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Background: Open cell stents (OC) and closed cell stents (CC) each offer unique advantages and potential drawbacks in the context of idiopathic intracranial hypertension (IIH) treatment. We aim to investigate the safety and efficacy of using OC and CC for IIH.Methods: We conducted a systematic review in PubMed, Embase, and Cochrane Library databases following the PRISMA guidelines. Eligible studies included ≥4 patients with IIH treated by OC or CC. Primary outcomes were headache, visual acuity, and papilledema status before and after the procedure. Additionally, failure rate, minor complications, major complications, and total complications were assessed. Pooled analysis of the OC group and CC group were done separately and then compared.Results: Twenty-four studies were included. Of these, 20 reported on OC and 6 reported on CC. Pooled analysis of failure rate was 8% (4%-12%) in OC and 5% (0%-11%) in CC. For headache improvement rate: 78% (70%-86%) in OC and 81% (66%-69%) in CC. For visual acuity improvement: 78% (65%-92%) in OC and 76% (29%-100%) in CC. For papilledema improvement: 88% (77%-98%) in OC and 82% (67%-98%) in CC. For minor complications: 0% (0%-1%) in OC and 0% (0%-2%) in CC. For major complications: 0% (0%-1%) in OC and 2% (0%-6%) in CC. Total complications: 0% (0%-1%) in OC and 2% (0%-6%) in CC.Conclusion: Low failure and complication rates were found in both OC and CC, with no significant difference between them in effectiveness. The CC showed a slight but significant increase in major and total complications compared to the OC. Additionally, a subtle yet significantly lower failure rate was identified in the CC.

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INTRODUCTION: Idiopathic Intracranial Hypertension (IIH) is a condition characterized by elevated intracranial pressure. Although several mechanisms have been proposed as underlying causes of IIH, no identifiable causative factor has been determined for this condition. Initial treatments focus on weight or CSF reduction, but severe cases may require surgery. This study compares outcomes in IIH patients treated with lumboperitoneal shunts (LPSs) versus ventriculoperitoneal shunts (VPSs). METHODS: This systematic-review and meta-analysis follows Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines and includes studies about VPS and LPS patients, reporting one of the outcomes of interest. We conducted searches on PubMed, Embase, Web of Science, and Cochrane Library. RESULTS: Our analysis involved twelve studies, comprising 5990 patients. The estimated odds ratio (OR) for visual improvement was 0.97 (95% CI 0.26-3.62; I2 = 0%) and for headache improvement was 0.40 (95% CI 0.20-0.81; I2 = 0%), favoring LPS over VPS. Shunt revision analysis revealed an OR of 1.53 (95% CI 0.97-2.41; I2 = 77%). The shunt complications showed an OR of 0.91 (95% CI 0.68-1.22; I2 = 0%). The sub-analyses for shunt failure uncovered an OR of 1.41 (95% CI 0.92-2.18; I2 = 25%) and for shunt infection events an OR of 0.94 (95% CI 0.50-1.75; I2 = 0%). CONCLUSIONS: The interventions showed general equivalence in complications, shunt failure, and other outcomes, but LPS seems to hold an advantage in improving headaches. Substantial heterogeneity highlights the need for more conclusive evidence, emphasizing the crucial role for further studies. The findings underscore the importance of considering a tailored decision between VPS and LPS for the management of IIH patients.

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Se presenta el caso de una paciente adulta joven con antecedente de ovario poliquístico e infección reciente por COVID 19 que inicia con cuadro de astenopia y visión borrosa junto con cefalea, se realiza fondo de ojo con papiledema bilateral, estudios de laboratorio y neuro imagen sin hallazgos positivos, también punción lumbar con presión de apertura elevada por lo que se diagnostica hipertensión intracraneal idiopática con posterior mejoría post punción.

The case of a young adult patient is presented with a history of polycystic ovary and recent infection by COVID 19 that starts with asthenopia and blurred vision along with headache, fundus examination with bilateral papilledema was performed, laboratory and neuroimaging studies without positive findings, also lumbar puncture with elevated opening pressure so idiopathic intracranial hypertension was diagnosed with subsequent post puncture improvement.

