Cerebrospinal Fluid Obstruction Research Articles

Diagnosis and Management of Tuberculous Meningitis in Children- an Update

UPDATE ON THE DIAGNOSIS AND MANAGEMENT OF TUBERCULOUS MENINGITIS IN CHILDREN: Ronald van Toorn, Regan Solomons Seminars in Pediatric Neurology Volume 21, Issue 1, March 2014, Pages 12-18 Tuberculous meningitis (TBM), the most devastating manifestation of tuberculosis, is often missed or overlooked because of nonspecific symptoms and difficulties in diagnosis. It continues to be an important cause of neurologic handicap in resource-poor countries. Owing to the suboptimal performance of diagnostic tests of TBM, diagnosis relies on thorough history, clinical examination, and relevant investigations. The development of affordable, accurate diagnostic tests for TBM in resource-poor settings remains a priority. Short intensified treatment is safe and effective in both human immunodeficiency virus (HIV)-infected and HIV-uninfected children. Treatment of tuberculous hydrocephalus depends on the level of the cerebrospinal fluid obstruction. Corticosteroids reduce risk of neurodisability and death in HIV-uninfected children. Thalidomide should be considered in children compromised by tuberculosis abscesses and tuberculous-related optochiasmic arachnoiditis. In resource-poor countries, home-based TBM treatment after initial in-hospital stabilization is feasible in carefully selected patients. Early diagnosis and treatment of TBM is the single most important factor determining outcome.

Intraventricular Neurocysticercosis: Comparative Analysis of Different Localizations. Clinical Course and Treatment. A Systematic Review.

Background Neurocysticercosis is significant due to its high prevalence and considerable morbidity and mortality. The intraventricular form of NCC is less common than parenchymal, may have a rapidly progressive course and it requires a corresponding therapeutic response. Despite the extensive literature dealing with NCC and intraventricular cystic lesions, no systematic reviews have addressed similar work related to the clinical course and treatment of the infestation. Our main objective was to analyze the clinical type of the disease and the management for each ventricle separately on the basis of case reports or series of patients with individual data on the course of the disease and its treatment. As a control group, we used data on signs&symptoms and treatment of patients from published series on intraventricular neurocysticercosis. Method We performed a search in the Medline database. Google Scholar was also randomly searched. We extracted the following data from the eligible case/series: age and gender, symptoms, clinical signs, diagnostic examinations and findings, localization, treatment, follow-up period, outcome, and publication year. All data are presented in the form of absolute and relative numbers. The frequency of signs and symptoms, treatment and outcomes of the observed groups were checked by the Chi-square test and Fisher's test. The hypothesis was tested with p <0.05 as statistical significance. Results We selected 160 cases of intraventricular neurocysticercosis (IVNCC) and divided them according to their localization into five categories. Hydrocephalus was recognized in 134 cases (83.4%). Patients with isolated IVNCCare are younger (P=.0264) and have a higher percentage of vesicular cysts (p <.00001). In mixed IVNCC, degenerative and multiple confluent cysts predominate (p = 0.00068). Individuals carrying fourth + third ventricular cysts (potentially obstructive form) are younger than individuals with lateral ventricles (potentially less obstructive forme) (p = .0083). The majority of patients had individual symptoms for a longer period before the acute onset of the disease (p <.00001). The predominant clinical manifestation is headache (88.7%); the proportion within the groups ranged from 100% to 75% without statistical significance (p.074214). The same was true for patients with symptoms of vomiting or nausea, who had a lower and roughly balanced percentage of 67.7% to 44.4% (p.34702). Altered level of consciousness (ranging from 21% to 60%) and focal neurological deficit (from 51.2% to 15%) are the only clinical category with statistical significance (p <0.001 and p.023948 ). Other signs and symptoms were less frequent and statistically irrelevant. Surgical resection of the parasite was the predominant type of treatment, varying from 55.5% to 87.5% (p- .02395); endoscopy (48.2%) and craniotomy (24.4%), each individually, showed statistical significance (.00001 and .000073, respectively). The difference was also relevant among patients who had CSF diversion performance with/without medical treatment (p-.002312. Postoperatively, 31.8% of patients received anthelmintics with/without anti-inflammatory or other drugs. Endoscopy, open surgery, and postoperative antiparasitic therapy showed statistical differences (p < 0.001). Favorable outcomes or regression of symptoms were recorded in 83.7%, mortality 7.5%. In the case series, the clinical signs&symptoms were as follows: headache-64%, nausea and vomiting 48.4%, focal neurological deficit 33.6% and altered level of consciousness 25%. Open surgery was the predominant form of intervention (craniotomy (57.6% or endoscopy 31.8%); with statistical significance between them (p< .00001). Conclusion. Ventricular neurocysticercosis is an alarming clinical condition. Hydrocephalus is the dominant diagnostic sign. Isolated IVNCC patients were recognized at a younger age than Mix.IVNCC individuals; poeple with cysts in the fourth and third ventricles (as a potentially more occlusive type of disease), presented their symptoms at a younger age than individuals with LVNCC. The majority of patients had long-term signs and symptoms before the acute onset of the disease. Headache, nausea& vomiting are the most common symptoms of infestation accompanied by altered sensorium and focal neurological deficits. Surgery is the best treatment option. A sudden increase in ICP due to cerebrospinal fluid obstruction with a successive cerebral hernia is the leading cause of fatal outcomes.

