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Medically refractory idiopathic intracranial hypertension (IIH) is frequently treated with venous sinus stenosis stenting with high success rates. Patient selection has been driven almost exclusively by identification of supraphysiological venous pressure gradients across stenotic regions based on theoretical assessment of likelihood of response.To explore the possibility of benefit in low venous pressure gradient patients.Using a single-center, prospectively maintained registry of patients with IIH undergoing venous stenting, we defined treatment groups by gradient pressures of ≤4, 5 to 8, and8 mmHg based on the most frequently previously published thresholds for stenting. Baseline demographics, clinical, and neuro-ophthalmological outcomes (including optical coherence tomography and Humphrey visual fields) were compared.Among 53 patients, the mean age was 32 years and 70% female with a mean body mass index was 36 kg/m 2 . Baseline characteristics were similar between groups. The mean change in lumbar puncture opening pressure at 6 months poststenting was similar between the 3 groups (≤4, 5-8, and8 mmHg; 13.4, 12.9, and 12.4 cmH 2 O, P = .47). Papilledema improvement was observed across groups at 6 months (100, 93, and 86, P = .7) as were all clinical symptoms. The mean changes in optical coherence tomography retinal nerve fiber layer (-30, -54, and -104, P = .5) and mean deviation in Humphrey visual fields (60, 64, and 67, P = .5) at 6 weeks were not significantly different.Patients with IH with low venous pressure gradient venous sinus stenosis seem to benefit equally from venous stenting compared with their higher gradient counterparts. Re-evaluation of our restrictive criteria for this potentially vision sparing intervention is warranted. Future prospective confirmatory studies are needed.

Idiopathic intracranial hypertension (IIH) can cause debilitating symptoms and optic nerve ischemia if untreated. Cerebrospinal fluid diversion is often necessary to reduce intracranial pressure; however, current ventriculoperitoneal and lumboperitoneal shunting techniques have high failure rates in patients with IIH.To describe our experience treating IIH with a novel stereotactic-guided transcerebellar cisternoperitoneal shunt (SGTC-CPS) technique that places the proximal shunt catheter in the posterior cisterna magnum.Retrospective perioperative and postoperative data from all patients who underwent SGTC-CPS placement for IIH from March 1, 2015, to December 31, 2020, were analyzed. Patients were positioned as for ventriculoperitoneal shunt placement but with the head turned farther laterally to adequately expose the retrosigmoid space. Using neuronavigation, an opening was made near the transverse-sigmoid junction, and the proximal catheter was inserted transcerebellarly into the posterior foramen magnum.Thirty-two patients underwent SGTC-CPS placement (29 female; mean body mass index, 36.0 ± 7.5; 14 with prior shunt failures). The mean procedure time for shunt placement was 145 minutes. No intraoperative complications occurred, and all patients were discharged uneventfully. At the 6-month follow-up, 81% of patients (21 of 26) had relief of their presenting symptoms. Shunt survival without revision was 86% (25 of 29) at 1 year and 67% (10 of 15) at 3 years, with no infections.The SGTC-CPS offers an alternative solution for cerebrospinal fluid diversion in patients with IIH and demonstrates a lower failure rate and more durable symptom relief compared with ventriculoperitoneal or lumboperitoneal shunt placement. Using proper techniques and equipment promotes safe and facile placement of the proximal catheter.

