Effectiveness and Safety of Ventriculoperitoneal Shunt Versus Lumboperitoneal Shunt for Idiopathic Intracranial Hypertension: A Systematic Review and Comparative Meta-Analysis.

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.

Multidisciplinary management of idiopathic intracranial hypertension in pregnancy: case series and narrative review

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.

Evaluation of pediatric patients presenting with acute-onset unilateral transient acquired blepharoptosis.

PURPOSE: To evaluate the clinical features of pediatric patients with acute-onset, unilateral transient acquired blepharoptosis. METHODS: In this retrospective study, the clinical records of patients between April 2015 and June 2020 were reviewed for evaluation of demographic features, accompanying neurological and ophthalmologic manifestations, symptom duration, etiological cause, and imaging findings. Patients with congenital and acquired blepharoptosis with chronic etiologies were excluded. RESULTS: Sixteen pediatric patients (10 boys and 6 girls) with acquired acute-onset unilateral transient blepharoptosis were included in this study. The patients' mean age was 6.93 ± 3.16 years. The most commonly identified etiological cause was trauma in 7 patients (43.75%) and infection (para-infection) in 5 patients (31.25%). In addition, Miller Fisher syndrome, Horner syndrome secondary to neuroblastoma, acquired Brown's syndrome, and pseudotumor cerebri were identified as etiological causes in one patient each. Additional ocular findings accompanied blepharoptosis in 7 patients (58.33%). Blepharoptosis spontaneously resolved, without treatment, in all the patients, except those with Miller Fisher syndrome, neuroblastoma, and pseudotumor cerebri. None of the patients required surgical treatment and had ocular morbidities such as amblyopia. CONCLUSION: This study demonstrated that acute-onset unilateral transient blepharoptosis, which is rare in childhood, may regress without the need for surgical treatment in the pediatric population. However, serious pathologies that require treatment may present with blepharoptosis.

Papilledema: A Review of the Pathophysiology, Imaging Findings, and Mimics.

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.

Multidisciplinary management of idiopathic intracranial hypertension in pregnancy: case series and narrative review.

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.

Unilateral papilledema and peripapillary polypoidal choroidal vasculopathy as the presenting manifestations of intracranial hypertension.

We have reported here the case of a 54-year-old woman with intracranial hypertension that presented with the unique features of unilateral papilledema and peripapillary polypoidal choroidal vasculopathy. Our investigations lead to the diagnosis of idiopathic intracranial hypertension and an incidental small right frontal meningioma. The patient was accordingly treated with oral acetazolamide, followed by three consecutive monthly intravitreal injections of bevacizumab, which resulted in the inactivation of the polypoidal choroidal vasculopathy, marked reduction of lipid exudation, and complete absorption of the subretinal fluid. This case serves as the first documentation of polypoidal choroidal vasculopathy associated with papilledema. It also demonstrates that choroidal vascular abnormalities may occur even when optic disk edema is unilateral, which is an uncommon manifestation of increased intracranial pressure. Prompt recognition of such findings and its appropriate management are essential for adequate treatment and prevention of irreversible visual loss.

Transcranial Doppler Plateau Wave in a Patient with Pseudo-Chiari Malformation.

CASE REPORT: A 26-year-old woman presented a superior sagittal and transverse sinus thrombosis with venous infarction. Anticoagulation was started. Six months later headache and visual impairment developed, and intracranial hypertension was diagnosed-secondary pseudotumor cerebri. It was managed with a lumbo-peritoneal shunt (LPS) resulting in a positive initial evolution with initial symptoms resolution, but headache and visual impairment eventually reappeared. Magnetic Resonance Imaging revealed a Pseudo-Chiari malformation, leading to lumbo-peritoneal shunt removal (Friedman et al. Neurology 81:1159-1165, 2013; Moncho et al. Rev Neurol 56(12):623-634, 2013). As symptoms reappeared, a short period of continuous transcranial Doppler neuromonitoring, including a change of head of bed elevation, was performed. A sudden decrease in cerebral blood flow velocity with a dramatic increase in pulsatility index developed when head of bed was moved from 45° to horizontal position. Transcranial Doppler changes were compatible with a plateau wave of intracranial hypertension. A ventricle-peritoneal shunt was inserted, which resulted in symptomatology, imaging, and digital campimetry improvement.

A case of bilateral fourth nerve palsy associated with pseudotumor cerebri syndrome / Caso de paralisia bilateral de quarto nervo associada com síndrome do pseudotumor cerebral

ABSTRACT Pseudotumor cerebri syndrome is puzzling because it results in elevated intracranial pressure with no identifiable underlying cause. Ocular motor nerve palsies, other than a unilateral or bilateral sixth cranial nerve palsy, are rarely seen in patients with this condition. We report here on a 25-year-old female patient with bilateral fourth cranial nerve palsy caused by pseudotumor cerebri syndrome, whose ocular and systemic signs and symptoms of nerve palsy were completely resolved after medical treatment. We infer that fourth nerve palsy could be associated with pseudotumor cerebri syndrome; therefore, clinicians should consider rare ocular motor nerve palsies, even bilaterally, in patients with pseudotumor cerebri syndrome.