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Abstract Idiopathic intracranial hypertension (IIH) is a neurological condition characterized by raised intracranial pressure of unknown etiology with normal cerebrospinal fluid (CSF) composition and no brain lesions. It occurs in pregnant patients at approximately the same frequency as in general population, but obstetric and anesthetic management of the pregnancy and labor remains controversial. In this article we provide a multidisciplinary review of the main aspects of IIH in pregnancy including treatment options, mode of delivery and anesthetic techniques. Additionally, we report three cases of pregnant women diagnosed with IIH between 2012 and 2019 in our institution.

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BACKGROUND: Papilledema is the main ocular finding in patients with idiopathic intracranial hypertension (IIH) although several chorioretinal abnormalities may also occur and contribute to visual loss. The purpose of this paper is to describe two cases of chorioretinal abnormalities associated with idiopathic intracranial hypertension: one with choroidal folds and another with polypoidal choroidal vasculopathy, an extremely unusual ocular complication in the disease. CASE PRESENTATION: Case 1: A 47-year-old woman previous diagnosed with idiopathic intracranial hypertension treated with weight loss and acetazolamide that over the following 6 months had optic disc edema gradually resolved. The patient was follow-up for a period of 10 years and the papilledema disappeared, but choroidal folds remained unchanged. Case 2: A 61-year-old female patient was seen as a follow-up examination of a 5-year history of IIH that presented with papilledema. The patient was asymptomatic but fundoscopy evaluation revealed a yellowish white peripapillary subretinal nodular lesion temporally in OD. Multimodal imaging studies were made, and the patient was diagnosed with a rare and just recent described association of IIH and polypoidal choroidal vasculopathy. CONCLUSION: Papilledema, RNFL and retinal ganglion cell loss are the most common structural complications of IIH, but chorioretinal complications are important findings and should be carefully evaluated in such patients. Awareness of such occurrence and the use of appropriated clinical and multimodal imaging studies are of great importance for its early detection, leading to proper treatment and prevention of further visual loss.

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Background: Intraparenchymal pericatheter cysts (IPCs) are a rare ventriculoperitoneal shunt (VPS) complication, with only a few cases recorded in the literature. Case Description: We report a 22-year-old woman admitted with headache, papilledema, vision loss, and a history of leukemia. Lumbar puncture revealed idiopathic intracranial hypertension (IIH). Three months after VPS implantation, she was readmitted with headache and worsening of visual impairment. CT evidenced a IPC with perilesional edema. Intraoperatively, a shunt revision and cyst drainage were opted for. We present a discussion and literature review on this unique complication of VPS, with emphasis on management. Conclusion: It is important to understand and consider IPCs as complications of VPS surgery, including in adult patients and IIH cases.

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Increased intracranial pressure is the most common cause of papilledema. Multiple etiologies such as cerebral edema, hydrocephalus, space occupying lesions, infection, and idiopathic intracranial hypertension among others should be considered. Imaging plays a critical role in the detection of pathologies that can cause papilledema. MRI with contrast and CE-MRV, in particular, are key for the diagnosis of idiopathic intracranial hypertension. This review will focus in common and infrequent causes of papilledema, the role of imaging in patients with papilledema as well as its potential mimickers.

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Idiopathic intracranial hypertension (IIH) is a neurological condition characterized by raised intracranial pressure of unknown etiology with normal cerebrospinal fluid (CSF) composition and no brain lesions. It occurs in pregnant patients at approximately the same frequency as in general population, but obstetric and anesthetic management of the pregnancy and labor remains controversial. In this article we provide a multidisciplinary review of the main aspects of IIH in pregnancy including treatment options, mode of delivery and anesthetic techniques. Additionally, we report three cases of pregnant women diagnosed with IIH between 2012 and 2019 in our institution.