This study evaluates the effectiveness of a multidisciplinary protocol for management of patients with papilledema and vision loss secondary to increased intracranial pressure.Retrospective record review of all adult patients who presented with vision-threatening papilledema (VTPE) and were treated under this protocol. Patients are admitted for lumbar drain placement and diuretics and followed daily to determine if they may be managed medically or require surgery (optic nerve sheath fenestration [ONSF] and/or cerebrospinal fluid [CSF] shunting).Nineteen patients were included. Twelve had body mass index in the obese range and 6 were morbidly obese. Fourteen had idiopathic intracranial hypertension. Five had secondary pseudotumor cerebri syndrome related to medication use, dural venous sinus thrombosis, hypothyroidism, end-stage renal disease, pulmonary disease, and diastolic heart failure. Three patients did not require surgery and were discharged on oral diuretics; 3 patients underwent unilateral ONSF, 9 underwent bilateral ONSF, and 4 underwent bilateral ONSF followed by ventriculoperitoneal shunt placement. The average follow-up was 10.1 months. The visual acuity improved bilaterally in 12 patients and unilaterally in 4 patients. The remaining 3 patients had worsened vision in both eyes. Fifteen patients had bilateral improvement in their visual fields. Five eyes in 3 patients showed further constriction of the visual field at follow-up.We demonstrate how a multidisciplinary complex care protocol for treating VTPE can expedite and streamline treatment and restore vision. We found that most patients had improved symptoms and signs, including visual acuity, visual fields, and papilledema. We encourage institutions that manage VTPE to adopt similar institutional protocols.

To determine whether the use of a tetracycline-class antibiotic is associated with an increased risk of developing pseudotumor cerebri syndrome (PTCS).We identified patients in the University of Utah Health system who were prescribed a tetracycline-class antibiotic and determined what percentage of those individuals were subsequently diagnosed with PTCS secondary to tetracycline use. We compared this calculation to the number of patients with PTCS unrelated to tetracycline use.Between 2007 and 2014, a total of 960 patients in the University system between the ages of 12 and 50 were prescribed a tetracycline antibiotic. Among those, 45 were diagnosed with tetracycline-induced PTCS. We estimate the incidence of tetracycline-induced PTCS to be 63.9 per 100,000 person-years. By comparison, the incidence of idiopathic intracranial hypertension (IIH) is estimated to be less than one per 100,000 person-years (Calculated Risk Ratio = 178).Although a causative link between tetracycline use and pseudotumor cerebri has yet to be firmly established, our study suggests that the incidence of pseudotumor cerebri among tetracycline users is significantly higher than the incidence of IIH in the general population.

Background Misclassification bias is introduced into medical claims-based research because of reliance on diagnostic coding rather than full medical record review. We sought to characterize this bias for idiopathic intracranial hypertension (IIH) and evaluate strategies to reduce it. Methods A retrospective review of medical records was conducted using a clinical data warehouse containing medical records and administrative data from an academic medical center. Patients with 1 or more instances of International Classification of Diseases (ICD)-9 or -10 codes for IIH (348.2 or G93.2) between 1989 and 2017 and original results of neuroimaging (head CT or MRI), lumbar puncture, and optic nerve examination were included in the study. Diagnosis of IIH was classified as definite, probable, possible, or inaccurate based on review of medical records. The positive predictive value (PPV) for IIH ICD codes was calculated for all subjects, subjects with an IIH code after all testing was completed, subjects with high numbers of IIH ICD codes and codes spanning longer periods, subjects with IIH ICD codes associated with expert encounters (ophthalmology, neurology, or neurosurgery), and subjects with acetazolamide treatment. Results Of 1,005 patients with ICD codes for IIH, 103 patients had complete testing results and were included in the study. PPV of ICD-9/-10 codes for IIH was 0.63. PPV in restricted samples was 0.82 (code by an ophthalmologist n = 57), 0.70 (acetazolamide treatment n = 87), and 0.72 (code after all testing, n = 78). High numbers of code instances and longer duration between the first and last code instance also increased the PPV. Conclusions An ICD-9 or -10 code for IIH had a PPV of 63% for probable or definite IIH in patients with necessary diagnostic testing performed at a single institution. Coding accuracy was improved in patients with an IIH ICD code assigned by an ophthalmologist. Use of coding algorithms considering treatment providers, number of codes, and treatment is a potential strategy to reduce misclassification bias in medical claims-based research on IIH. However, these are associated with a reduced sample size.

