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Background Cerebrospinal compliance describes the ability of the cerebrospinal space to buffer changes in volume. Diminished compliance is associated with increased risk of potentially threatening increases in intracranial pressure (ICP) when changes in cerebrospinal volume occur. However, despite various methods of estimation proposed so far, compliance is seldom used in clinical practice. This study aimed to compare three measures of cerebrospinal compliance. Methods ICP recordings from 36 normal-pressure hydrocephalus patients who underwent infusion tests with parallel recording of transcranial Doppler blood flow velocity were retrospectively analysed. Three methods were used to calculate compliance estimates during changes in the mean ICP induced by infusion of fluid into the cerebrospinal fluid space: (a) based on Marmarou’s model of cerebrospinal fluid dynamics (C CSF ), (b) based on the evaluation of changes in cerebral arterial blood volume (C CaBV ), and (c) based on the amplitudes of peaks P1 and P2 of ICP pulse waveform (C P1/P2 ). Results Increase in ICP caused a significant decrease in all compliance estimates ( p < 0.0001). Time courses of compliance estimators were strongly positively correlated with each other (group-averaged Spearman correlation coefficients: 0.94 [0.88–0.97] for C CSF vs. C CaBV , 0.77 [0.63–0.91] for C CSF vs. C P1/P2 , and 0.68 [0.48–0.91] for C CaBV vs. C P1/P2 ). Conclusions Indirect methods, C CaBV and C P1/P2 , allow for the assessment of relative changes in cerebrospinal compliance and produce results exhibiting good correlation with the direct method of volumetric manipulation. This opens the possibility of monitoring relative changes in compliance continuously.

Background Positive shunt response (SR) remains the gold standard for diagnosing idiopathic normal pressure hydrocephalus (iNPH). However, multiple pathologies mimic iNPH symptoms, making it difficult to select patients who will respond to shunt surgery. Although presenting features, extended lumbar drainage (ELD), infusion test (IT), intracranial pressure monitoring (ICPM), and tap test (TT) have been used to predict SR, uncertainty remains over which diagnostic test to choose. Objective To conduct a systematic review and meta-analysis to identify clinical predictors of shunt responsiveness, evaluate their diagnostic effectiveness, and recommend the most effective diagnostic tests. Methods Embase, MEDLINE, Scopus, PubMed, Google Scholar, and JSTOR were searched for original studies investigating clinical predictors of SR in iNPH patients. Included studies were assessed using the QUADAS-2 tool, and eligible studies were evaluated using univariate and bivariate meta-analyses. Results Thirty-five studies were included. Nine studies discussed the diagnostic use of presenting clinical features, 8 studies ELD, 8 studies IT, 11 studies ICPM, and 6 studies TT. A meta-analysis of 21 eligible studies was conducted for TT, ELD, IT, and ICPM. ICPM yielded the highest diagnostic effectiveness, with diagnostic odds ratio (DOR) = 50.9 and area under curve (AUC) = 0.836. ELD yielded DOR = 27.70 and AUC = 0.753, IT had DOR = 5.70 and AUC = 0.729, and TT scored DOR = 3.86 and AUC = 0.711. Conclusion Intraparenchymal ICPM is statistically the most effective diagnostic test, followed by ELD, IT, and lastly TT. Due to the higher accessibility of TT and IT, they are recommended to be used first line, using a timed-up-and-go improvement ≥ 5.6 s or a Rout cut-off range between 13 and 16 mmHg, respectively. Patients who test negative should ideally be followed up with ICPM, using mean ICP wave amplitude ≥ 4 mmHg, or 1- to 4-day ELD with an MMSE cut-off improvement ≥ 3. Future research must use standardized methodologies for each diagnostic test and uniform criteria for SR to allow better comparison.

