Objectives: Aquaductal web (AW) is a special form of aqueductal stenosis with similar clinical presentation with idiopathic normal pressure hydrocephalus (iNPH). iNPH is indeed a communicating ...hydrocephalus syndrome whereas AW is a noncommunicating subtype. Here, we aimed to investigate the similarities and differences between these two different chronic hydrocephalus syndromes in terms of clinical signs and symptoms, response to shunt treatment and postoperative complications.
Methods: Forty-one patients who underwent shunt operation with the diagnosis of iNPH or AW at our clinic between January 2010-May 2019 were retrospectively analyzed. Patients were evaluated by age, gender, clinical sign and symptoms, comorbidities, intraoperative and postoperative complications, and early and late postoperative outpatient follow-up findings.
Results: Twenty-six patients were classified as iNPH group and 15 patients as AW group. Patients in the AW group were significantly younger (45.5 ± 15.6 years vs. 60.3 ± 15.4 years) than the iNPH group (p = 0.006). There was no statistical difference between the groups in terms of subdural effusion formation, need for shunt revision (p = 1.000). Chronic hydrocephalus symptoms regressed in 23 (88.5%) patients in the NPH group, and at least one of them improved. symptoms. This rate was 66.7% (n = 10) in the AW group. Both groups showed similar clinical improvement with VPS (p = 0.1169).
Conclusions: The placement of ventriculoperitoenal shunt is widely used in the treatment of iNPH. As iNPH and AW has clinical similarities despite the discrepancies between underlying pathophysiological mechanisms and both clinical entities respond similarly to shunt treatment we advocate VPS surgery in the management of AW as well.
ObjectiveTo summarize the clinical and imaging characteristics with Dandy-Walker syndrome (DWS) in children before and after peritoneal shunt. Methods and Results All 6 children with DWS admitted to ...Fujian Sanbo Funeng Brain Hospital from January 2016 to January 2023 were selected as the objects of observation, including one case of Dandy-Walker malformation (DWM) and 5 cases of Dandy- Walker variant (DWV). Before surgery, the main manifestations were cerebellar vermis defect, upward movement of the tentorial and sinusional area, severe dilatation of the fourth ventricle, posterior cranial fossa cystic lesions, supratentorial ventricle dilatation and hydrocephalus (one case); or cerebellar vermis dysplasia, cerebellar hemisphere compression, the fourth ventricle dilatation, posterior cranial fossa cystic lesions, supratentorial ventricle dilatation and hydrocephalus (5 cases). Right lateral ventriculoperitoneal shunt (2 cases) or posterior cranial fossa cyst-peritoneal shunt (4 cases) were performed, and the
Patients diagnosed with low-pressure hydrocephalus typically present with enlarged ventricles and unusually low intracranial pressure, often measuring below 5 cmH2O or even below atmospheric ...pressure. This atypical presentation often leads to low recognition and diagnostic rates. The development of low-pressure hydrocephalus is believed to be associated with a decrease in the viscoelasticity of brain tissue or separation between the ventricular and subarachnoid spaces. Risk factors for low-pressure hydrocephalus include subarachnoid hemorrhage, aqueduct stenosis, prior cranial radiotherapy， ventricular shunting, and cerebrospinal fluid leaks. For potential low-pressure hydrocephalus, diagnostic criteria include neurological symptoms related to hydrocephalus, an Evans index >0.3 on imaging, ICP ≤ 5 cm H2O, symptom improvement with negative pressure drainage, and exclusion of ventriculomegaly caused by neurodegenerative diseases. The pathogenesis and pathophysiological features of low-pressure hydrocephalus differ significantly from other types of hydrocephalus, making it challenging to restore normal ventricular morphology through conventional drainage methods. The primary treatment options for low-pressure hydrocephalus involve negative pressure drainage and third ventriculostomy. With appropriate treatment, most patients can regain their previous neurological function. However, in most cases, permanent shunt surgery is still necessary. Low-pressure hydrocephalus is a rare condition with a high rate of underdiagnosis and mortality. Early identification and appropriate intervention are crucial in reducing complications and improving prognosis.
•In this review, we discussed the underlying mecnanism contributing to the development of LPH•We shed light on the distinctive pathophysiological features of LPH that differentiate it from other types of hydrocephalus•We outlined the primary treatments for LPH, including negative pressure drainage and third ventriculostomy.
Frailty is known to predict dementia. However, its link with neurodegenerative alterations of the central nervous system (CNS) is not well understood at present. We investigated the association ...between the biomechanical response of the CNS and frailty in older adults suspected of normal pressure hydrocephalus (NPH) presenting with markers of multiple co-existing pathologies. The biomechanical response of the CNS was characterized by the CNS elastance coefficient inferred from phase contrast magnetic resonance imaging and intracranial pressure monitoring during a lumbar infusion test. Frailty was assessed with an index of health deficit accumulation. We found a significant association between the CNS elastance coefficient and frailty, with an effect size comparable to that between frailty and age, the latter being the strongest known risk factor for frailty. Results were independent of CSF dynamics, showing that they are not specific to the NPH neuropathological condition. The CNS biomechanical characterization may help to understand how frailty is related to neurodegeneration and detect the shift from normal to pathological brain ageing.