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BACKGROUND: Intracranial pressure (ICP) monitoring has been variously explored as a diagnostic and therapeutic modality in many pathological conditions leading neurological injury. This monitoring standardly depends on an invasive procedure such as cranial or lumbar catheterization. The gold standard for ICP monitoring is through an intraventricular catheter, but this invasive technique is associated with certain risks such as haemorrhage and infection. (1) Also, it is a high-cost procedure and consequently not available in a variety of underprivileged places and clinical situations in which intracranial hypertension is prevalent (3). An accurate non-invasive and low-priced method to measure elevated ICP would therefore be desirable. Under these circumstances, Brazilian scientists developed a non-invasive method for intracranial pressure monitoring (ICP-NI), which uses an electric resistance extensometer that measures micro deformations of the skull and transforms it into an electrical signal. In this case report, the authors describe a pediatrician patient with the diagnosis of idiopathic intracranial hypertension who was successfully submitted to a lumbar puncture under monitorization with this device. CASE DESCRIPTION: 7 year old girl with progressive symptoms that lead to the diagnosis of idiopathic intracranial hypertension. The patient was submitted to a lumbar punction with continuous non-invasive ICP monitoring. CONCLUSION: Estimating ICP (non-invasive) from LP monitoring (invasive) often reflect inaccurate ICP results, and affects negatively on IIH diagnosis and a non-invasive diagnostic method could reduce the requirement for invasive approaches, improving patient health outcomes.

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Neurological symptoms in COVID-19 patients can also be found in the pediatric population, but they are usually described as mild symptoms. Herein, we described a case series of four pediatric patients with severe and highly heterogeneous central and peripheral nervous system manifestations. The objective was to report neurological manifestations of COVID-19 in children and adolescents. The design is case series. The participants are four children and adolescents with confirmed COVID-19. The main outcome and measures are as follows: Clinical data were gathered from electronic medical records, and data of all neurologic symptoms were checked by a trained neurologist. We reported four pediatric patients with COVID-19 and different neurologic symptoms. Case 1 was a 16-year-old girl with a sensory and motor polyradiculopathy with RT-qPCR for COVID-19 and dengue both detected in CSF that improved after appropriate treatment. Case 2 was a 15-year-old boy with Guillain-Barre syndrome and had good response after using human immunoglobulin. Case 3 was a 5-year-old girl with acute intracranial hypertension that improved after going through lumbar puncture and using acetazolamide. Case 4 was a 2-month-old male infant with focal epileptic seizures that recovered after antiepileptic treatment. We highlight the need to consider different neurologic manifestations as part of the COVID-19 clinical spectrum.

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Resumen Introducción: La Hipertensión Intracraneal Idiopática (HICI) es un síndrome neurológico caracterizado por un aumento de la presión intracraneal en ausencia de lesión estructural o hidrocefalia. Los síntomas incluyen cefalea, tinnitus pulsátil, oscurecimientos visuales transitorios y pérdida visual. Dentro de los signos destacan diplopía por parálisis del VI par, edema de papila y disminución de la agudeza visual. Los pacientes no tienen compromiso de conciencia ni signos neurológicos focales. La principal complicación es la pérdida visual que puede ser irreversible. La asociación entre HICI y nitrofurantoína (NTF) se reportó en 1974. Caso clínico: Mujer de 42 años, con sobrepeso, que desarrolló una HICI aproximadamente 18 meses posterior al inicio de nitrofurantoína profiláctica. Consultó por cefalea frontal, opresiva que aumentaba con la maniobra de Valsalva, asociada a disminución fluctuante de la agudeza visual y episodios de oscurecimiento. Al examen destacó edema de papila bilateral, sin déficit neurológico focal. La presión del líquido cefalorraquídeo (LCR) fue de 25,5 cm de agua. La resonancia magnética mostró signos de aumento de la presión del LCR, sin lesiones estructurales ni hidrocefalia. El cuadro se recuperó concomitantemente a la suspensión de la NTF y el uso de topiramato. No se constató daño visual permanente. Conclusiones: Se debe sospechar la HICI en mujeres en edad fértil con sobrepeso. Dentro de los gatillantes del síndrome destacan varios fármacos, entre ellos la NTF. El principal objetivo del tratamiento de la HICI es preservar la función visual.