Background Patients with the dementia subtype idiopathic normal pressure hydrocephalus (iNPH) may improve clinically following cerebrospinal fluid (CSF) diversion (shunt) surgery, though the predictors of shunt response remain debated. Currently, radiological features play an important role in the diagnosis of iNPH, but it is not well established which radiological markers most precisely predict shunt responsive iNPH. Objective To conduct a systematic review and meta-analysis to identify radiological predictors of shunt responsiveness, evaluate their diagnostic effectiveness, and recommend the most predictive radiological features. Methods Embase, MEDLINE, Scopus, PubMed, Google Scholar, and JSTOR were searched for original studies investigating radiological predictors of shunt response in iNPH patients. Included studies were assessed using the ROBINS-1 tool, and eligible studies were evaluated using a univariate meta-analysis. Results Overall, 301 full-text papers were screened, of which 28 studies were included, and 26 different radiological features were identified, 5 of these met the inclusion criteria for the meta-analysis: disproportionately enlarged subarachnoid space (DESH), callosal angle, periventricular white matter changes, cerebral blood flow (CBF), and computerized tomography cisternography. The meta-analysis showed that only callosal angle and periventricular white matter changes significantly differentiated iNPH shunt responders from non-responders, though both markers had a low diagnostic odds ratio (DOR) of 1.88 and 1.01 respectively. None of the other radiological markers differentiated shunt responsive from shunt non-responsive iNPH. Conclusion Callosal angle and periventricular changes are the only diagnostically effective radiological predictors of shunt responsive iNPH patients. However, due to the DORs approximating 1, they are insufficient as sole predictors and are advised to be used only in combination with other diagnostic tests of shunt response. Future research must evaluate the combined use of multiple radiological predictors, as it may yield beneficial additive effects that may allow for more robust radiological shunt response prediction.

Abstract BackgroundIdiopathic normal pressure hydrocephalus (iNPH) is a neurodegenerative disease and dementia subtype involving disturbed cerebrospinal fluid (CSF) homeostasis. Patients with iNPH may improve clinically following CSF diversion through shunt surgery, but it remains a challenge to predict which patients respond to shunting. It has been proposed that CSF and blood biomarkers may be used to predict shunt response in iNPH.ObjectiveTo conduct a systematic review and meta-analysis to identify which CSF and venous biomarkers predict shunt-responsive iNPH most accurately.MethodsOriginal studies that investigate the use of CSF and venous biomarkers to predict shunt response were searched using the following databases: Embase, MEDLINE, Scopus, PubMed, Google Scholar, and JSTOR. Included studies were assessed using the ROBINS-I tool, and eligible studies were evaluated utilising univariate meta-analyses.ResultsThe study included 13 studies; seven addressed lumbar CSF levels of amyloid-β 1–42, nine studies CSF levels of Total-Tau, six studies CSF levels of Phosphorylated-Tau, and seven studies miscellaneous biomarkers, proteomics, and genotyping. A meta-analysis of six eligible studies conducted for amyloid-β 1–42, Total-Tau, and Phosphorylated-Tau demonstrated significantly increased lumbar CSF Phosphorylated-Tau (− 0.55 SMD, p = 0.04) and Total-Tau (− 0.50 SMD, p = 0.02) in shunt-non-responsive iNPH, though no differences were seen between shunt responders and non-responders for amyloid-β 1–42 (− 0.26 SMD, p = 0.55) or the other included biomarkers.ConclusionThis meta-analysis found that lumbar CSF levels of Phosphorylated-Tau and Total-Tau are significantly increased in shunt non-responsive iNPH compared to shunt-responsive iNPH. The other biomarkers, including amyloid-β 1–42, did not significantly differentiate shunt-responsive from shunt-non-responsive iNPH. More studies on the Tau proteins examining sensitivity and specificity at different cut-off levels are needed for a robust analysis of the diagnostic efficiency of the Tau proteins.

