Search Results (61)

Idiopathic intracranial hypertension (IIH) with (IIHWP) and without papilledema (IIHWOP) is characterized by increased cerebrospinal fluid (CSF) pressure and no evident cause, mostly affecting obese women of childbearing age and possibly leading to vision loss. However, in neonates, infants, and toddlers, these conditions remain understudied entities. This review investigates clinical features, risk factors, treatments, and outcomes to inform their care. From 2278 publications found in PubMed, 2974 in Scopus, and 1684 in the Web of Science Core Collection, 104 relevant articles were analyzed. Among 300 cases, 48.3% were male and 26.0% female, with 43.0% meeting the modified Dandy criteria. Typical signs and symptoms, besides papilledema (23.0%) or its absence (49.0%), included bulging fontanelle (67.7%), irritability (34.3%), vomiting (33.0%), and fever (18.3%). The most triggering factors were medications (35.3%) and infections (15.0%). The mean CSF opening pressure was 35.1 cm H₂O, ranging from 9.5 to 77 cm H₂O. Main treatment options were lumbar punctures (72.7%), discontinuation of triggering medications (26.3%), acetazolamide (18.7%), and corticosteroids (7.7%); 3.0% required shunting. Unlike in adults, males were more commonly affected, and papilledema was less frequent. Most cases resolved with conservative treatment. A nosological distinction between IIHWP and IIHWOP seems unlikely. Considering our findings and age-specific CSF pressure limits, new diagnostic criteria are proposed. Full article

Background/Objectives: Idiopathic normal-pressure hydrocephalus (iNPH) is a potentially reversible neurological disorder characterized by gait disturbance, cognitive impairment, and urinary incontinence. Its pathophysiology involves impaired cerebrospinal fluid (CSF) absorption, and recent research has highlighted the role of the glymphatic and meningeal lymphatic systems in this process. However, the factors that trigger the clinical manifestations of iNPH in subclinical cases remain poorly understood. Case Presentation: Herein, we report two rare cases of iNPH in which clinical symptoms only became apparent following systemic infections. An 82-year-old man presented with transient neurological deficits during a course of sepsis caused by Klebsiella pneumoniae. Neuroimaging revealed periventricular changes and mild ventricular enlargement. Shunting and a tap test led to significant improvements to both his gait and cognition. An 80-year-old man with a history of progressive gait disturbance and cognitive decline developed worsening urinary incontinence and acute cerebral infarction caused by Staphylococcus haemolyticus bacteremia. Magnetic resonance imaging revealed a ventriculomegaly with features of disproportionally enlarged subarachnoid space hydrocephalus and a corona radiata infarct. Clinical improvement was achieved after a ventriculoperitoneal shunt was placed. Conclusions: Our two present cases suggest that systemic inflammatory states may act as catalysts for the manifestation of iNPH in patients with predisposing cerebral ischemia or subclinical abnormalities in CSF flow, highlighting the need for higher clinical awareness of iNPH in older patients who present with neurological deterioration during systemic infections. Early diagnosis and timely shunting after appropriate infection control may facilitate significant functional recovery in such patients. Full article

Background: Idiopathic normal pressure hydrocephalus (iNPH) is a progressive neurological disorder characterized by cognitive decline, gait disturbances, and urinary incontinence. Surgical interventions such as ventriculoperitoneal shunt (VPS) and endoscopic third ventriculostomy (ETV) are the primary treatment options. While VPS is the standard of care, ETV offers a minimally invasive alternative with potentially fewer complications. However, comparative evidence regarding their impact on cognitive, motor, and structural outcomes remains limited. This study, titled ENVENTOR-iNPH (endoscopic ventriculostomy versus shunt on neuropsychological and motor performance in patients with iNPH), aims to address this gap through a rigorously designed comparative protocol. Methods: This protocol is designed as a multicenter, randomized, controlled trial (ENVENTOR-iNPH) to compare the effects of ETV and VPS in patients diagnosed with iNPH. The study will enroll 100 patients aged 60 years or older, randomly assigned to undergo ETV (n = 50) or VPS (n = 50). Preoperative and postoperative evaluations will include comprehensive cognitive and motor assessments, standardized quality-of-life instruments, and advanced neuroimaging techniques such as MRI with flowmetry and diffusion tensor imaging (DTI). Functional outcomes will also be evaluated using navigated transcranial magnetic stimulation (nTMS) and wearable motion analysis systems. The objective of this study is to compare the efficacy and safety of ETV versus VPS in restoring cognitive and motor performance in patients with iNPH. Results: Primary outcomes include cognitive and motor function improvements. Secondary endpoints are surgical complications, hospital stay duration, and changes in quality of life. Neuroimaging will assess changes in white matter integrity and cerebrospinal fluid dynamics, while nTMS will provide insights into neuroplasticity and motor pathway recovery. ETV is hypothesized to demonstrate clinical outcomes comparable or superior to VPS, particularly in terms of complication reduction and hospital recovery metrics. Conclusions: The ENVENTOR-iNPH protocol establishes the framework for a comprehensive, multicenter study comparing ETV and VPS in iNPH patients. The findings from this initial study will inform the design of larger-scale multicenter trials, guide clinical decision making, and potentially position ETV as a preferred treatment option for eligible patients. Full article