Cerebrospinal Fluid Flow and Brain Motion in Chiari I Malformation: Past, Present, and Future.

Cranio-spinal volume and pressure changes associated with the cardiac-cycle and respiration are altered in Chiari I malformation (CMI) due to obstruction of cerebrospinal fluid (CSF) flow at the foramen magnum. With the introduction of motion-sensitive MRI sequences, it was envisioned that these could provide noninvasive information about volume-pressure dynamics at the cranio-cervical junction in CMI hitherto available only through invasive pressure measurements. Since the early 1990s, multiple studies have assessed CSF flow and brain motion in CMI. However, differences in design and varied approaches in the presentation of results and conclusions makes it difficult to fully comprehend the role of MR imaging of CSF flow and brain motion in CMI. In this review, a cohesive summary of the current status of MRI assessment of CSF flow and brain motion in CMI is presented. Simplified versions of the results and conclusions of previous studies are presented by dividing the studies in distinct topics: 1) comparing CSF flow and brain motion between healthy subjects (HS) and CMI patients (before and after surgery), 2) comparing CSF flow and brain motion to CMI severity and symptoms, and 3) comparing CSF flow and brain motion in CMI with and without syringomyelia. Finally, we will discuss our vision of the future directions of MR imaging in CMI patients. EVIDENCE LEVEL: 2. TECHNICAL EFFICACY: 5.

A case of successful intraventricular thrombolysis and external ventricular drain treatment in primary intraventricular hemorrhage

Intraventricular hemorrhage (IVH), accounting for about 45% of acute spontaneous intracranial hemorrhage (ICH), is often associated with obstructive hydrocephalus and poor functional outcomes [1] [2]. Obstructive hydrocephalus and increased intracranial pressure are common after IVH because of an obstruction of normal cerebrospinal fluid (CSF) flow and absorption by blood clots. An external ventricular drain (EVD) - a temporary system that permits drainage of cerebral spinal fluid (CSF) from the ventricles to an external closed system) has been indicated for patients with IVH with hydrocephalus and neurologic decline [3]. However, the treatment with EVD alone is often not sufficiently effective due to a likely potential obstruction of the catheter by blood clots. Intraventricular fibrinolysis (IVF) via the extra-ventricular drain has been known to speed clot resolution and maintain the EVD functionality [4]. However, it is also possible that the risk of hemorrhage, infection, and CFS pleocytosis may be increased with IVF therapy, but these have not been demonstrated so far. We report a clinical case of primary IVH with acute obstructive hydrocephalus, successfully treated with a combination of EVD and direct intraventricular injection of rt-PA into the lateral ventricles. This treatment contributes to confirm that combination of EVD and thrombolysis could be considered as a treatment option for selected patients.