Idiopathic intracranial hypertension (IIH) is a disorder characterized by elevated intracranial pressure without secondary causes on neuroimaging. IIH typically occurs in young, obese female patients and, when severe, can cause permanent and irreversible vision loss. The association between skull base thinning in patients with intracranial hypertension and obesity has been previously reported; however, no study has reported these findings in IIH. The goal of our study is to determine whether IIH is independently associated with skull base and calvarial thinning.A retrospective, matched case-control study was performed. Each patient diagnosed with IIH (case) was matched with a patient diagnosed with headache (control) by age, gender, and race. Patients were included if they underwent computed tomographic imaging of the head, maxillofacial, or orbits within 3 months of their diagnosis. Exclusion criteria were history of skull base or frontal bone pathology because of surgery or skull trauma, central nervous system infections, or incomplete radiologic data. Patient demographics, medical history, clinical examination, and skull base, calvarial, and zygoma thickness were recorded. Skull base thickness was measured by the height of the auditory canal in the coronal plane. Calvarial thickness was measured just anterior to the foramen rotundum in the coronal plane. Extracranial zygoma thickness was measured and used as an internal imaging control because the zygoma is not subject to intracranial forces.One hundred twenty-six patients were included in the study, 63 cases and 63 controls. Each group comprised 61 female patients (97%), 24 (38%) Caucasian, 23 (37%) black, 1 (2%) Asian, and 15 (24%) others. The average age was 31.5 ± 8.7 years. Patients with IIH were more likely to be obese (n = 60, 95%) compared with the control patients (n = 23, 37%, P0.001). All patients with IIH underwent lumbar puncture (LP) with an average opening pressure (OP) of 40.5 ± 15.6 cm H2O, whereas only 13 (20%) controls underwent an LP with a mean OP of 19.5 ± 8.5 cm H2O. There was no statistical difference in mean visual acuity between the IIH and control groups (logMar 0.22 [20/30] ± 0.45 vs logMar 0.09 [20/25] ± 0.30, P = 0.093, respectively). Compared with the controls, patients with IIH were more likely to have headache (97% vs 74%, P = 0.001), pulsatile tinnitus (48% vs 7%, P0.001), horizontal binocular diplopia (24% vs 4%, P = 0.006), confrontational visual field deficit (23% vs 2%, P = 0.003), and papilledema (74% vs 0%, P0.001). Patients with IIH had thinner skull base and calvarium width compared with the controls (mean skull base thickness 4.17 ± 0.94 mm vs 5.05 ± 1.12 mm, P0.001 and mean calvarial width 1.50 ± 0.50 mm vs 1.71 ± 0.61 mm, P = 0.024). Zygoma thickness was similar in both groups (mean zygoma thickness 1.18 ± 0.30 mm in the IIH group vs 1.26 ± 0.35 mm in the control group, P = 0.105). In a subgroup analysis controlling for obesity (body mass index30 kg/m2), there was no statistically significant difference in skull base, calvarial, or zygoma thickness between obese and nonobese patients.Patients with IIH have thinner mean skull base and calvarial thickness compared with the controls. There was no difference in the mean extracranial zygoma thickness, which was the internal imaging control. Contrary to previous reports, we did not find an association between obesity and skull base or calvarial thinning. These findings suggest that IIH is associated with skull base and calvarial thinning.