Background CSF diversion with shunt placement is frequently associated with need for later revisions as well as surgical complications. We sought to review revision and complication rates following ventriculoperitoneal, ventriculoatrial and cystoperitoneal shunt placement in adult patients, and to identify potential risk factors for revision surgery and postoperative complications. Method Included patients were adults (≥ 18 years) who underwent primary shunt insertion at St. Olavs Hospital in Trondheim, Norway, from 2008 through 2017. The electronic medical records and diagnostic imaging from all hospitals in our catchment area were retrospectively reviewed. Follow-up ranged from 1 to 11 years. Complications were graded according to the Landriel Ibañez classification system. Results Of the 227 patients included, 47 patients (20.7%) required revision surgery during the follow-up. In total, 90 revision surgeries were performed during follow-up. The most common cause for the first revision was infection (5.7%) and for all revisions proximal occlusion (30.0%). A total of 103 patients (45.4%) experienced ≥ 1 complication(s). Mild to moderate complications (grade I and II) were detected in 35.0% of all procedures. Severe or fatal complications (grade III and IV) were observed in 8.2% of all procedures . Urinary tract infections and pneumonia were common postoperatively (13.9% and 7.3%, respectively), and the most common IIb complication was shunt misplacement (proximally or distally). Two out of fourteen deaths within 30 days were directly associated with surgery. We did not find that aetiology/indication, age or gender influenced the occurrence of revision surgery or a grade III or IV complication. Conclusions Shunt surgery continues to be a challenge both in terms of revision rates and procedure-related complications. However, the prediction of patients at risk remains difficult. A multidimensional focus is probably needed to reduce risks.

ObjectivePseudotumour cerebri syndrome (PTCS including idiopathic intracranial hypertension) is characterised by the symptoms and signs of raised cerebrospinal fluid pressure (CSFp) in the absence of ventricular dilatation or an intracranial mass lesion. Its aetiology is unknown in the majority of cases but there is much evidence for impaired CSF absorption. Traditionally, sagittal sinus pressure has been considered to be independent of CSF pressure in adults. However, the discovery of stenoses of intracranial venous sinuses and introduction of venous sinus stenting has highlighted the importance of the venous drainage in PTCS. In this study, we have explored the relationship between CSFp and SSp before and during a CSF infusion test and during CSF drainage.Materials and methodsTen patients (9 females:1 male) with PTCS underwent infusion studies in parallel with direct retrograde cerebral venography. Both SSp and CSFp were recorded at a baseline and during CSFp elevation in a course of a CSF infusion test. The drainage of CSF after the CSF infusion was performed in 7 patients. In 5 cases, jugular venous pressure was also measured.ResultsCSFp and SSp including their amplitudes correlated significantly and strongly both at baseline (R = 0.96; p = 0.001) and during infusion (R = 0.92; p = 0.0026). During drainage, this correlation was maintained until SSp reached a stable value, whereas CSFp continued to decrease.ConclusionsIn this series of ten patients with PTCS, CSFp and SSp were coupled, both at baseline and during infusion. The implications of such coupling for the calculation of CSF outflow resistance are discussed.

In patients with hydrocephalus, prognosis and intervention are based on multiple factors. This includes, but is not limited to, time of onset, patient age, treatment history, and obstruction of cerebrospinal fluid flow. Consequently, several distinct hydrocephalus classification systems exist. The International Classification of Diseases (ICD) is universally applied, but in ICD-10 and the upcoming ICD-11, hydrocephalus diagnoses incorporate only a few factors, and the hydrocephalus diagnoses of the ICD systems are based on different clinical measures. As a consequence, multiple diagnoses can be applied to individual cases. Therefore, similar patients may be described with different diagnoses, while clinically different patients may be diagnosed identically. This causes unnecessary dispersion in hydrocephalus diagnostics, rendering the ICD classification of little use for research and clinical decision-making. This paper critically reviews the ICD systems for scientific and functional limitations in the classification of hydrocephalus and presents a new descriptive system. We propose describing hydrocephalus by a system consisting of six clinical key factors of hydrocephalus: A (anatomy); S (symptomatology); P (previous interventions); E (etiology); C (complications); T (time–onset and current age). The “ ASPECT Hydrocephalus System ” is a systematic, nuanced, and applicable description of patients with hydrocephalus, with a potential to resolve the major issues of previous classifications, thus providing new opportunities for standardized treatment and research.