RESUMO A sindrome do pseudotumor cerebral é uma síndrome enigmática caracterizada por aumento da pressão intracraniana sem causa definida. Na síndrome do pseudotumor cerebral, as paralisias de nervo oculomotor além da paralisia unilateral ou bilateral do sexto nervo craniano são raramente vistas. Nós reportamos o caso de uma paciente feminina de 25 anos com paralisia bilateral de quarto nervo craniano como resultado da síndrome do pseudotumor cerebral. Após tratamento médico para síndrome do pseudotumor cerebral, os sinais e sintomas oculares e sistêmicos da paralisia nervosa foram resolvidos. Em conclusão, a paralisia de quarto nervo craniano pode estar associada com síndrome de pseudotumor cerebral, portanto médicos devem considerar paralisias raras de nervo oculomotor, mesmo bilateralmente, em pacientes com síndrome do pseudotumor cerebral.

A case of bilateral fourth nerve palsy associated with pseudotumor cerebri syndrome.

Pseudotumor cerebri syndrome is puzzling because it results in elevated intracranial pressure with no identifiable underlying cause. Ocular motor nerve palsies, other than a unilateral or bilateral sixth cranial nerve palsy, are rarely seen in patients with this condition. We report here on a 25-year-old female patient with bilateral fourth cranial nerve palsy caused by pseudotumor cerebri syndrome, whose ocular and systemic signs and symptoms of nerve palsy were completely resolved after medical treatment. We infer that fourth nerve palsy could be associated with pseudotumor cerebri syndrome; therefore, clinicians should consider rare ocular motor nerve palsies, even bilaterally, in patients with pseudotumor cerebri syndrome.

Factors affecting visual loss and visual recovery in patients with pseudotumor cerebri syndrome.

PURPOSE: To investigate the frequency of visual loss (VL), possible predictive factors of VL, and improvement in patients with pseudotumor cerebri (PTC) syndrome. METHODS: We reviewed 50 PTC patients (43 females, seven males) who underwent neuro-ophthalmic examination at the time of diagnosis and after treatment. Demographic data, body mass index (BMI), time from symptom onset to diagnosis (TD), maximum intracranial pressure (MIP), occurrence of cerebral venous thrombosis (CVT), and treatment modalities were reviewed. VL was graded as mild, moderate, or severe on the basis of visual acuity and fields. Predictive factors for VL and improvement were assessed by regression analysis. RESULTS: The mean ± SD age, BMI, and MIP were 35.2 ± 12.7 years, 32.0 ± 7.5 kg/cm2, and 41.9 ± 14.5 cmH2O, respectively. Visual symptoms and CVT were present in 46 and eight patients, respectively. TD (in months) was <1 in 21, 1-6 in 15, and >6 in 14 patients. Patients received medical treatment with (n=20) or without (n=30) surgery. At presentation, VL was mild in 16, moderate in 12, and severe in 22 patients. Twenty-eight patients improved and five worsened. MIP, TD, and hypertension showed a significant correlation with severe VL. The best predictive factor for severe VL was TD >6 months (p=0.04; odds ratio, 5.18). TD between 1 and 6 months was the only factor significantly associated with visual improvement (p=0.042). CONCLUSIONS: VL is common in PTC, and when severe, it is associated with a delay in diagnosis. It is frequently permanent; however, improvement may occur, particularly when diagnosed within 6 months of symptom onset.

Relationship Between Pattern Electroretinogram, Frequency-Domain OCT, and Automated Perimetry in Chronic Papilledema From Pseudotumor Cerebri Syndrome.