We aim to compare the clinical characteristics, disease course and visual outcomes between Canadian patients with idiopathic intracranial hypertension (IIH) who were incidentally discovered and those who sought care due to symptoms of IIH.Retrospective chart review of consecutive IIH patients conducted at tertiary neuro-ophthalmology clinics. Patients were divided into Group 1 (incidentally discovered disease) and Group 2 (patients seeking medical care due to symptoms of intracranial hypertension).One hundred eighty-six patients were included in the study; of which, 75 (40.3%; Group-1) were incidentally discovered and 111 presented due to symptoms of IIH (Group-2). There were no differences in proportion of females (P = 0.101), age (P = 0.450), body mass index (P = 0.386), MRI findings of empty or partially empty sella (P = 0.41), and sella grade (P = 0.704). Group-1 patients were less likely to have experienced headache (P = 0.001), transient visual obscurations (P0.001), and diplopia (P = 0.026) at presentation. Group-1 patients were less likely to receive medical (P0.001) and surgical (P = 0.004) treatment. There was no difference in proportion of patients who lost weight between the groups (P = 0.848). At baseline, Group-1 patients exhibited better visual acuity (P = 0.001), Humphrey mean deviation (P0.001) and retinal nerve fiber layer thickness (P0.001). Group-1 patients continued to have better visual acuity (P = 0.002) and Humphrey mean deviation (P0.001) at final follow-up.A significant portion of IIH patients were incidentally discovered. This group exhibited a favorable prognosis and only a minority of these patients required treatment. The way in which patients enter the medical system may be a valuable way to risk stratify IIH patients.

Although prompt and suitable treatment of pseudotumor cerebri syndrome (PTCS) leads to an excellent prognosis and can prevent optic nerve atrophy, adults show long-lasting neurocognitive deficits even with prompt treatment. The purpose of our study was to evaluate cognitive outcomes in pediatric patients with PTCS.We performed a prospective study on children diagnosed with PTCS and a healthy control group. Children with pre-existing neurological conditions or psychiatric drug use were excluded. Both groups underwent a neurocognitive evaluation, using the NeuroTrax computerized battery of tests. The PTCS group were tested 3 months after the initial diagnosis.We evaluated 82 children (49 females [60%], 6.5-16 years old, mean age 13.3), including 26 diagnosed with idiopathic PTC and 56 controls. Global cognitive score (P0.001), verbal memory (P0.001), executive function (P0.001), attention (P0.003), and information processing speed (P0.004) were all significantly lower in the PTCS group. No differences were found between children currently being treated and those whose symptoms had resolved and treatment was stopped.Children with PTCS experience comprehensive cognitive decline that persists after the resolution of the symptoms and treatment.

Lumbar puncture CSF pressure measurements in a large group of adults (116) having lumbar puncture (LP) for diagnostic reasons with no clinical indication of raised intracranial pressure were used to establish the normal range of CSF pressure. The cerebrospinal fluid (CSF) pressure was also measured in a smaller unselected series of patients (35) with the syndrome of idiopathic intracranial hypertension (IIH). All the lumbar punctures were performed by the same highly skilled operator, a consultant nurse, to ensure accuracy of measurement.The results showed that the mean CSF pressure was 18.7 cm H 2 O with a range of 1-29 cm H 2 O in the group with normal CSF pressure. Ninty-five percentage of values was below 29 cm H 2 O in the group with normal CSF pressure. In the series with IIH, the mean and range were 37.7 cm H 2 O and 29.5-66 cm H 2 O, respectively. The lowest recorded pressure in the IIH group was 29.5 cm H 2 O with 95% of values above 31.This is the first time that the normal range of CSF pressure and that found in a group of patients with IIH has been reported after LP performed by the same operator to ensure inaccuracy of measurement has not biased the results. It suggests that the current upper limit of normal adopted by the revised diagnostic criteria for IIH (25 cm H 2 O) may be too low. The range of normal CSF pressure and that found in patients with IIH in our study suggests that consideration should be given to revising the upper limit of normal CSF pressure to around 30 cm H 2 O.