In vitro and in vivo assessment of a novel ultra-flexible ventriculoamniotic shunt for treating fetal hydrocephalus.

Fetal aqueductal stenosis (AS) is one of the most common causes of congenital hydrocephalus, which increases intracranial pressure due to partial or complete obstruction of cerebrospinal fluid (CSF) flow within the ventricular system. Approximately 2-4 infants per 10,000 births develop AS, which leads to progressive hydrocephalus, which enlarges the head often necessitating delivery by cesarean section. Most babies born with AS are severely neurologically impaired and experience a lifetime of disability. Therefore, a new device technology for venticuloamniotic shunting is urgently needed and has been studied to ameliorate or prevent fetal hydrocephalus development, which can provide a significant impact on patients and their family's quality of life and on the decrease of the healthcare dollars spent for the treatment. This study has successfully validated the design of shunt devices and demonstrated the mechanical performance and valve functions. A functional prototype shunt has been fabricated and subsequently used in multiple in vitro tests to demonstrate the performance of this newly developed ventriculoamniotic shunt. The shunt contains a main silicone-nitinol composite tube, a superelastic 90° angled dual dumbbell anchor, and an ePTFE valve encased by a stainless-steel cage. The anchor will change its diameter from 1.15mm (collapsed state) to 2.75mm (deployed state) showing up to 1.4-fold diameter change in human body temperature. Flow rates in shunts were quantified to demonstrate the valve function in low flow rates mimicking the fetal hydrocephalus condition showing "no backflow" for the valved shunt while there is up to 15mL/h flow through the shunt with pressure difference of 20Pa. In vivo ovine study results show the initial successful device delivery and flow drainage with sheep model.

Postural control in Chiari I malformation: protocol for a paediatric prospective, observational cohort - potential role of posturography for surgical indication.

IntroductionChiari I malformation (CM1) is an anatomical abnormality characterised by the cerebellar tonsils descending at least 5 mm below the foramen magnum. CM1 causes obstruction of cerebrospinal fluid (CSF) circulation as well as direct compression on the brainstem, thus causing typical consequences (syringomyelia), and typical clinical features (characteristic headaches and neurological impairment). Surgery is the only available treatment, indicated when symptomatology is present. However, sometimes patients have atypical complaints, which are often suggestive of otolaryngological (ears, nose and throat, ENT) involvement. This may be difficult for a neurosurgeon to explain. Our study aims to investigate the relationship between one of these atypical symptoms, for example, postural instability, in a paediatric population using a Computerised Dynamic Posturography (Equitest, NeuroCom, Clackamas, OR). To our knowledge, there are no previously published studies carried out on children with CM1, using dynamic posturography.Methods and analysisForty-five children aged 6–18 years old presenting with radiologically confirmed CM1 and presenting ENT clinical complaints will be included in the study for a duration of 3 years. As primary endpoint, posturographic results will be described in the population study. Second, posturographic results will be compared between patients with and without indication for surgery. Finally, preoperative and postoperative posturographic results, as well as CSF circulation quality at foramen magnum level, syringomyelia, sleep apnoea syndrome, scoliosis and behaviour will be compared in the operated patient group.Ethics and disseminationThis protocol received ethical approval from the Clinical Research Delegation of Nancy University Hospital, in accordance with the National Commission on Informatics and Liberties (Commission Nationale de l'Informatique et des Libertés) (protocol number 2019PI256-107). Our data treatment was in accordance with the Methodology of reference Methodology Reference-004 specification for data policy. The study findings will be disseminated via peer-reviewed publications and conference presentations, especially to the Neurosphynx’s rare disease healthcare network.Trial registration numberNCT04679792; Pre-results.

Predicting Ventriculoperitoneal Shunt Dependence in High Grade Aneurysmal Subarachnoid Hemorrhage.