Introduction: The presence of syringomyelia associated with Dandy-Walker malformation is rarely described in adults. Case report: We report a case of a 28-year-old woman with a history of Dandy-Walker malformation who developed syringomyelia. She had been previously treated in childhood with a ventriculoperitoneal and cystoperitoneal shunt for hydrocephalus, but over time she developed progressive neurological symptoms, including numbness and weakness in the upper extremities. Magnetic resonance imaging revealed a syrinx extending from C4 to T1 associated with large posterior fossa cyst. The patient was treated with cyst fenestration and cystoperitoneal shunts were removed, with complete resolution of symptoms and disappearance of syrinx. Discussion: A literature review revealed only 6 cases of syringomyelia associated with Dandy-Walker malformation in adults. The pathophysiology of this entity is multifactorial and may be related to obstructed cerebrospinal fluid flow, altered pressure dynamics, and formation of arachnoid adhesions. Conclusions: Individualized surgical approaches are essential for optimizing outcomes in this rare condition. Further research is needed to standardize treatment protocols and clarify underlying mechanisms and help to improve the management of these patients. Full article

Background: Leptomeningeal disease (LMD) from cancer indicates advanced cancer and can lead to obstructive hydrocephalus, for which palliative cerebrospinal fluid (CSF) diversion may be indicated to alleviate symptoms. We investigated surgical outcomes for hydrocephalus for adult patients with LMD and conducted a systematic review on pediatric and adult cases. Methods: We analyzed outcomes from a 10-year period of patients with neoplastic LMD, obstructive hydrocephalus, and documented date of death. We also searched databases from inception until 20 August 2022, using search terms including ‘cancer’, ‘hydrocephalus’, and ‘shunt’. Preferred reporting items for systematic reviews and meta-analyses guidelines were followed. Results: Among 50 patients, 30 (60%) underwent CSF diversion after LMD diagnosis with comparable median age in both the surgery (58.4 ± 14.4 years) and non-surgery (57.8 ± 14.5 years) groups. Twenty-three patients (76.7%) achieved symptom relief. The surgery group lived longer after LMD diagnosis than the non-surgery group (6.6 ± 6.0 vs. 1.3 ± 4.3 months, p < 0.001) and had a higher likelihood of survival (hazard ratio 2.49, 95% confidence interval 1.37–4.52, p = 0.002). Median survival after surgery was 2.8 ± 3.8 months. From 23 articles, 995 patients (34.3%) presented with LMD. Complication rates were 0–37.7% with no extraneural metastases. Symptom relief rates were 50–100%. Three studies reported median survival after surgery (2–3.3 months). Conclusions: A rare entity with dismal prognosis, LMD can present as symptomatic hydrocephalus in patients with cancer. Symptom relief and improved survival can be achieved with palliative CSF diversion with low complication rates. Prospective studies are needed to assess the outcomes and needs of these patients. Full article