BACKGROUND Idiopathic intracranial hypertension syndrome (IIH) is most common among obese women. Weight loss is an important factor in improving papilledema. Over the last decade, growing evidence has identified gut microbiota as a potential factor in the pathophysiology of obesity. Accordingly, we investigated whether the gut microbiome is modified in IIH patients compared with healthy controls, and provide possible new treatment venues. METHODS Shotgun metagenomic sequencing of the gut microbiome of 25 cases of IIH patients (according to the modified Dandy criteria) and 20 healthy controls. Participants were further stratified according to their body mass index. The total DNA from each sample was extracted using the PureLink Microbiome DNA Purification Kit A29789 (Invitrogen, Thermo Fisher Scientific, US). Library preparation was performed using the Nextera DNA Flex Library Prep Kit. Samples were sequenced on the Illumina Novaseq 6000 device. A list of bacterial species that significantly differed between the IIH patients and healthy controls was produced in addition to species diversity. In addition, patients' cohort alone was analyzed, (excluding the healthy controls), and the effect of acetazolamide treatment on their gut microbiota was analyzed. RESULTS IIH patients have a lower diversity of bacterial species compared with healthy individuals. These bacteria, that is, Lactobacillus ruminis (L. ruminis) (p

The purpose of this study was to compare perioperative safety and efficacy of posterior vault distraction osteogenesis (PVDO) in patients with primary nonsynostotic cephalo-cranial disproportion, namely slit ventricle syndrome and idiopathic intracranial hypertension (IIH), to a cohort of patients with craniosynostosis (CS). A retrospective review of patients undergoing PVDO from 2009 to 2019 at our institution was performed. Craniosynostosis patients were matched by sex and age at PVDO to the nonsynostotic cohort. Operative details, perioperative outcomes, and distraction patterns were analyzed with appropriate statistics. Nine patients met inclusion criteria for the non-CS cohort. Six patients (67%) underwent PVDO for slit ventricle and the remaining 3 patients (33%) underwent PVDO for IIH. The majority of CS patients were syndromic (n = 6, 67%) and had multisuture synostosis (n = 7, 78%). The non-CS cohort underwent PVDO at a median 56.1 months old [Q1 41.1, Q3 86.6] versus the CS cohort at 55.7 months [Q1 39.6, Q3 76.0] (P = 0.931). Total hospital length of stay was longer in the non-CS patients (median days 5 [Q1 4, Q3 6] versus 3 [Q1 3, Q3 4], P = 0.021). Non-CS patients with ventriculoperitoneal shunts had significantly less shunt operations for ICP concerns post-PVDO (median rate: 1.74/year [Q1 1.30, Q3 3.00] versus median: 0.18/year [Q1 0.0, Q3 0.7]; P = 0.046). In this pilot study using PVDO to treat slit ventricle syndrome and IIH, safety appears similar to PVDO in the synostotic setting. The cohort lacks adequate follow-up to assess long term efficacy, although short-midterm follow-up demonstrates promising results with less need for shunt revision and symptomatic relief. Future studies are warranted to identify the preferred surgical approach in these complex patients.

BACKGROUND The relationship between trans-stenotic blood flow velocity differences and the cerebral venous pressure gradient (CVPG) in transverse sinus (TS) stenosis (TSS) has not been studied. OBJECTIVE To evaluate the hemodynamic manifestations of TSS and the relationship between trans-stenotic blood flow velocity differences and the CVPG. METHODS Thirty-three patients with idiopathic intracranial hypertension (IIH) or pulsatile tinnitus (PT) and TSS who had undergone diagnostic venography using venous manometry were included in the patient group. Thirty-three volunteers with no stenosis and symptoms were included in the control group. All the 2 groups underwent prospective venous sinus 4-dimensional (4D) flow magnetic resonance imaging (MRI). The average velocity (Vavg) difference and maximum velocity (Vmax) difference between downstream and upstream of the TS in 2 groups were measured and compared. Correlations between the CVPG and trans-stenotic Vavg difference/Vmax difference/index of transverse sinus stenosis (ITSS) were assessed in the patient group. RESULTS The differences in Vavg difference and Vmax difference between the patient and control groups showed a statistical significance (P