Aneurysmal subarachnoid hemorrhage (aSAH) commonly presents with hydrocephalus due to obstruction of cerebrospinal fluid (CSF) passage across the ventricular system in the brain. Placement of an external ventricular device (EVD) and in some cases ventriculoperitoneal shunt (VPS) are often necessary for patients requiring prolonged CSF diversion. The study aimed at evaluating critical factors that play a role in determining the need for extended extraventricular drainage. We performed a retrospective observational cohort study of two groups of patients with radiological imaging confirmed high grade aSAH (Hunt & Hess grades 3-5) who required VPS placement, shunt-dependent group, and who did not require long term CSF diversion, non-shunt-dependent group. We collected and analyzed data regarding the daily CSF output for 10 days following EVD placement, daily EVD height, intracranial pressure (ICP) and cerebral perfusion pressure (CPP), indicators of hydrocephalus, and CSF characteristics. The cohort, comprising of 8 patients in the shunt-dependent group and 32 patients in the non-shunt-dependent group, displayed median daily CSF output of 275.1 mL/day and 193.4 mL/day, respectively (P = .0005). ROC curve for CSF drainage for the two groups showed an area under the curve (AUC) of 0.71 with a 95% confidence interval (CI) 0.65 to 0.77. Qualitative analysis of CSF characteristics revealed that the shunt-dependent group had more proteinaceous, darker red color, and greater proportion of red blood cells (RBCs) although not statistically significant. Determinants of prolonged CSF drainage requirements in patients with high grade aSAH are not fully elucidated to this date and there is no standardized protocol for CSF diversion. Our study revealed potential markers that can be used in the assessment for the need for long term CSF diversion. Our limited sample size necessitates further research to establish clear correlations and cutoffs of these parameters in predicting long term CSF diversion requirements.

Molecular Mechanisms and Risk Factors for the Pathogenesis of Hydrocephalus.

Hydrocephalus is a neurological condition due to the aberrant circulation and/or obstruction of cerebrospinal fluid (CSF) flow with consequent enlargement of cerebral ventricular cavities. However, it is noticed that a lot of patients may still go through symptomatic progression despite standard shunting procedures, suggesting that hydrocephalus is far more complicated than a simple CSF circulative/obstructive disorder. Growing evidence indicates that genetic factors play a fundamental role in the pathogenesis of some hydrocephalus. Although the genetic research of hydrocephalus in humans is limited, many genetic loci of hydrocephalus have been defined in animal models. In general, the molecular abnormalities involved in the pathogenesis of hydrocephalus include brain development and ependymal cell dysfunction, apoptosis, inflammation, free radical generation, blood flow, and cerebral metabolism. Moreover, recent studies have indicated that the molecular abnormalities relevant to aberrant cerebral glymphatic drainage turn into an attractive subject in the CSF circulation disorder. Furthermore, the prevalent risk factors could facilitate the development of hydrocephalus. In this review, we elicited some possible fundamental molecular mechanisms and facilitating risk factors involved in the pathogenesis of hydrocephalus, and aimed to widen the diagnosis and therapeutic strategies for hydrocephalus management. Such knowledge could be used to improve patient care in different ways, such as early precise diagnosis and effective therapeutic regimens.

Tumor-related hydrocephalus in infants: a narrative review.

Primary brain tumors diagnosed during infancy present several challenges for the pediatric neurosurgeon including the management of tumor-related hydrocephalus. Infant tumor-related hydrocephalus (iTRH) is present in over 70% of new diagnoses overall and in over 90% of infants with infratentorial neoplasms. iTRH may be related to direct obstruction of cerebrospinal fluid (CSF) pathways, shedding of protein and/or cells leading to dysfunctional subarachnoid, tumor-related CSF production, or, most frequently, a combination of these mechanisms. Treatment plans must consider the acuity of presentation, the mechanism of iTRH, and the additional neoadjuvant or adjuvant therapies required to treat the neoplasm. This narrative review provides a broad reference on the epidemiology, pathophysiology, and treatment of iTRH.

