 immunogold cytochemistry. Electron microscopy revealed significantly reduced density of AQP4 water channels in astrocytic endfoot membranes along cortical microvessels in patients with iNPH versus reference subjects. There was a significant positive correlation between density of AQP4 toward endothelial cells (perivascular) and toward parenchyma, but the reduced density of AQP4 toward parenchyma was not significant in iNPH. We conclude that perivascular AQP4 expression is attenuated in iNPH, potentially contributing to impaired glymphatic circulation, and waste clearance, and subsequent neurodegeneration. Hence, restoring normal perivascular AQP4 distribution may emerge as a novel treatment strategy for iNPH.     

Arterial blood pressure vs intracranial pressure in normal pressure hydrocephalus

Eide PK, Park E‐H, Madsen JR. Arterial blood pressure vs intracranial pressure in normal pressure hydrocephalus.
Acta Neurol Scand: 2010: 122: 262–269.
© 2010 John Wiley & Sons A/S. Objective – To characterize the association between arterial blood pressure (ABP) and intracranial pressure (ICP) in idiopathic normal pressure hydrocephalus (iNPH) patients, and its impact on outcome of shunt surgery. Materials and methods – We analyzed all 35 iNPH patients whose ABP and ICP were recorded simultaneously during 6 years (2002–2007). The static and pulsatile pressures were averaged over consecutive 6‐s intervals; the moving correlations between ICP and ABP (static and pulsatile) were determined during consecutive 4‐min periods to explore time‐related variations. Results – Neither static nor pulsatile ABP were altered in iNPH shunt responders. Elevated pulsatile ICP, but normal static ICP, was seen in responders. The time‐varying correlations of static and of pulsatile pressures were generally low, and did not differ between shunt responders/non‐responders. Conclusions – In iNPH shunt responders, static or pulsatile ABP were not altered and only pulsatile ICP was elevated.     

CSF biomarkers in the evaluation of idiopathic normal pressure hydrocephalus

BACKGROUND - To evaluate cerebrospinal fluid (CSF) markers for neuronal degeneration and demyelination in idiopathic normal pressure hydrocephalus (INPH), subcortical arteriosclerotic encephalopathy (SAE), and neurologically healthy subjects. METHODS - Lumbar CSF concentrations of sulfatide, neurofilament protein light (NFL), total-tau (T-tau), hyperphosphorylated tau (P-tau), and beta-amyloid(1-42) (Abeta42) were analyzed in 62 INPH patients, 26 SAE patients, and 23 neurologically healthy controls. In INPH patients, samples before and after shunt surgery were analysed. RESULTS - The CSF concentration of NFL was elevated in INPH and SAE compared with the controls, and levels of T-tau, P-tau, and Abeta42 were lower in INPH compared with SAE and controls. No difference was seen for sulfatide. All markers except Abeta42 were significantly elevated after shunt surgery. CONCLUSIONS - The most striking finding was the power of the combined pattern of NFL, P-tau, and Abeta42 in distinguishing between the clinical diagnoses of INPH, SAE, and neurologically healthy elderly.