Objectives To characterize patterns of sensorineural hearing loss (SNHL) and tinnitus in patients with idiopathic intracranial hypertension (IIH). Study design Retrospective chart review. Setting Tertiary referral center. Patients Adult patients diagnosed with IIH via lumbar puncture (LP) between 2010 and 2020 who had available audiograms. The study included a total of 40 patients; 33 women, and 7 men with a median age of 43. Interventions Diagnostic LP and audiogram. Main outcome measures Otologic symptoms, ophthalmologic signs, hearing thresholds, cerebrospinal fluid opening pressures. Results The most commonly reported symptoms were tinnitus in 28 (70%, 23 pulsatile and 5 tonal), aural fullness in 11 (28%), and vertigo in 10 (25%). Twenty-nine patients had ophthalmologic examinations and 18 had evidence of papilledema. Twenty-five (63%) patients had hearing loss in at least one ear at one frequency range. Patients presented with both unilateral and bilateral hearing loss across low, middle, and high frequency ranges. No significant association was observed between hearing loss threshold and LP opening pressure except for 250 Hz in the left ear. After stratification by tinnitus group (pulsatile, tonal, and no tinnitus), no significant difference was found between mean hearing loss threshold at different frequencies. In addition, no significant mean age difference was identified in patients within each tinnitus group. Conclusions There was no classic pattern or presentation for hearing loss in our IIH patients. They developed sudden, unilateral, or bilateral SNHL in low, middle, or high frequency range. The degree of hearing loss did not correlate with CSF opening pressure.

To evaluate the change in intraocular pressure (IOP) in patients with idiopathic intracranial hypertension (IIH) who underwent optic nerve sheath fenestration (ONSF) and to determine if radiographic evidence of posterior scleral or globe indentation influenced IOP.This is a retrospective analysis of IOP in IIH patients who underwent ONSF. The study included all patients from September 2010 to March 2018 operated on by a single surgeon (R.M.). IOPs preoperatively and postoperatively were recorded along with the acetazolamide dosage and whether there was evidence of posterior scleral or globe indentation on preoperative MRI.A total of 29 patients (35 eyes) with IIH underwent ONSF. The average reduction in IOP among all patients was 1.24 mm Hg (P = 0.0218), but this increased to 2.69 mm Hg (P = 0.004) in patients who were maintained on the same dosage of acetazolamide in the preoperative and postoperative period. Furthermore, the reduction in IOP in those with posterior scleral or globe indentation was 2.5 mm Hg (P = 0.0095). When the perioperative period was evaluated, the mean decrease in IOP was 1.83 mm Hg (P = 0.0217).Reducing the cerebral spinal fluid pressure (CSFP) at the level of the intraorbital optic nerve through an ONSF can slightly reduce the IOP. In those with evidence of posterior globe or scleral indentation/flattening, the reduction in IOP was higher, which supports the theory that CSF pressure indents the globe and leads to an increase in IOP. Although these changes in IOP are small, this study provides further evidence for a connection between IOP and CSFP.

SIGNIFICANCE Choroidal vascularity index measured by image binarization method from peripapillary optical coherence tomography sections has been found significantly lower in papilledema patients than healthy controls. PURPOSE The purpose of this study was to compare peripapillary choroidal parameters in papilledema patients with control subjects. METHODS Peripapillary spectral domain optical coherence tomography scans of 34 patients with papilledema and 34 healthy controls are acquired for the study. Images are binarized with the ImageJ software (National Institutes of Health, Bethesda, MD) to calculate total choroidal area, stromal area, luminal area, and choroidal vascularity index. RESULTS Total choroidal area, luminal area, and choroidal vascularity were significantly lower in papilledema patients compared with healthy controls on right (1.343 ± 0.286 vs. 1.694 ± 0.344, P < .001; 0.880 ± 0.209 vs. 1.167 ± 0.255, P < .001; 65.28 ± 2.99% vs. 68.68 ± 2.81%, P < .001, respectively) and left eyes (1.376 ± 0.308 vs. 1.647 ± 0.339, P < .001; 0.899 ± 0.231 vs. 1.134 ± 0.237, P < .001; 64.92 ± 3.44 vs. 68.84 ± 3.23, P < .001, respectively). No difference was found between active and remitted stages of papilledema in terms of choroidal parameters. CONCLUSIONS Peripapillary total choroidal area, luminal area, and choroidal vascularity index are significantly reduced in patients with papilledema. These parameters might be beneficial tools for evaluating choroidal vascularity in papilledema quantitatively and differential diagnosis for optic disc edema.