PURPOSE: We evaluated the ability of transient pattern electroretinogram (PERG) parameters to differentiate between eyes with visual field (VF) loss and resolved papilledema from pseudotumor cerebri syndrome (PTC) and controls, to compare PERG and optical coherence tomography (OCT) with regard to discrimination ability, and to assess the correlation between PERG, frequency domain OCT (FD-OCT), and VF measurements. METHODS: The VFs and full-field stimulation PERGs based on 48 and 14-min checks were obtained from patients with PTC (n = 24, 38 eyes) and controls (n = 26, 34 eyes). In addition, FD-OCT peripapillary retinal nerve fiber layer (RNFL) and segmented macular layer measurements were obtained and correlation coefficients were determined. RESULTS: Compared to controls, PERG N95 and P50+N95 amplitude measurements with 48-minute checks were significantly reduced in eyes with resolved papilledema from PTC. Both PERG N95 amplitude and OCT parameters were able to discriminate papilledema eyes from controls with a similar performance. Significant correlations, ranging from 0.25 (P < 0.05) to 0.43 (P < 0.01) were found between PERG amplitude values and OCT-measured macular ganglion cell layer thickness, RNFL thickness, and total retinal thickness. The PERG amplitude also was significantly associated with VF sensitivity loss with correlation coefficients ranging from 0.24 (P < 0.05) and 0.35 (P < 0.01). CONCLUSIONS: The PERG measurements were able to detect neural loss in PTC eyes with resolved papilledema and were reasonably well correlated with OCT measurements and VF parameters. Thus, PERG may be a useful tool in the monitoring of retinal neural loss in eyes with active papilledema from PTC.

Factors affecting visual loss and visual recovery in patients with pseudotumor cerebri syndrome / Fatores que influenciam na perda e na recuperação visual de pacientes com a síndrome do pseudotumor cerebral

ABSTRACT Purpose: To investigate the frequency of visual loss (VL), possible predictive factors of VL, and improvement in patients with pseudotumor cerebri (PTC) syndrome. Methods: We reviewed 50 PTC patients (43 females, seven males) who underwent neuro-ophthalmic examination at the time of diagnosis and after treatment. Demographic data, body mass index (BMI), time from symptom onset to diagnosis (TD), maximum intracranial pressure (MIP), occurrence of cerebral venous thrombosis (CVT), and treatment modalities were reviewed. VL was graded as mild, moderate, or severe on the basis of visual acuity and fields. Predictive factors for VL and improvement were assessed by regression analysis. Results: The mean ± SD age, BMI, and MIP were 35.2 ± 12.7 years, 32.0 ± 7.5 kg/cm2, and 41.9 ± 14.5 cmH2O, respectively. Visual symptoms and CVT were present in 46 and eight patients, respectively. TD (in months) was <1 in 21, 1-6 in 15, and >6 in 14 patients. Patients received medical treatment with (n=20) or without (n=30) surgery. At presentation, VL was mild in 16, moderate in 12, and severe in 22 patients. Twenty-eight patients improved and five worsened. MIP, TD, and hypertension showed a significant correlation with severe VL. The best predictive factor for severe VL was TD >6 months (p=0.04; odds ratio, 5.18). TD between 1 and 6 months was the only factor significantly associated with visual improvement (p=0.042). Conclusions: VL is common in PTC, and when severe, it is associated with a delay in diagnosis. It is frequently permanent; however, improvement may occur, particularly when diagnosed within 6 months of symptom onset. .

RESUMO Objetivo: Investigar a frequência de perda visual (PV) e os possíveis fatores preditivos para perda e para melhora visual em pacientes com a síndrome do pseudotumor cerebral (SPC). Métodos: Foram revisados 50 pacientes com SPC submetidos a exame neuroftalmológico no momento do diagnóstico e após o tratamento. Dados demográficos, índice de massa corpórea (IMC), tempo decorrido entre o início dos sintomas e o diagnóstico (TD), pressão intracraniana máxima (PIM), ocorrência de trombose venosa cerebral (TVC), e as modalidades de tratamento foram revisadas. PV foi graduada em discreta, moderada e grave, baseada na acuidade e no campo visual. Fatores preditivos para perda e melhora visual foram avaliados por análise de regressão linear. Resultados: Quarenta e três pacientes eram do sexo feminino. A média de idade, o IMC e a PIM (± desvio padrão) foram: 35,2 ± 12,7 anos, 32,0 ± 7,5 kg/cm2 e 41,9 ± 14,5 cmH2O, respectivamente. Sintomas visuais estavam presentes em 46 e TVC em 8 pacientes. TD (em meses) foi <1 em 21, 1-6 em 15 e >6 em 14 pacientes. Pacientes receberam tratamento clinico apenas (n=30) ou associado a tratamento cirúrgico (n=20). Na apresentação a PV era discreta em 16, moderada em 12 e grave em 22 pacientes. Vinte e oito pacientes melhoraram e 5 pioraram. PIM, TD e hipertensão arterial correlacionaram significativamente com PV grave. O melhor fator preditivo para PV grave foi o TD>6 meses (p=0,04; razão de chances 5,18). TD entre 1 e 6 meses foi o único fator significativamente associado com melhora visual após tratamento (p=0,042). Conclusões: Perda visual é comum na SPC e quando grave se mostra relacionado a atraso no diagnóstico. É usualmente permanente mas pode haver melhora visual especialmente quando a doença é diagnosticada nos primeiros 6 após o início dos sintomas. .

