正常压力性脑积水分流术效果的预测

正常压力性脑积水(NPH)是一种以步态障碍、痴呆、尿失禁为表现的神经外科常见疾病。病因不明,治疗上以脑脊液分流术最为有效。然而患者分流术后病情改善情况,结果不一。国内外学者从患者临床表现、辅助检查(CT、MRI)、脑脊液(CSF)动力学、血液动力学等方面研究,以期待通过对这些术前表现的分析来对术后效果进行预测,然而结论不一。     

正常压力脑积水分流手术效果的预测

正常压力脑积水分流手术治疗的效果仍有争议,正确估计病人的预后,选择合适病人手术是问题的关键。本文从临床、神经放射学、脑脊液循环动力学、脑血流的变化几个方面综述了影响分流手术效果的预后因素。     

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.     

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     

正常压力脑积水低压和中压分流比较

目的：对正常压力脑积水（ＮＰＨ）病人行低压脑室腹腔分流（ＬＰＶ）和中压脑室腹腔分流（ＭＰＶ）的结果比较和总结。方法：均用美国生产的脑室腹腔引流管,２０例ＮＰＨ病人平均分为两组,每组１０人,即ＬＰｖ（压力４０±１０ｍｍＨ２Ｏ）组和ＭＰＶ（压力１００±１０ｍｍＨ２Ｏ）组。ＮＰＨ的主要诊断标准为进行性痴呆、步态失调和尿便失禁。术后１、３、６、９、１２个月复查。平均进步：ＬＰＶ组则为７０％,ＭＰＶ组为５０     

脑室-腹腔分流术治疗外伤性正常压力性脑积水的疗效

目的探讨脑室-腹腔分流术(V-P分流术)治疗外伤性正常压力性脑积水的效果。方法回顾性分析48例外伤性正常压力性脑积水病人的临床资料,根据术前临床症状分为典型症状组和不典型症状组,均行V-P分流术,其中行去骨瓣减压术22例。并评估分流术后病情及预后情况。结果随访12个月,根据GOS评分,术后恢复良好12例,轻度残疾23例,重度残疾8例,植物生存3例,死亡2例。根据电话及门诊随访,病情改善41例(85.4%),病情无明显好转甚至恶化7例;其中典型症状组病情改善率100%,不典型症状组病情改善率72%。影像学检查提示:分流术后所有病人脑室均有不同程度缩小。22例行去骨瓣减压术的病人,分流术后6个月内均顺利行颅骨修补术,术后病情均明显改善。结论对外伤性正常压力性脑积水病人,把握好手术适应证,即使无典型临床症状,行V-P分流术后,大部分病人预后可获得改善。     

正常压力脑积水低压和中压分流比较

目的：对正常压力脑积水（NPH）病人行低压脑室腹腔分流（LPV）和中压脑室腹腔分流（MPV）的结果比较和总结。方法：均用美国生产的脑室腹腔引流管,20例NPH病人平均分为两组,每组10人,即LPv（压力40±10mmH2O）组和MPV（压力100±10mmH2O）组。NPH的主要诊断标准为进行性痴呆、步态失调和尿便失禁。术后1、3、6、9、12个月复查。平均进步：LPV组为70％,MPV组为50％;明显进步乃至症状完全消失者LPV组为50％,MPV组则为25％。整个结果说明LPV组效果较好。LPV组脑室缩小较MPV组明显,但硬膜下积液发生率为70％,而MPV组为30％,然而硬膜下积液对病人影响是不明显的。结论：对NPH病人LPV分流较MPV好,NPH的治疗以LPV分流为宜。     

醋甲唑胺治疗正常压力性脑积水的体会

目的 探讨醋甲唑胺在正常压力性脑积水治疗中的应用价值。方法 回顾性分析2015年1月至2020年1月5年保守治疗的24例正常压力性脑积水的临床资料,其中10例采用常规治疗（常规组）,14例在常规治疗的基础上加用醋甲唑胺治疗（醋甲唑胺）。结果 治疗1个月,醋甲唑胺组症状改善率（35.71%）与常规组（10.0%）无统计学差异（P>0.05）;治疗2、3个月,醋甲唑胺组症状改善率（71.43%、78.57%）较常规组（20.0%、20.0%）明显增高（P<0.05）。结论 在正常压力性脑积水保守治疗过程中,加用醋甲唑胺治疗,可以改善临床症状。     

正常压力型脑积水的影像学研究进展

总结近年影像学在正常压力型脑积水(NPH)的诊断和预后判断方面的进展。在Pubmed中应用下列检索词进行检索:"normal pressure hydrocephalus"、"imaging"+"diagnosis"、"prognosis"等,共检索到67篇文献,总结并加以讨论。在NPH的诊断和预后方面,影像学检查方法有着不可替代的优点,各种影像学方法的潜在价值也正在被不断探明。但创伤性检查仍将在未来一段时间内作为NPH的诊断和预后主要检查手段。     

特发性正常压力脑积水分流手术效果的术前评价

特发性正常压力脑积水（idiopathicnormalpressurehydrocephalus,INPH）,又称Hakim—Adams综合征,是指脑室有扩大而脑脊液压力正常的交通性脑积水综合征。其主要特点为典型的三联征（痴呆、步态障碍和尿失禁）、腰穿压力正常、CT或MRI显示交通性脑积水。自1965年INPH被首次提出至今的40余年里,分流手术被广泛应用,判断患者是否能从分流手术中获益的价值尚不确定。本文对预测INPH患者分流手术效果的相关因素进行综述。     

脑积水分流术后感染的外科治疗策略

对于脑积水分流术后感染,尽管已有一系列指南和共识推荐,不同医师给出的实际治疗策略仍然有很大差异.相当比例的医师会出于各种考虑进行保守治疗.笔者汇报1例分流感染、腹腔端阻塞而行保守治疗的患者,初始因考虑抗生素治疗有效,仅行处理分流管腹腔端阻塞的手术,后因感染控制不佳而行长通道脑室外引流,彻底控制感染后重新行脑室心房分流术.分享该病例旨在阐明分流感染治疗决策的复杂性,简要讨论保守治疗的可能性,并介绍用于安全廓清感染性脑脊液的长通道脑室外引流技术.     