OBJECTIVE To assess the frequency of radiographic features of elevated intracranial pressure (ICP) in patients with sigmoid sinus wall anomalies (SSWA) and compare to those in idiopathic intracranial hypertension (IIH) and spontaneous CSF (sCSF) leaks. STUDY DESIGN Retrospective review. SETTING Tertiary care center. PATIENTS 110 patients - 62 SSWAs, 19 IIH, 29 sCSF leaks. MAIN OUTCOME MEASURES Demographics, comorbidities and radiographic features by diagnosis. RESULTS Imaging findings indicative of elevated ICP were similar across all three groups, as were body mass index, hyperlipidemia and diabetes. On univariate analysis, sCSF leak patients were significantly older than SSWA (60 vs. 41 years, p

BACKGROUND Lumbar puncture (LP) opening pressures (OPs) are known to fluctuate based on diurnal, environmental, and pathologic conditions. Despite their dynamic nature, single OPs are often deemed sufficient for diagnosis of elevated intracranial pressures (ICPs) in nonspecialists' hands. The purpose of this study was to determine the variability of consecutive LP OPs at a large referral center to determine the potential range of variability for a given LP OP. METHODS In this retrospective cohort study, medical records of all patients seen at Mayo Clinic, Rochester, MN, from January 1, 2001, through June 1, 2016, were screened for ≥2 LP OPs within 30 days of each other. Patients with pathologic conditions known to influence ICP were excluded. RESULTS There were 148 eligible patients (39.2% female) with mean age of 63.5 ± 15.5 years and mean body mass index (BMI) 28.0 ± 6.0. The LPs were a mean of 10 ± 9 days apart. Mean OP for the first and second LP was 149 ± 51 mm H2O and 148 ± 48 mm H2O (P = 0.78), respectively, with a mean difference of 1 mm H2O, providing an overall coefficient of repeatability (CR) of 86.4 between consecutive LPs. There was a significant correlation between initial OP and BMI (r = 0.39, P 200 mm H2O had a significantly higher CR of 111.4, compared to OP

BACKGROUND Weight loss is important in the management of idiopathic intracranial hypertension (IIH); however, extremely caloric restrictive diets have been used in published studies, which may not be practical for all patients. We aim to review the weight loss outcomes of IIH patients and weight loss methods used by patients who achieve clinical remission (CR) in a standard clinical setting. METHODS The medical records of IIH patients were retrospectively reviewed. Measures evaluated included: reported symptoms, visual acuity, retinal nerve fiber layer analysis, automated visual fields, optic disc appearance, and cessation of medications. Patients were divided into different outcome groups and weight changes were compared. Patients who achieved CR were contacted to assess weight loss methods used. RESULTS Of the 39 patients included in the analysis, 28 patients (71.8%) achieved CR with concomitant weight loss, 6 patients (15.4%) improved clinically with concomitant weight loss but did not achieve CR. Among the patients with CR, 10 patients (35.7%) were successfully weaned off their medications completely, whereas another 10 patients (35.7%) are in the process of this. Median weight loss from baseline to lowest weight for this group was 10.5 kg (11.5%) achieved using self-directed methods. CONCLUSION This study demonstrated that a large proportion of IIH patients were able to achieve CR or improvement with self-directed weight loss regimens, reinforcing the evidence and the practicality of weight loss in the management of IIH in the clinical setting.