Objective: The objective of this study was to evaluate the demographic, clinical, laboratory, and microbiological features of ventriculoperitoneal shunt (VPS) infections through a 13-year retrospective study. VPS bacterial agents and their antibiotic susceptibility were also investigated through the occurrence of single VPS (SVPS) and recurrent VPS (RVPS) infections. Methods: This study included 110 patients with SVPS infections and 55 patients with RVPS infections. Results: In patients who developed multiple infections, Gram-negative organisms were found to be the most predominant (60%, 54/90). The resistance rates were 85.2% for third-generation cephalosporins (3GCs), 83.3% for piperacillin–tazobactam, and 10.4% for carbapenem. Of the patients in the SVPS infection group, 49% were treated with combinations with carbapenem; of the patients in the RVPS infection group, 84.4% were treated in the same way. Central nervous system (CNS) tuberculosis as the etiology of hydrocephalus; short duration of antibiotics used for treatment; high cerebrospinal fluid (CSF) protein and blood C-reactive protein (CRP) levels; and prolonged use of prophylactic antibiotics were found to be related to an increased rate of recurrent infection occurrence. A two-stage shunt change approach decreased the risk of recurrent infections. Conclusions: Based on the findings of our study, it is essential to closely monitor patients with independent risk factors for RVPS infection development, due to the high rates of resistant Gram-negative bacterial growth and the initiation of empirical antimicrobial treatment with glycopeptide plus carbapenem. Certain treatment options, such as 3GCs plus glycopeptide, should be revised based on clinical progress and microbiological culture results. Full article

Background/Objectives: Ventriculoperitoneal shunting is a validated procedure for the treatment of idiopathic normal-pressure hydrocephalus. To select shunt-responsive patients, infusion and tap tests can be used. Only gait is evaluated after the procedure to establish a potential improvement. In this study, we present our Hydro-Real-Time Neuropsychological Testing protocol to assess the feasibility of performing an ultra-fast assessment of patients during the infusion and tap test. Methods: We tested 57 patients during the infusion and tap test to obtain real-time feedback on their cognitive status. Data were obtained immediately before the infusion phase (T0), when the pressure plateau was reached (T1), and immediately after cerebrospinal fluid subtraction (T2). Based on cerebrospinal fluid dynamics, 63.15% of the patients presented a resistance to outflow > 12 mmHg/mL/min, while 88% had a positive tap test response. Results: Compared to T0, cerebrospinal fluid removal significantly improved performance on tasks exploring executive functions (counting backward, p < 0.001; verbal fluency, p < 0.001). Patients were significantly faster at counting backward at T2 vs. T1 (p < 0.05) and at T2 vs. T0 (p < 0.001) and were significantly faster at counting forward at T2 vs. T1 (p < 0.005), suggesting an improvement in speed at T2. There was a significantly smaller index at T1 vs. T0 (p = 0.005) and at T2 vs. T0 (p < 0.001), suggesting a more marked improvement in patients’ executive abilities at T2 and a smaller improvement at T1. Regarding verbal fluency, patients were worse at T1 vs. T0 (p < 0.001) and at T2 vs. T0 (p < 0.001). Conclusions: Patients’ performance can be monitored during the infusion and tap test as significant changes in executive functions are observable. In future, this protocol might help improve patients’ selection for surgery. Full article

(1) Background: Fetal surgery for meningomyelocele (MMC) should reduce the occurrence of Arnold–Chiari malformations, hydrocephalus, the associated need for craniocervical decompression, and the need for cerebrospinal fluid shunt insertion. Fetal surgery should improve ambulatory status. (2) Methods: We used retrospective analysis of the documentation and descriptive statistics to summarize the clinical data and measured MRI parameters. The neurosurgical results are presented as the frequency of findings in percentages and compared with the results of the Management of Myelomeningocele Study (MOMS). (3) Results: A total of eight patients who underwent prenatal correction of MMC between 2016 and 2020 participated. MRI detected Chiari II malformation in all patients during prenatal imaging and in 87.5% of the patients at the age of 12 months. Craniocervical decompression was used in 25% of the patients. Shunt-dependent hydrocephalus occurred in 50% of the cases. In 87.5% of the cases, the functional level exceeded the expected ambulatory status. (4) Conclusions: We present the clinical status of our patients. Analysis of the complete cohort confirmed that prenatal surgery is associated with a reduced occurrence of Chiari malformations and reduced associated occurrence of hydrocephalus. Specific lesion levels are not associated with the need for craniocervical decompression. The results of our study are valuable in prenatal counseling and important for treatment planning. Full article