侧脑室-腹腔分流术治疗正常压力脑积水28例

目的 探讨正常压力脑积水的手术治疗.方法 采用中、低压分流管行侧脑室-腹腔分流术.结果 本组28例,术后均得到6个月～3年随访,18例患者恢复正常生活,8例患者生活自理,2例患者术后症状无明显改善.结论 侧脑室-腹腔分流术是治疗正常压力脑积水最有效的方法.     

侧脑室-腹腔分流术治疗正常压力脑积水28例

目的 探讨正常压力脑积水的手术治疗。方法 采用中、低压分流管行侧脑室-腹腔分流术。结果 本组28例，术后均得到6个月～3年随访，18例患者恢复正常生活，8例患者生活自理，2例患者术后症状无明显改善。结论 侧脑室-腹腔分流术是治疗正常压力脑积水最有效的方法。     

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.     

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。     

Quantitative evaluation of changes in gait after extended cerebrospinal fluid drainage for normal pressure hydrocephalus

Idiopathic normal pressure hydrocephalus (iNPH) is characterized by gait instability, urinary incontinence and cognitive dysfunction. These symptoms can be relieved by cerebrospinal fluid (CSF) drainage, but the time course and nature of the improvements are poorly characterized. Attempts to prospectively identify iNPH patients responsive to CSF drainage by evaluating presenting gait quality or via extended lumbar cerebrospinal fluid drainage (eLCD) trials are common, but the reliability of such approaches is unclear. Here we combine eLCD trials with computerized quantitative gait measurements to predict shunt responsiveness in patients undergoing evaluation for possible iNPH. In this prospective cohort study, 50 patients presenting with enlarged cerebral ventricles and gait, urinary, and/or cognitive difficulties were evaluated for iNPH using a computerized gait analysis system during a 3 day trial of eLCD. Gait speed, stride length, cadence, and the Timed Up and Go test were quantified before and during eLCD. Qualitative assessments of incontinence and cognition were obtained throughout the eLCD trial. Patients who improved after eLCD underwent ventriculoperitoneal shunt placement, and symptoms were reassessed serially over the next 3 to 15 months. There was no significant difference in presenting gait characteristics between patients who improved after drainage and those who did not. Gait improvement was not observed until 2 or more days of continuous drainage in most cases. Symptoms improved after eLCD in 60% of patients, and all patients who improved after eLCD also improved after shunt placement. The degree of improvement after eLCD correlated closely with that observed after shunt placement.     

Clinical parameters in 74 consecutive patients shunt operated for normal pressure hydrocephalus

Seventy four consecutive patients diagnosed as normal pressure hydrocephalus (NPH) and operated with a ventriculo-peritoneal shunt were followed prospectively for an average of 2.1 years. The mean age was 64 years. The effect of the operation was estimated by calculating 6 indices expression social functioning, neurological signs, gait ability, continence, psychometric performance and psychiatric condition. Ninety-six % had mental symptoms, 95% gait disturbances and 75% incontinence. Improvement was observed in 78% after shunt surgery, while 22% deteriorated. Psychiatric improvement was seen in 80% and 76% improved in gait ability. Improvement was highest in the group caused by subarachnoid hemorrhage (98%) while 73% with idiopathic NPH improved. Old age was not correlated to poorer response, while long-standing pre-operative symptoms yielded worse results. No single symptom or sign could predict a positive outcome of the operation. Eleven % of the patients could leave long-term care institutions and there was a 36% reduction of aid in daily living. Complications to shunt surgery were observed in 50% of the patients and shunt related mortality was 1%. Shunt malfunction was the most frequent complication (31%) followed by infection (19%). Epilepsy developed in 9% after surgery. All shunt complication except one appeared within the first year after surgery.     

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。     

脑室腹腔分流术治疗成人外伤性脑积水分流管的选择

目的探讨脑室一腹腔分流术治疗成人外伤性脑积水时分流管的选择。方法回顾性分析2010年1月至2012年12月脑室-腹腔分流术治疗48例成人外伤性脑积水患者的临床资料。选择抗虹吸分流管的42例,选择非抗虹吸分流管的6例;选择可调压分流阀的28例,选择固定压力分流阀的20例。结果术后1周临床症状缓解37例（77．1％,37／48）症状缓解不明显11例（22．9％,11／48）。头颅CT或MRI复查示分流管脑室端位于脑室内,脑室缩小。术后随访2～38个月,重新再调整阀门压力8例,每例调整2～4次。结论脑室一腹腔分流术仍是治疗成人外伤性脑积水的首选方式,有条件的患者尽量选择可调压抗虹吸分流管,对于不愿选择可调压分流管的患者,可选择低压或中压分流管。