Purpose: Periventricular caps (PVC) are a common finding on MR imaging. It is not fully understood to what extent PVC are caused by transependymal edema or gliosis. To understand the underlying ...pathophysiology, we compared the quantitative water content of PVC and gliotic white matter lesions (WML), hypothesizing that periventricular caps are caused by transependymal edema rather than gliosis. Methods: In a prospective study, we compared the water content of PVC and WML in 50 patients, using a quantitative multiple-echo gradient-echo MR water mapping sequence. Results: Compared to normal white matter, the water content of PVC was significantly higher than that of WML (p = .002) with an average increase of 17 + or - 5% in PVC and 11 + or - 4% in WML. ROC analysis revealed that a relative increase of water content of >15% rather corresponds to PVC than WML with a specificity of 93% and a sensitivity of 60% (p<.001). Conclusion: Our results, which show that the water content of PVC was significantly higher than that in WML, imply a differing pathophysiology of these lesions. Quantifying the water content of T2-hyperintensities may be a useful additional tool for the characterization and differentiation of T2-hyperintensities in diseases such as idiopathic normal pressure hydrocephalus.
Objective Ventriculoperitoneal (VP) shunt surgery is the predominant mode of therapy for patients with hydrocephalus. However, it has potential complications that may require multiple surgical ...procedures during a patient's lifetime. The objective of this study is to review our long-term experience and evaluate the risk factors for VP shunt failure after initial shunt surgery and after subsequent revisions. Methods Patients who underwent VP shunt surgery for hydrocephalus were included. Medical charts, operative reports, imaging studies, and clinical follow-up evaluations were reviewed and analyzed retrospectively. Results A total of 1015 patients with the median age of 41.6 (range, 0–90.3) years at the time of VP shunt surgery were included. The mean and median follow up was 9.2 and 6.5 years, respectively. Adult patients (≥17 years) accounted for 70.0% of the patients. The overall shunt failure rate requiring shunt revision(s) was 46.3%, and the majority of shunt revisions occurred during the first 6 months after shunt placement. The shunt revision rate was significantly greater in pediatric (<17 years) than in adult (>17 years) patients (78.2% vs. 32.5%, P < 0.001). Age at the time of shunt surgery, previous treatments to shunt surgery, etiology of hydrocephalus, and hydrocephalus type were independently associated with the incidence of shunt revision. Age at shunt placement and sex were significantly associated with multiple shunt revisions. Among populations with at least one shunt revision, pediatric patients had significantly lower shunt survival rate and shorter median time to subsequent shunt revision than the adult (>17 years) patients; male patients had greater odds for multiple revisions than female patients. Conclusion The findings of the study indicate that age at shunt placement, etiology of hydrocephalus, type of hydrocephalus, and previous treatments before shunt surgery were independently significantly associated with the shunt survival. Prospective controlled studies are required to address the observed associations between the risk factors and incidence of shunt revisions in these patients.
Normal pressure hydrocephalus (NPH) is an important differential diagnosis of neurodegenerative diseases. The prevalence of dementia is increasing in line with the worldwide increase in life ...expectancy. NPH can be divided into idiopathic (iNPH) and secondary (sNPH) which is important in terms of clinical symptoms, future progress, and the outcome of possible treatment. The full clinical triad is not prevalent in all of the cases and the pathophysiology of iNPH remains unclear. Diagnosis is based on the evaluation of clinical symptoms (Hakim’s triad) combined with an MRI assessment, evaluation of CSF dynamic parameters by different methods such as a tap test, lumbar infusion test (LIT), and external lumbar drainage (ELD). Despite the development of diagnostic techniques and strategies in management, NPH remains to be a challenge for the specialists despite more than 50 years of research. However, results of this research have brought new opportunities in the diagnosis, therapy, and quality of life as well as survival time of NPH patients with improved symptoms. The aim of this article is to present the pathophysiological hypotheses of NPH and an overview of the diagnostic techniques used for the evaluation of NPH patients.
Congenital hydrocephalus (CH), characterized by enlarged brain ventricles, is considered a disease of excessive cerebrospinal fluid (CSF) accumulation and thereby treated with neurosurgical CSF ...diversion with high morbidity and failure rates. The poor neurodevelopmental outcomes and persistence of ventriculomegaly in some post-surgical patients highlight our limited knowledge of disease mechanisms. Through whole-exome sequencing of 381 patients (232 trios) with sporadic, neurosurgically treated CH, we found that damaging de novo mutations account for >17% of cases, with five different genes exhibiting a significant de novo mutation burden. In all, rare, damaging mutations with large effect contributed to ~22% of sporadic CH cases. Multiple CH genes are key regulators of neural stem cell biology and converge in human transcriptional networks and cell types pertinent for fetal neuro-gliogenesis. These data implicate genetic disruption of early brain development, not impaired CSF dynamics, as the primary pathomechanism of a significant number of patients with sporadic CH.
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