Migrating Lateral Ventricle Choroid Plexus Cyst into the Fourth Ventricle Causing Acute Hydrocephalus

AbstractChoroid plexus cysts consist of abnormal folds of the choroid plexus that typically resolve prior to birth. Rarely, these cysts persist and may cause outflow obstruction of cerebrospinal fluid. In this article, we presented a 5-month-old male child patient who presented with lethargy, vomiting, and a bulging anterior fontanelle. Magnetic resonance imaging showed one large choroid plexus cyst had migrated from the right lateral ventricle through the third ventricle and cerebral aqueduct into the fourth ventricle causing outflow obstruction. The cyst was attached to the lateral ventricle choroid plexus by a pedicle. The cyst was endoscopically retrieved from the fourth ventricle intact and then fenestrated and coagulated along with several other smaller cysts. Histologic examination confirmed the mass was a choroid plexus cyst. The patient did well after surgery and did not require any cerebrospinal fluid diversion. Nine months after surgery, the patient continued to thrive with no neurological deficits. This case is the first we have found in the literature of a lateral ventricular choroid plexus cyst migrating into the fourth ventricle and the youngest of any migrating choroid plexus cyst. Only three other cases of a migrating choroid plexus cyst have been documented and those only migrated into the third ventricle. New imaging advances are making these cysts easier to identify, but may still be missed on routine sequences. High clinical suspicion for these cysts is necessary for correct treatment of this possible cause of hydrocephalus.

An overview of leptomeningeal disease.

Leptomeningeal disease (LMD) is a poor prognosis pattern of disease progression in patients with metastatic malignancy with limited treatment options. Patients may be asymptomatic or present with non-specific neurologic deficits, therefore gadolinium-enhanced magnetic resonance imaging of the brain and spine is critical for establishing a diagnosis. Although the treatment intent is palliative in the context of LMD, a multidisciplinary approach is still important to ensure patients receive a timely diagnosis and appropriate treatment to maximize symptom control and preserve quality of life. Radiotherapy is typically delivered to the whole brain or focal spinal regions for the purposes of treating bulky disease, stabilizing symptoms, or relieving cerebrospinal fluid obstruction. Whole craniospinal irradiation (CSI) is generally avoided given its toxicity profile and should only be considered in carefully selected patients where the potential benefit may outweigh the adverse effects. CSI with proton radiotherapy (oppose to conventional photon radiotherapy) has shown promise with improved toxicity for patients with primary CNS tumors. This may be a preferred option for patients being considered for CSI at centres with the proton therapy capabilities. Focal hypofractionated stereotactic radiotherapy (SRT) to intracranial targets is an emerging approach to LMD that may be useful in select patients with limited disease particularly in the setting of reirradiation. Chemotherapies may be delivered intrathecally, although the evidence supporting its efficacy is limited and heterogeneous in regards to the tumor sites examined. Finally, targeted therapy and novel applications of immune checkpoint inhibitors are promising; however, further research is required to guide the use of these agents.

Intradural Pathology Causing Cerebrospinal Fluid Obstruction in Syringomyelia and Effectiveness of Foramen Magnum and Foramen of Magendie Dredging Treatment

This article discusses the procedure of foramina magnum and Magendie dredging, summarizing the pathologic changes in the intradural region of the craniocervical junction in patients with syringomyelia and the pathophysiologic mechanism of cerebrospinal fluid (CSF) circulation obstruction. Clinical data from 50 adult patients with syringomyelia treated at Xuanwu Hospital from July 2018 to January 2019 were collected and retrospectively analyzed. All operations were performed with foramina magnum and Magendie dredging, and all intradural factors that may have induced the obstruction of CSF circulation were recorded. Intradural pathology was found in all patients. The pathologic changes that may have caused obstruction of the CSF circulation include tonsil occupying the foramen magnum and overlying foramen of Magendie in 88% (44/50), intertonsillar arachnoid adhesions in 36% (18/50), tonsil to medulla arachnoid adhesions in 18% (9/50), medialized tonsils in 70% (35/50), vermian branch of posterior inferior cerebellar artery in 22% (11/50), arachnoid veil in 16% (8/50), cisterna magna cyst in 4% (2/50), and tonsil to dura mater arachnoid adhesions in 8% (4/50). Mean duration of follow-up was 13.3 months. The long-term effective rate was 96.0%. Postoperative magnetic resonance imaging revealed that the size of the syringomyelia was reduced or completely resolved in 88% of patients. The mean preoperative Japanese Orthopaedic Association score was 12.9 ± 3.1, which improved to 14.7 ± 3.2 (P < 0.05) at last clinical follow-up. Intradural pathology that causes CSF circulation obstruction exists in many forms. Relieving the obstruction of the foramen magnum and foramen of Magendie is key to surgical treatment.