Background Idiopathic intracranial hypertension (IIH) is a rare disease of unknown aetiology related possibly to disturbed cerebrospinal fluid (CSF) dynamics and characterised by elevated intracranial pressure (ICP) causing optic nerve atrophy if not timely treated. We studied CSF dynamics of the IIH patients based on the available literature and our well-defined cohort. Method A literature review was performed from PubMed between 1980 and 2020 in compliance with the PRISMA guideline. Our study includes 59 patients with clinical, demographical, neuro-ophthalmological, radiological, outcome data, and lumbar CSF pressure measurements for suspicion of IIH; 39 patients had verified IIH while 20 patients did not according to Friedman’s criteria, hence referred to as symptomatic controls. Results The literature review yielded 19 suitable studies; 452 IIH patients and 264 controls had undergone intraventricular or lumbar CSF pressure measurements. In our study, the mean CSF pressure, pulse amplitudes, power of respiratory waves (RESP), and the pressure constant (P 0 ) were higher in IIH than symptomatic controls ( p < 0.01). The mean CSF pressure was higher in IIH patients with psychiatric comorbidity than without ( p < 0.05). In IIH patients without acetazolamide treatment, the RAP index and power of slow waves were also higher ( p < 0.05). IIH patients with excess CSF around the optic nerves had lower relative pulse pressure coefficient (RPPC) and RESP than those without ( p < 0.05). Conclusions Our literature review revealed increased CSF pressure, resistance to CSF outflow and sagittal sinus pressure (SSP) as key findings in IIH. Our study confirmed significantly higher lumbar CSF pressure and increased CSF pressure waves and RAP index in IIH when excluding patients with acetazolamide treatment. In overall, the findings reflect decreased craniospinal compliance and potentially depleted cerebral autoregulation resulting from the increased CSF pressure in IIH. The increased slow waves in patients without acetazolamide may indicate issues in autoregulation, while increased P 0 could reflect the increased SSP.

BackgroundTraditionally, clinical findings of normal pressure hydrocephalus are mainly characterized by the Hakim triad. The aim of this study is to evaluate the performance of patients suffering from idiopathic normal pressure hydrocephalus (iNPH) in a more holistic manner regarding motor skills, cognitive impairment, and quality of life.MethodsIn total, 30 individuals diagnosed with iNPH as well as a reference group with another 30 individuals were included. The iNPH patients and the reference group were age, educational, and morbidity matched. A standardized test battery for psychomotor skills, gait, neuropsychological abilities as well as questionnaires for quality of life was applied. The iNPH group was tested prior to surgery, at 6 weeks, and 3 months postoperatively. The reference group was tested once.ResultsPatients showed a significant improved performance in various items of the test battery during the first 3 months postoperatively. This included neuropsychological evaluation, motor skills including gait and upper motor function as well as the quality of life of the patients. Compared to reference individuals, neuropsychological aspects and quality of life of iNPH patients improved in some parts nearly to normal values.ConclusionOur findings underline that shunt surgery does not only improve the symptoms in iNPH patients but also ameliorates the quality of life to a great extent close to those of age and comorbidity matched reference individuals. This data enables an optimized counseling of iNPH patients regarding the expectable outcome after shunt surgery especially regarding cognitive performance, motor skills as well as life quality.

BackgroundNormal pressure hydrocephalus (NPH) is a neurological disorder, characterized by gait- and balance disturbance, cognitive deterioration, and urinary incontinence, combined with ventricular enlargement. Gait ability, falls, cognitive status, and health-related quality of life pre and post surgery have not previously been studied at Karolinska University Hospital.MethodsOne hundred and eighteen patients with iNPH that underwent shunt surgery at Karolinska University Hospital during the years from 2016 to 2018 were included. Results of walking tests, test for cognitive function, and self-estimated health-related quality of life, before and 3 months after surgery, were collected retrospectively as a single-center study.ResultsWalking ability, cognitive function, and health-related quality of life significantly increased 3 months after shunt surgery. A positive significant correlation was seen between a higher self-estimated quality of life and walking ability.ConclusionsPatients with suspected iNPH treated with shunt surgery at Karolinska University Hospital improved their walking ability and cognitive functioning 3 months after shunt surgery. A positive significant correlation was seen between a higher self-estimated quality of life and walking ability but not with increased cognitive function. We then concluded that the selection of patients for shunting maintained a high standard.