Macular thickness measurements with frequency domain-OCT for quantification of axonal loss in chronic papilledema from pseudotumor cerebri syndrome.

PURPOSE: To evaluate the ability of frequency domain-optical coherence tomography (FD-OCT)-measured macular thickness parameters to differentiate between eyes with resolved chronic papilledema and healthy eyes and to evaluate the correlation between FD-OCT measures and visual field (VF) loss on standard automated perimetry (SAP). METHODS: Fifty-two eyes from 29 patients suffering from pseudotumor cerebri syndrome (PTC) and 62 eyes from 31 normal controls underwent FD-OCT scanning and ophthalmic evaluation including VF with SAP. All patients had previously been submitted to treatment of PTC and had clinically resolved papilledema and stable VF for at least 6 months before the study. Macular and peripapillary retinal nerve fiber layer (RNFL) thickness measurements were determined for both groups. Comparisons were made using Generalized Estimated Equations. Correlations between FD-OCT and VF measurements were verified. RESULTS: In eyes with resolved papilledema, the macular thickness parameters corresponding to the inner and outer superior, temporal, inferior and nasal segments, average macular thickness and most RNFL thickness measurements were significantly reduced when compared with controls. The discrimination ability was similar for macular thickness measurements and RNFL thickness measurements. Both sets of OCT measurements correlated well with VF sensitivity loss. CONCLUSIONS: Eyes with resolved chronic papilledema show a significant reduction in macular thickness, which is well correlated with the severity of VF loss. Macular thickness measurements can potentially be used to estimate and monitor the amount of ganglion cell loss in eyes with papilledema from patients with PTC.

[Acute optic neuropathy: differential diagnoses]. / Neuropatía óptica aguda: diagnósticos diferenciales.

INTRODUCTION: Functional impairment of the optic nerve is characterized by visual loss, dyschromatopsia, visual field defects, relative afferent pupillary defect, and swelling or atrophy of the optic nerve. AIM: To describe the spectrum of acute optic neuropathies, focusing on clinical features, diagnosis and treatment with an emphasis on pediatric entities. DEVELOPMENT: Optic neuritis may be monophasic, recurrent, or part of a polysymptomatic demyelinating process. The aim of the treatment is to reduce number and severity of attacks and prevent future disability. Infectious neuritis is secondary to different microorganisms (bacteria, virus, fungi, or protozoa). Treatment is related to etiology. Nonarteritic ischemic optic neuropathy or idiopathic optic neuropathy is the most frequent form and is secondary to a disorder of small retinal vessels. Leber hereditary optic neuropathy is an important cause of chronic visual impairment and is characterized by selective involvement of the retinal ganglion cells. Until now, no curative treatment is available. Visual acuity is frequently conserved in papilledema associated with intracranial hypertension. The aim of treatment is to reduce intracranial hypertension and risk factors in case it is secondary. CONCLUSIONS: Acute optic neuropathies are broad group of entities, of different etiologies, and with a variable visual prognosis. Findings of neurological examination, fundoscopy, and neuroimaging guide diagnosis and prompt treatment.

TITLE: Neuropatia optica aguda: diagnosticos diferenciales.Introduccion. La alteracion funcional del nervio optico se caracteriza por un deficit en la agudeza visual, en la vision cromatica y en el campo visual, defecto pupilar aferente y, en algunos casos, edema del nervio o atrofia y palidez. Objetivo. Describir el espectro de neuropatias opticas agudas, su clinica, diagnostico y tratamiento, con mayor interes en aquellas de presentacion en la edad pediatrica. Desarrollo. La neuritis optica puede ser monofasica, recurrente o el componente de un cuadro desmielinizante polisintomatico. El objetivo del tratamiento es reducir el numero y la gravedad de los ataques y prevenir discapacidad. La infecciosa es secundaria a diferentes microorganismos (bacterias, virus, hongos y protozoos). El tratamiento depende de la etiologia. La isquemica anterior no arteritica o idiopatica es la forma mas frecuente y es secundaria a enfermedad de pequeños vasos (ciliares posteriores). La neuropatia optica hereditaria o de Leber representa una causa importante de afectacion visual cronica y se caracteriza por la afectacion selectiva de las celulas ganglionares de la retina. Hasta el momento, la terapia solo es de apoyo. En el papiledema asociado a hipertension endocraneal, la agudeza visual generalmente se conserva pero existe aumento de la mancha ciega. El tratamiento se basa en disminuir la hipertension y el factor etiologico si existe. Conclusiones. Las neuropatias opticas agudas constituyen un amplio grupo de entidades, de etiologia diversa y con un pronostico visual variable. La presencia de signos del examen neurologico, fondo de ojo y neuroimagenes pueden orientar hacia el diagnostico y tratamiento oportuno.