Classification of Chiari I malformation based on various cerebrospinal fluid flow patterns at the cranial-vertebral junction and selection of surgical procedure

Objective To discuss about the classification of Chiari Ⅰ malformation based on different CSF (cerebrospinal fluid) flow patterns at the cranial-vertebral junction (CVJ) and to investigate its surgical strategies and outcomes. Methods A total of 126 patients with Chiari Ⅰ malformation were admitted to Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University from January 2008 to December 2015 and retrospectively enrolled into this study. According to the preoperative findings obtained by using cine phase-contrast MRI (cine PC-MRI) and based on the abnormal CSF flow dynamics at the CVJ, all those patients of Chiari Ⅰ malformation were classified into 3 patterns: type Ⅰ (36 cases), type Ⅱ (48 cases) and type Ⅲ (42 cases). Intraoperative ultrasound to detect cerebrospinal fluid again determined the surgical strategy. In type Ⅰ patients, 34 patients underwent subdural decompression, and the remaining 2 patients underwent subarachnoid decompression due to cerebrospinal fluid obstruction. In type Ⅱ patients, 36 patients underwent subarachnoid decompression and the remaining 12 cases underwent subdural decompression. In type Ⅲ patients, 40 cases underwent subarachnoid decompression, and the remaining 2 cases underwent subdural decompression due to unobstructed cerebrospinal fluid flow. Two weeks after surgery, the patients underwent an imaging review and assessment based on cervical spine modified Japanese Orthopaedic Association (mJOA) score. All patients underwent clinical follow-up. The follow-up included the use of the cervical spine mJOA score to assess the improvement of syringomyelia-related symptoms. The Chicago Chiari Outcome Scale (CCOS) was used to assess postoperative neurological recovery. Results All 126 patients had successful surgery. There were no cases of new neurological deficits after operation. Eight cases (6.3%) had fever, and 5 cases (4.0%) had cerebrospinal fluid leakage, which improved after symptomatic treatment. Two weeks after surgery, imaging examination showed that there was no significant improvement in syringomyelia in 2 patients (type Ⅲ, subarachnoid decompression). Further improvement of symptoms was observed after syringomyelia-thoracic shunt. The mJOA score was improved compared with preoperative conditions (12.7±2.0 vs. 10.7±1.6, P<0.01). The follow-up time of 126 patients was 24.8 ± 10.9 months (12 to 96 months). At the last follow-up, the cervial spine mJOA scores of 126 patients were significantly higher than those before surgery (12.8±1.9 vs. 10.7±1.6, P<0.01), but there was no significant difference when compared with two weeks after surgery (P=0.48). There was no significant difference in CCOS score compared with 2 weeks post surgery (14.7±1.5 vs. 14.4±1.5, P=0.576). Conclusions Chiari malformation type Ⅰ could be classified into 3 subtypes according to cerebrospinal fluid dynamic abnormalities in the cranial-cervical junction area. Type Ⅰ is suitable for subdural decompression and type Ⅲ for subarachnoid decompression. In type Ⅱ, specific decompression methods could be chosen based on intraoperative ultrasound through bone window after craniectomy. The postoperative results seem good. Key words: Arnold-Chiari malformation; Hydrodynamics; Neurosurgical procedures; Treatment outcome; Cerebrospinal fluid; Typing