Abstract Introduction: Idiopathic intracranial hypertension (IIH) is a syndrome characterized by increased intracranial pressure without a space occupying lesion or hydrocephalus. The symptoms are headache, pulsatile tinnitus, transient visual obscurations, and visual loss. Signs are diplopia caused by sixth cranial nerve paresis and papilledema with its associated loss of sensory visual function. The patient maintains an alert and oriented mental state, but has no localizing neurologic findings. The only major morbidity with IIH is visual loss. The association between IIH and nitrofurantoin was reported in 1974. Case: A 42 years old female, overweighed, who developed IIH 18 months after the start of prophylactic nitrofurantoin. She had frontal oppressive headache that increased with the Valsalva maneuver, fluctuant visual loss and transient visual obscurations. She had bilateral papilledema without localizing neurologic findings. The cerebrospinal fluid (CSF) pressure was 25.5 cm H2O. Magnetic resonance imaging showed signs of increased CSF pressure without structural lesions or hydrocephalus. IIH recovered with the withdrawal of nitrofurantoin and the use of topiramate. There was not permanent visual loss. Conclusions: It is recommendable to suspect IIH in obese women in the childbearing years. There are several drugs associated with IIH including nitrofurantoin. The main objective of treatment is to prevent visual loss.

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La hipertensión endocraneana idiopàtica se asocia infrecuentemente con la hipovitaminosis A y D. Se presenta el caso de una paciente femenina de 8 años con visión borrosa de 24 horas y papiledema bilateral. Resonancia magnética nuclear normal. Presión de apertura de líquido cefalorraquídeo: 260 mm^O. Presentó déficit de vitamina A y D, e inició un tratamiento sustitutivo. El segundo caso corresponde a un paciente masculino de 12 años con fiebre y odinofagia de 3 días. Con antecedente de glomerulonefritis y sobrepeso. Presentaba edema bipalpebral y papiledema. Tomografia axial computada de la órbita: aumento de líquido en la vaina de ambos nervios ópticos. Resonancia magnética nuclear: aracnoidocele intraselar. Presión de apertura de líquido cefalorraquídeo: 400 mm^O. Presentó déficit de vitamina D y B6, e inició el tratamiento sustitutivo. La elevación de la presión intracraneal desencadena mecanismos de compensación que, al fallar, pueden comprometer la vida o provocar graves discapacidades neurológicas. Reconocer la causa para un enfoque terapéutico preciso es clave para disminuir la morbimortalidad asociada a esta patología.

Idiopathic endocranial hypertension is infrequently associated with hypovitaminosis A and D. The case of an 8-year-old female with 24-hour blurred vision and bilateral papilledema is presented. Nuclear magnetic resonance was normal. Opening pressure of cerebrospinal fluid: 260 mm^O. She presented vitamin A and D deficiency and started replacement therapy. The second case corresponds to a 12-year-old male with fever and odynophagia of 3 days. History of glomerulonephritis and overweight. He had bipalpebral edema and papilledema. Computed tomography scan of the orbit: increase of fluid in the sheath of both optic nerves. Nuclear magnetic resonance: intrasellar arachnoidocele. Opening pressure of cerebrospinal fluid: 400 mmH2O. He presented vitamin D and B6 deficiency and started replacement treatment. The elevation of intracranial pressure triggers compensation mechanisms that, when they fail, can compromise life or cause serious neurological disabilities. Recognizing the cause for an accurate therapeutic approach is key to reduce the morbidity and mortality associated with this pathology.

