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、     

腰椎穿刺术在特发性正常压力脑积水治疗中的应用

目的探讨腰椎穿刺技术在老年人特发性正常压力脑积水治疗中的应用价值。方法回顾性分析54例特发性正常压力脑积水患者的临床资料。结果本组43例患者术后均得到6—12月随访,显效25例;有效18例;本组43例腰椎穿刺脑脊液放液试验症状改善患者手术有效率100％,术后患者步态障碍、尿失禁改善明显,智能障碍均有不同程度改善,影像学检查见脑室系统有不同程度缩小。结论腰椎穿刺放液试验是筛选特发性正常压力脑积水及预测手术疗效的有效方法。     

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。     

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.     

脑脊液引流试验对原发性正常压力性脑积水分流手术效果的预测

目的应用脑脊液引流试验(Tap试验)对原发性正常压力性脑积水(iNPH)病人进行术前评估,预测分流手术的有效性。方法前瞻性研究50例iNPH病人,先行Tap试验,5 d后行脑室-腹腔分流术(V-P分流术)。Tap试验前1 h、Tap试验后24 h及分流术后7 d分别对病人的神经功能进行评估。通过筛检试验统计分析评估Tap试验并预测iNPH病人分流手术的有效性。结果 Tap试验阳性31例,其中V-P分流术临床症状改善30例,未改善1例;Tap试验阴性19例,其中V-P分流术后症状改善6例,未改善13例。结果提示Tap试验对iNPH分流手术的灵敏度为83.3%,特异度92.9%,阳性预测率96.8%,阴性预测率68.4%。结论 Tap试验是理想的iNPH分流手术疗效的阳性预测指标。     

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发病机制的研究新进展

特发性正常压力脑积水(idiopathic normal pressure hydrocephalus,iNPH),是颅内压在正常范围的一种特殊类型的交通性脑积水,主要发生于成人,以步态异常、认知障碍和尿失禁为典型表现。尽管临床特征和影像学表现典型,但其发病机制仍不十分清楚。目前认为颅内静脉系统顺应性降低、蛛网膜颗粒功能受损,导致脑脊液(cerebrospinal fluid,CSF)的流动和吸收障碍,进而产生相应临床症状[1]。然而,CSF循环动力学异常、炎症反应、渗透异常和淋巴引流障碍等都与iNPH的发病机制息息相关。本文就iNPH发病机制的研究新进展进行综述。     

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.     

Changes in carotid flow velocity induced by lowering cerebrospinal fluid pressure in normal pressure hydrocephalus

Summary This is a report of changes in blood flow velocity in the carotid system induced by lumbar puncture in five patients who had clinical and neuroradiological signs of normal pressure hydrocephalus.After lowering cerebrospinal fluid pressure an increase of carotid flow velocity was found on Doppler hematotachography. These changes of carotid blood flow velocity could not be demonstrated in a control group of four patients with normal cerebrospinal fluid dynamics.This method is easy to perform, does not overtax the patient and seems to be indicated for the diagnosis of NPH. It is presented as a new, simple, reproducible aid to the diagnosis of NPH by Doppler hematotachography.

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Zusammenfassung Diese Präliminarstudie gibt eine Analyse der veränderten Blutstromgeschwindigkeit des Carotissystems nach einer Lumbalpunktion bei 5 Patienten, welche klinische und neurologische Symptome eines Hydrocephalus mit normalem Liquordruck zeigten.Nach Drucksenkung der Zerebrospinalflüssigkeit zeigte sich auf der Doppler-Hämatotachographie eine schnellere Durchströmung der Halsschlagader. In einer Kontrollgruppe von 4 Patienten mit normaler Dynamik der Zerebrospinalflüssigkeit konnten die Änderungen in der Blutdurchströmung der Halsschlagader nicht nachgewiesen werden.Die Durchführung dieser Methode ist einfach, sie beansprucht den Patienten nicht allzusehr und scheint indiziert für die Erkennung des Hydrocephalus mit normalem Druck.

     

Late onset X-linked hydrocephalus with normal cerebrospinal fluid pressure

A family with X-linked hydrocephalus with normal cerebrospinal fluid (CSF) pressure and in which three brothers and a grandson of case 1, a proband, were affected is reported. The symptoms at onset were epileptic attacks that started in adulthood in the three brothers and at the age of 6 years in the grandson. In the three brothers, from 10 to 27 years after the onset of epileptic episodes, disorganization of intelligence and psychiatric deterioration were gradually noticed by their families. At the same time, they showed occasional urinary incontinence. Brain computed tomography (CT) scans revealed dilatation of the ventricular systems. Based on the results of the measurement of CSF pressure and radioactive-iodinated human serum albumin (RISA)-cysternography, two of the brothers were diagnosed as having normal pressure hydrocephalus (NPH), and they were treated neurosurgically. However, no obvious improvement in clinical symptoms was observed. Although the grandson had shown normal psychomotor development during his early childhood, temporal epilepsy and temper tantrums started at the age of 6 years. Computed tomography-scanning revealed dilatation of the ventricular system similar to the other three cases at the age of 8 years. With the diagnosis of NPH, the patient underwent a shunt operation, which resulted in no obvious effects. As it is reasonable to surmise that the pathological gene would have been transferred via the daughter of the proband to the grandson, it is suggested that the inheritance manner might be X-linked recessive. The cases presented here are different from the cases of hydrocephalus due to stenosis of the aqueduct Sylvius (HSAS) and other types of X-linked hydrocephalus reported previously in terms of the age of onset, course, symptoms, and CT findings. Thus, it is suggested that the present cases might be a new type of X-linked hydrocephalus.     