Background/Objectives: Obstructive hydrocephalus associated with vestibular schwannoma (VS) is the most common in giant VS. Despite tumor removal, some patients may require ongoing ventriculo-peritoneal (VP) surgery. This investigation explores the factors contributing to the requirement for VP surgery following VS surgery in instances of persistent hydrocephalus (HCP). Methods: Volumetric MRI analyses of pre- and postoperative tumor volumes, cerebellum, cerebrum, ventricle system, fourth ventricle, brainstem, and peritumoral edema were conducted using Brainlab Smartbrush and 3D Slicer. The total brain volume was defined as the sum of the cerebrum, cerebellum, and brainstem. ROC analyses were performed to identify the optimum cut-off values of the volumetric data. Results: Permanent cerebrospinal fluid (CSF) diversion after surgery was indicated in 12 patients (12/71; 16.9%). The ratio of baseline volume fraction of brain ventricles to total brain ventricle volume (VTB ratio) was found to predict postoperative VP shunt dependency. The AUC was 0.71 (95% CI: 0.51–0.91), and the optimum threshold value (</≥0.449) yielded a sensitivity and specificity of 67% and 81%, respectively. Multivariable logistic regression analyses of imaging data (pre- and postoperative VS volume, VTB ratio, and extent of resection (%) (EoR)) and patient-specific factors revealed that an increased VTB ratio (≥0.049, OR: 6.2, 95% CI: 1.0–38.0, p = 0.047) and an EoR < 96.4% (OR: 9.1, 95% CI: 1.2–69.3, p = 0.032) were independently associated with postoperative VP shunt dependency. Conclusions: Primary tumor removal remains the best treatment to reduce the risk of postoperative persistent hydrocephalus. However, patients with an increased preoperative VTB ratio are prone to needing postoperative VP shunt surgery and may benefit from perioperative EVD placement. Full article

Neurocysticercosis (NCC) is caused by the larval stage of Taenia solium. This parasitic disease is endemic in many areas of the world and is emerging in Europe. NCC can affect different brain regions, but simultaneous involvement of the parenchymal, subarachnoid, and ventricular regions is rare. We report the case of a 39-year-old woman from Honduras, resident in Rome for 10 years, who presented to the Emergency Department complaining of headaches, transient hemianopsia, and bilateral papilledema. MRI showed a concomitant parenchymal, subarachnoid, and ventricular involvement in the brain. T. solium IgG antibodies were detected in the blood. The etiological diagnosis of NCC was obtained by identifying T. solium in cerebrospinal fluid using Next Generation Sequencing. Endoscopic neurosurgery with the placement of a ventricular shunt and medical long-term anti-parasitic treatment with a cumulative number of 463 days of albendazole and 80 days of praziquantel were performed. A successful 4-year follow-up is reported. NCC is one of the most common parasitic infections of the human CNS, but it is still a neglected tropical disease and is considered to be an emerging disease in Europe. Its diagnosis and clinical management remain a challenge, especially for European clinicians. Full article

This review highlights the need for therapeutic guidelines for syringomyelia associated with tethered cord syndrome (TCS) caused by spinal dysraphism (SD). A comprehensive literature review was conducted, selecting twelve articles to analyze common therapeutic strategies. Surgical cord untethering alone has recently become a preferred treatment, with 45 ± 21.1% of patients experiencing remission or improvement, 47 ± 20.4% unchanged and asymptomatic, and 4 ± 8% worsened. Untethering with direct surgical drainage for the syrinx had better outcomes than untethering alone (78% vs. 45%, p = 0.05). Terminal syringostomy was beneficial for syrinxes extending to the filum terminale but not for asymptomatic small syrinxes with a syrinx index < 0.4. Syrinx shunting was recommended for symptomatic large syrinxes (>2 cm in length and syrinx index > 0.5). Various shunt procedures for syrinxes are still advocated, mainly for refractory syringomyelia in Chiari malformation, posttraumatic cases, SD, or other causes. Personalized surgical methods that address the root cause of syringomyelia, particularly those improving cerebrospinal fluid flow, offer promising results with minimized complications. Ongoing studies are required to enhance management strategies for syringomyelia associated with TCS, optimize patient outcomes, and reduce the risk of recurrent symptoms. Full article