Lumber Puncture Shortfalls in Dementia Workup

Objectives: To extract from germane literature methodological shortfalls in obtaining cerebrospinal fluid specimens by lumbar puncture. Methods: 20 peer reviewed articles were analyzed in which cerebrospinal fluid (CSF) obtained biomarkers were used to assess Alzheimer’s Disease. Binary assessment of eight variables was performed: albumin, total protein, CSF pressure, cephalad CSF obstruction, obtained but unreported data, inclusion of erythrocyte contaminated samples, exclusion of erythrocyte contaminated samples, exclusion of white blood cell contaminated samples, and failed lumbar puncture attempts. Results: An apparent shortfall exists in the assessment of contravening variables, which could alter CSF results. Conclusions: We suggest that more attention be placed on lumbar puncture methodological difficulties in obtained CSF specimens, and that all practical obtainable data be routinely reported.

Neonatal Hydrocephalus

Hydrocephalus is one of the most common congenital abnormalities affecting the nervous system, occurring in 0.3 to 2.5 per 1,000 live births. It results from obstruction of cerebrospinal fluid (CSF) pathways by a diverse range of developmental, genetic, and acquired abnormalities and can have negative consequences on the neurodevelopmental outcome of affected neonates. Historically, hydrocephalus was diagnosed after birth and managed with a shunt procedure; however, with the advent of advanced antenatal imaging techniques, it may now be detected and treated before delivery in some individuals. Moreover, surgical options for the treatment of hydrocephalus have increased over the past few decades, and temporary CSF diversion may prevent the need for permanent shunt placement. Posthemorrhagic hydrocephalus is the most common cause of hydrocephalus in the preterm newborn, but the timing of surgical intervention for this condition remains controversial. Recent evidence suggests that earlier, rather than later, intervention may have some benefits, but more data are needed to adequately inform clinical practice. Regardless of etiology or timing of diagnosis, a thorough understanding of the natural history of hydrocephalus and the range of treatment options available is needed for parental counseling, prognostication, and appropriate surgical management.

Treatment of Syringomyelia in Patients with Arachnoiditis at the Craniocervical Junction.

Craniocervical junction arachnoiditis (CCJA) is an uncommon cause of syringomyelia. The pathophysiology of syrinx formation is uncertain, and the appropriate management unclear. A series of cases is reported to demonstrate variations in etiology, uniformity of functional cerebrospinal fluid obstruction at the foramen magnum, and results of surgical intervention. We retrospectively analyzed the clinical and radiologic features of a consecutive series of patients treated for syringomyelia related to CCJA. Eight patients (5 male, 28-66 years old) were treated from 2000 to 2016. Magnetic resonance imaging demonstrated cervicothoracic syringomyelia in all cases, with the rostral extension of the syrinx suggesting communication with the fourth ventricle in all but one case. There was reduction of foramen magnum cerebrospinal fluid space in all cases, cerebellar ectopia in 5 cases, and fourth ventricular entrapment in 3 cases. Treatment consisted of posterior fossa decompression with either a GoreTex or pericranial patch graft. Six patients had a fourth-ventricle spinal subarachnoid shunt. Two patients had titanium mesh cranioplasty. The immediate postoperative period was associated with reduction in syrinx cavity size and improvement in neurologic symptoms in all cases. At follow-up 10-60 months postoperatively, 3 patients exhibited recurrence of the syrinx and underwent successful reoperation at the craniocervical junction. One patient with persistence of the inferior component of the syrinx was treated with a syrinx-spinal subarachnoid shunt. Most syrinx cavities associated with CCJA communicate with the fourth ventricle. Posterior fossa decompression and fourth ventricle to spinal subarachnoid space shunting appears a reasonable treatment for this form of syringomyelia.

Chiari malformation type 1-related headache: the importance of a multidisciplinary study.