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Idiopathic endocranial hypertension is infrequently associated with hypovitaminosis A and D. The case of an 8-year-old female with 24-hour blurred vision and bilateral papilledema is presented. Nuclear magnetic resonance was normal. Opening pressure of cerebrospinal fluid: 260 mmH2O. She presented vitamin A and D deficiency and started replacement therapy. The second case corresponds to a 12-year-old male with fever and odynophagia of 3 days. History of glomerulonephritis and overweight. He had bipalpebral edema and papilledema. Computed tomography scan of the orbit: increase of fluid in the sheath of both optic nerves. Nuclear magnetic resonance: intrasellar arachnoidocele. Opening pressure of cerebrospinal fluid: 400 mmH2O. He presented vitamin D and B6 deficiency and started replacement treatment. The elevation of intracranial pressure triggers compensation mechanisms that, when they fail, can compromise life or cause serious neurological disabilities. Recognizing the cause for an accurate therapeutic approach is key to reduce the morbidity and mortality associated with this pathology.

La hipertensión endocraneana idiopática se asocia infrecuentemente con la hipovitaminosis A y D. Se presenta el caso de una paciente femenina de 8 años con visión borrosa de 24 horas y papiledema bilateral. Resonancia magnética nuclear normal. Presión de apertura de líquido cefalorraquídeo: 260 mmH2O. Presentó déficit de vitamina A y D, e inició un tratamiento sustitutivo. El segundo caso corresponde a un paciente masculino de 12 años con fiebre y odinofagia de 3 días. Con antecedente de glomerulonefritis y sobrepeso. Presentaba edema bipalpebral y papiledema. Tomografía axial computada de la órbita: aumento de líquido en la vaina de ambos nervios ópticos. Resonancia magnética nuclear: aracnoidocele intraselar. Presión de apertura de líquido cefalorraquídeo: 400 mmH2O. Presentó déficit de vitamina D y B6, e inició el tratamiento sustitutivo. La elevación de la presión intracraneal desencadena mecanismos de compensación que, al fallar, pueden comprometer la vida o provocar graves discapacidades neurológicas. Reconocer la causa para un enfoque terapéutico preciso es clave para disminuir la morbimortalidad asociada a esta patología.

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Pseudotumor cerebri is a relatively common pathology that is characterized by intracranial hypertension in the absence of mass lesions. It commonly affects young and obese women, and its presentation with visual loss and bilateral papilledema is well-described in the literature. We present a case of a 44-year-old, non-obese, female patient presenting with unilateral papilledema and iron-deficiency anemia. This case emphasizes this unusual presentation and the rare association with iron deficiency.

O pseudotumor cerebral é uma patologia relativamente comum, caracterizada pela hipertensão intracraniana na ausência de lesões causando efeito de massa. Frequentemente afeta mulheres jovens e obesas, e sua apresentação, com perda do campo visual e papiledema bilateral, é bem descrita na literatura. Nós apresentamos um caso de uma paciente de 44 anos, não obesa, com papiledema unilateral e anemia ferropriva. Esse caso destaca essa apresentação incomum e a rara associação com a deficiência de ferro.

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BACKGROUND: Primary idiopathic intracranial hypertension (PIIH) in children is rare and has a poorly understood pathophysiology. It is characterized by raised intracranial pressure (ICP) in the absence of an identified brain lesion. Diagnosis is usually confirmed by the measurement of a high cerebrospinal fluid (CSF) opening pressure and exclusion of secondary causes of intracranial hypertension. Refractory PIIH may lead to severe visual impairment. The purpose of this study was to evaluate a cranial morcellation decompression (CMD) technique as a new surgical alternative to stabilize intracranial pressure in PIIH. MATERIALS AND METHODS: A literature review was carried out, disclosing only 7 pediatric cases of PIIH treated with surgical skull expansion. In addition, we describe here one case of our own experience treated by CMD. CONCLUSIONS: CMD surgery is a safe and effective option to control refractory PIIH in selected patients.