Two patients with CSF shunting systems exhibited symptoms of altered intracranial pressure. Initial neuroimaging led to misinterpretation, but integrating clinical history and follow-up imaging revealed the true diagnosis. In the first case, reduced ventricular size was mistaken for CSF overdrainage, while the actual problem was increased intracranial pressure, as seen in slit ventricle syndrome. In the second case, symptoms attributed to intracranial hypertension were due to CSF overdrainage causing tonsillar displacement and hydrocephalus. Adjusting the spinoperitoneal shunt pressure resolved symptoms and imaging abnormalities. These cases highlight the necessity of correlating clinical presentation with a deep understanding of CSF dynamics in shunt assessments. Full article

The main subject of the current review is a specific subtype of headache, which is related to shunt over-drainage and slit ventricle syndrome, in pediatric patients harboring an implanted shunt device for the management of hydrocephalus. This clinical entity, along with its impairment regarding the quality of life of the affected individuals, is generally underestimated. This is partly due to the absence of universally agreed-upon diagnostic criteria, as well as due to a misunderstanding of the interactions among the implicated pathophysiological mechanisms. A lot of attempts have been performed to propose an integrative model, aiming at the determination of all the offending mechanisms of the shunt over-drainage syndrome, as well as the determination of all the clinical characteristics and related symptomatology that accompany these secondary headaches. This subcategory of headache, named postural dependent headache, can be associated with nausea, vomiting, and/or radiological signs of slim ventricles and/or subdural collections. The ultimate goal of our review is to draw clinicians’ attention, especially that of those that are managing pediatric patients with permanent, long-standing, ventriculoperitoneal, or, less commonly, ventriculoatrial shunts. We attempted to elucidate all clinical and neurological characteristics that are inherently related to this type of headache, as well as to highlight the current management options. This specific subgroup of patients may eventually suffer from severe, intractable headaches, which may negatively impair their quality of daily living. In the absence of any other clinical condition that could be incriminated as the cause of the headache, shunt over-drainage should not be overlooked. On the contrary, it should be seriously taken into consideration, and its management should be added to the therapeutic armamentarium of such cases, which are difficult to be handled. Full article

Hydrocephalus is caused by an overproduction of cerebrospinal fluid (CSF), an obstruction of fluid movement, or improper reabsorption. CSF accumulation in the brain’s ventricles causes ventriculomegaly, increased intracranial pressure, inflammation, and neural cell injury. Hydrocephalus can arise from brain trauma, hemorrhage, infection, tumors, or genetic mutations. Currently, there is no cure for hydrocephalus. Treatments like shunting and endoscopic third ventriculostomies are used, but, unfortunately, these therapeutic approaches require brain surgery and have high failure rates. The choroid plexus epithelium (CPe) is thought to be the major producer of CSF in the brain. It is a polarized epithelium that regulates ion and water movement from a fenestrated capillary exudate to the ventricles. Despite decades of research, control of electrolyte movement in the CPe is still not fully understood. This review discusses important transporters on the CPe, how some of these are regulated, and which of them could be potential targets for hydrocephalus treatment. To advance the development of hydrocephalus treatments, physiologically relevant preclinical models are crucial. This review covers some of the current animal and cell culture methods used to study hydrocephalus and highlights the need to develop standardized preclinical models that are used by multiple investigators in order to replicate critical findings and resolve controversies regarding potential drug targets. Full article

This study introduces an application of innovative medical technology, Photon Counting Computer Tomography (PC CT) with novel detectors, for the assessment of shunt valves. PC CT technology offers enhanced visualization capabilities, especially for small structures, and opens up new possibilities for detailed three-dimensional imaging. Shunt valves are implanted under the skin and redirect excess cerebrospinal fluid, for example, to the abdominal cavity through a catheter. They play a vital role in regulating cerebrospinal fluid drainage in various pathologies, which can lead to hydrocephalus. Accurate imaging of shunt valves is essential to assess the rate of drainage, as their precise adjustment is a requirement for optimal patient care. This study focused on two adjustable shunt valves, the proGAV 2.0^® and M. blue^® (manufactured by Miethke, Potsdam, Germany). A comprehensive comparative analysis of PC CT and traditional X-ray techniques was conducted to explore this cutting-edge technology and it demonstrated that routine PC CT can efficiently assess shunt valves’ adjustments. This technology shows promise in enhancing the accurate management of shunt valves used in settings where head scans are already frequently required, such as in the treatment of hydrocephalus. Full article