Chiari type 1 Malformation (CM 1) is a structural defect consisting of a displacement of the cerebellar tonsils through the foramen magnum causing obstruction of cerebrospinal fluid (CSF) outflow. CM 1 has a variety of presentation with headache being the most common symptom. The evaluation and treatment of headache related to CM 1 are often difficult, because the pain in the occipital-suboccipital region or headache that is of cough-type suggests symptomatic CM 1, but patients suffering from CM 1 can also report migraine or tension-type headache. In 2015 we started a collaborative project in which our group of neurologists, neurosurgeons and neuroradiologists contribute to create a Chiari Special Outpatient Service; this was set up to provide a multidisciplinary evaluation, treatment and follow-up of patient suffering from CM 1. 201 patients (58 males, 143 females) suffering from CM 1 were multidisciplinary evaluated. Headache characteristics, clinical features, and treatment of patients are discussed. Further progress in multidisciplinary care of headache and CM 1 should be performed to define guidelines.

Imaging of Intracranial Pressure Disorders.

Intracranial pressure (ICP) is the pressure inside the bony calvarium and can be affected by a variety of processes, such as intracranial masses and edema, obstruction or leakage of cerebrospinal fluid, and obstruction of venous outflow. This review focuses on the imaging of 2 important but less well understood ICP disorders: idiopathic intracranial hypertension and spontaneous intracranial hypotension. Both of these ICP disorders have salient imaging findings that are important to recognize to help prevent their misdiagnosis from other common neurological disorders.

Spinal Intradural Arachnoid Webs Causing Spinal Cord Compression with Inconclusive Preoperative Imaging: A Report of 3 Cases and a Review of the Literature

Spinal arachnoid webs are a rare variant of spinal arachnoid cysts where 1 or multiple focal membranes of arachnoid tissue obstruct the subarachnoid space. Only 11 prior cases of arachnoid webs have been reported in the literature. We present a series of 3 consecutive cases of arachnoid webs from our institution and review the literature on this rare condition to provide recommendations for its management. Retrospective chart review was performed for 3 consecutive cases of intradural arachnoid webs causing spinal cord compression at our institution, with inconclusive preoperative imaging, treatment with surgical decompression, and resection. There were no external sources of funding. Our cases occurred dorsally in the thoracic spine and were associated with syringomyelia. Preoperative magnetic resonance imaging, computed tomography myelography, and cine magnetic resonance imaging were inconclusive, and the definitive diagnosis was made with intraoperative ultrasound. Patients underwent laminectomies and resection of arachnoid webs. Unique from prior reports of arachnoid webs, the webs in the present cases were composed of multiple septated longitudinal membranes rather than a transverse band. All patients had improvement of presenting symptoms postoperatively. Intradural arachnoid webs causing spinal cord compression are rare. Preoperative imaging may be inconclusive. Because of the septated longitudinal nature of the visualized membranes, we propose a 1-way valve mechanism of cerebrospinal fluid obstruction causing gradual cord compression and resultant syringomyelia.

Sustained high-pressure in the spinal subarachnoid space while arterial expansion is low may be linked to syrinx development

Syringomyelia (a spinal cord cyst) usually develops as a result of conditions that cause cerebrospinal fluid (CSF) obstruction. The mechanism of syrinx formation and enlargement remains unclear, though previous studies suggest that the fluid enters via the perivascular spaces (PVS) of the penetrating arteries of the spinal cord, and that alterations in the CSF pulse timing and pressure could contribute to enhanced PVS inflow. This study uses an idealised computational model of the PVS to investigate the factors that influence peri-arterial fluid flow. First, we used three sample patient-specific models to explore whether changes in subarachnoid space (SAS) pressures in individuals with and without syringomyelia could influence PVS inflow. Second we conducted a parametric study to determine how features of the CSF pulse altered perivascular fluid, including alterations to timing and magnitude of the peak SAS pressure, the timing of reversal from high to low pressure (diastolic phase), and the area under the pressure–time curve. The model for the patient with syringomyelia had higher net CSF inflow to the PVS than the two subjects without syringomyelia. In the parametric study, only increasing the area under the high pressure region of the SAS pulse substantially increased PVS inflow, when coupled with a temporal shift in arterial and SAS pulses. This suggests that a period of sustained high SAS pressure while arterial diameter is low may increase net CSF pumping into the PVS.