Differences in cerebrospinal fluid dynamics do not affect the levels of biochemical markers in ventricular CSF from patients with aqueductal stenosis and idiopathic normal pressure hydrocephalus

To compare levels of biochemical markers in ventricular cerebrospinal fluid (vCSF) between patients with aqueductal stenosis (AS) and idiopathic normal pressure hydrocephalus (INPH) and relate these results to clinical outcome after surgery. Neurofilament light protein, tau protein, sulfatide, vasoactive intestinal peptide (VIP), neuropeptide PYY (NPY) and CSF/serum albumin ratio were measured in vCSF from 18 consecutive AS and 19 consecutive INPH patients. Clinical outcome was evaluated after surgery by standardized indices. The levels of markers were related to clinical outcome. No differences in any of the markers were found between AS and INPH patients. The concentration of sulfatide and albumin ratio correlated inversely with psychometric improvement, whilst VIP and NPY correlated inversely with improvement in alertness. The similar levels of biochemical markers in vCSF from AS and INPH patients indicate similarities in pathophysiology and turnover rate of vCSF despite differences in CSF dynamics. High albumin ratio and sulfatide concentrations in vCSF in hydrocephalus patients have negative implications for surgical outcome and might indicate concomitant cerebrovascular disorder.     

High cerebrospinal fluid concentration of glial fibrillary acidic protein (GFAP) in patients with normal pressure hydrocephalus

The concentration of glial fibrillary acidic protein (GFAP) in lumbar cerebrospinal fluid (CSF) was measured in 12 patients with normal pressure hydrocephalus (NPH) 11 patients with primary degenerative dementia (PDD), 8 patients with various other neurological diseases, and 18 patients without signs of organic nervous disease (controls).

Mean CSF GFAP concentration was significantly higher in NPH patients: 96 ± 23 ng/ml (SEM) when compared with PDD patients: 8.2 ± 1.9 ng/ml (P < 0.01), or with controls: 4.3 ± 0.7 ng/ml (P < 0.01). Only 2 NPH patients had a GFAP concentration within the range of the control group (2–14 ng GFAP/ml CSF). No significant differences were found between the PDD patients and the control group, or between the group of patients with other neurological diseases and the control group.

In addition, a rostro-caudal gradient of GFAP in CSF could be demonstrated. In 6 NPH and 2 PDD patients both ventricular and lumbar CSF samples were investigated. In all cases the ventricular GFAP concentration was higher than the lumbar concentration. The difference was statistically significant (P < 0.01).

Our results suggest that determination of CSF GFAP concentration might be of diagnostic value in discrimination between NPH patients and patients with enlarged ventricles associated with degenerative brain disease.     

Ventricular cerebrospinal fluid neurofilament protein levels decrease in parallel with white matter pathology after shunt surgery in normal pressure hydrocephalus

Normal pressure hydrocephalus (NPH) is characterized by disturbed cerebrospinal fluid (CSF) dynamics and white matter lesions (WML). Although the morphology of these lesions is described, little is known about the biochemistry. Our aim was to explore the relationship between ventricular CSF markers, periventricular WML and postoperative clinical outcome in patients with NPH. We analysed lumbar and ventricular concentrations of 10 CSF markers, 12 clinical symptoms and signs, magnetic resonance imaging (MRI) periventricular white matter hyperintensities (PVH) and ventricular size before and 3 months after shunt surgery in 35 patients with NPH. Higher ventricular CSF neurofilament protein (NFL), an axonal marker, correlated with more extensive PVH. A larger postoperative reduction in NFL correlated with larger reduction in PVH and a more pronounced overall improvement. Albumin ratio, HMPG, NPY, VIP and GD3 increased postoperatively whereas NFL, tau and HVA decreased. Variations in ventricular size were not associated with CSF concentrations of any marker. We conclude that NPH is characterized by an ongoing periventricular neuronal dysfunction seen on MRI as PVH. Clinical improvement after shunt surgery is associated with CSF changes indicating a restitution of axonal function. Other biochemical effects of shunting may include increased monoaminergic and peptidergic neurotransmission, breakdown of blood brain barrier function, and gliosis.     

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.