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BACKGROUND: The conventional treatment for idiopathic intracranial hypertension involves weight loss, steroids, diuretics, and/or serial lumbar punctures; however, if the symptoms persist or worsen, surgical intervention is recommended. Surgical options include cerebrospinal fluid diversion procedures, such as ventriculoperitoneal and lumboperitoneal shunts, and optic nerve decompression with nerve sheath fenestration. The latter can be carried out using an endoscopic approach, but the outcomes of this technique have not been firmly established. METHODS: This systematic review examined the outcomes of performing endoscopic optic nerve decompression (EOND) in patients with idiopathic intracranial hypertension (IIH). Six studies were included for a total of 34 patients. RESULTS: The patients presented with visual field disturbances (32 of 32 [100%]), visual acuity disruptions (33 of 34 [97.1%]), papilledema (26 of 34 [76.5%]), and persistent headache (30 of 33 [90.1%]). The mean duration of symptoms ranged from 7 to 32 months. Overall, the patients showed post-EOND improvement in signs and symptoms associated with IIH, specifically visual field deficits (93.8%), visual acuity (85.3%), papilledema (81.4%), and headaches (81.8%). Interestingly, 11 cases showed postoperative improvement in their symptoms with bony decompression of the optic canal alone, without nerve sheath fenestration. There were no major adverse events or complications reported with this approach. CONCLUSION: EOND appears to be a promising and safe surgical alternative for patients with IIH who fail to respond to medical treatment. Further studies are needed before we can attest to the clinical validity of this procedure.

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In contrast to virtually all organ systems of the body, the central nervous system was until recently believed to be devoid of a lymphatic system. The demonstration of a complex system of paravascular channels formed by the endfeet of astroglial cells ultimately draining into the venous sinuses has radically changed this idea. The system is subsidized by the recirculation of cerebrospinal fluid (CSF) through the brain parenchyma along paravascular spaces (PVSs) and by exchanges with the interstitial fluid (IF). Aquaporin-4 channels are the chief transporters of water through these compartments. This article hypothesizes that glymphatic dysfunction is a major pathogenetic mechanism underpinning idiopathic intracranial hypertension (IIH). The rationale for the hypothesis springs from MRI studies, which have shown many signs related to IIH without evidence of overproduction of CSF. We propose that diffuse retention of IF is a direct consequence of an imbalance of glymphatic flow. This imbalance, in turn, may result from an augmented flow from the arterial PVS into the IF, by impaired outflow of the IF into the paravenous spaces, or both. Our hypothesis is supported by the facts that (i) visual loss, one of the main complications of IIH, is secondary to the impaired drainage of the optic nerve, a nerve richly surrounded by water channels and with a long extracranial course in its meningeal sheath; (ii) there is a high association between IIH and obesity, a condition related to paravascular inflammation and lymphatic disturbance, and (iii) glymphatic dysfunction has been related to the deposition of ß-amyloid in Alzheimer's disease. We conclude that the concept of glymphatic dysfunction provides a new perspective for understanding the pathophysiology of IIH; it may likewise entice the development of novel therapeutic approaches aiming at enhancing the flow between the CSF, the glymphatic system, and the dural sinuses.

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La hipertensión intracraneal idiopática, también conocida como pseudotumor cerebral, es una enfermedad caracterizada por un incremento de la presión intracraneal no atribuible a masas cerebrales o a alteraciones estructurales focales, con composición normal del líquido cefalorraquídeo. En estudios imagenológicos pueden observarse ventrículos normales o pequeños. Se desconoce su etiología y patogenia. Es causa eludible de pérdida de la visión, tanto en adultos como en niños. El tratamiento es con frecuencia efectivo y la mayoría de los pacientes experimentan una resolución completa de los síntomas sin persistencia de déficits.

Idiopathic intracranial hypertension, also known as pseudotumor cerebri is a condition characterized by increased intracranial pressure, not caused to mass lesions or focal structural abnormalities, or with normal composition of the cerebrospinal fluid. Ventricles can be observed normal or small in imaging studies. Its etiology and pathogenesis are unknown. Idiopathic intracranial hypertension is an avoidable cause of visual loss, in both adults and children. Treatment is usually effective, and most patients have observed complete resolution of symptoms without persistent deficits.