The pathophysiology underlying pseudotumor cerebri syndrome (PTCS) is complex and not well understood. There are clear differences between PTCS in adults and pediatrics. Few and isolated case reports have suggested that adrenal function may be involved, yet no large cohort study has examined this relationship.We conducted a retrospective single-center study of children who presented with a diagnosis of PTCS and had cortisol testing measured between January 2010 and September 2019. We included all subjects meeting the revised PTCS diagnostic criteria after the chart review. Based on morning, random or 1-μg cosyntropin stimulated cortisol levels, adrenal functioning was classified as: (1) insufficient (peak cortisol16 μg/dL and AM cortisol5 μg/dL), (2) at risk (peak cortisol 16-20 μg/dL, AM cortisol 5-13 μg/dL, or random13 μg/dL), or (3) sufficient (peak cortisol20 μg/dL and AM or random cortisol13 μg/dL).A total of 398 individuals were reviewed, and 64 were included for analysis. Of these, 40.6% were men, of mixed race and ethnicity with a mean age of 10.5 (SD 4.7) years. Of these, 23% and 52% had insufficient or at-risk cortisol levels. The majority of those in the insufficient (70%) or at-risk (80%) groups were exposed to topical, nasal, or inhaled glucocorticoids but not systemic. Only 60% and 12% of those with PTCS with insufficient or at-risk cortisol testing, respectively, underwent definitive testing with a stimulation test.Glucocorticoid use and hypocortisolism are prevalent in PTCS and need consideration as a potential underlying cause. Most children had insufficient or at-risk cortisol levels, and many did not undergo further testing/workup. Children who present with PTCS, particularly young, males should be evaluated for adrenal insufficiency and its risk factors, including nonsystemic steroids. Prospective studies are necessary to further evaluate the effect of cortisol in relation to pediatric PTCS.

PURPOSE Idiopathic intracranial hypertension (IIH) is raised intracranial pressure without any identifiable etiology. The inner ear structures are susceptible to cerebrospinal fluid (CSF) pressure changes because of connections between the CSF space and the labyrinth to explain the audiovestibular symptoms, such as pulsatile tinnitus or dizziness, reported in 50% to 60% of these patients. The aim of this study was to investigate the vestibular functions using cervical vestibular evoked myogenic potentials in IIH. METHODS Cervical vestibular evoked myogenic potentials were recorded in 30 patients with IIH before lumbar puncture. Thirty healthy volunteers constituted the control group. The latencies of peaks p13 and n23 and peak-to-peak amplitude of p13-n23 were measured. RESULTS Responses were gathered bilaterally from all healthy controls. In 30 patients with IIH, 49 responses could be gathered from 60 tests (81.7%). The potential was absent bilaterally in five and unilaterally in one patient. When recorded, the latency and amplitude values of the responses of the patients were not significantly different from the healthy controls (P > 0.005). A correlation between CSF pressure and response persistence could not be determined. CONCLUSIONS Cervical vestibular evoked myogenic potentials are affected in patients with IIH and the main finding is the absence of the responses. Increased intracranial pressure causing sound transmission changes within the inner ear can affect the saccular afferents and may end up with absent responses on air-conducted cVEMP recordings. To comment on the correlation between the CSF pressure and cVEMP changes, successive cVEMP recordings with longitudinal CSF pressure monitoring seem necessary.

BACKGROUND The tortuosity of the optic nerve can be quantified radiologically by measuring the angle of optic nerve deformation (the "optic nerve angle" [ONA]). In patients with idiopathic intracranial hypertension (IIH), lowering the intracranial pressure (ICP) to a normal range by lumbar puncture leads to straightening of the optic nerve and an increase in the measured sagittal ONA on MRI. It is uncertain whether there is any correlation between ONA and cerebrospinal fluid (CSF) opening pressure or visual function. METHODS Retrospective study of patients with and without IIH who had MRI of the brain followed by lumbar puncture with CSF opening pressure within 24 hours of MRI. Before LP and within 24 hours of MRI of the brain, all patients with IIH had neuro-ophthalmologic assessment including visual acuity, Humphrey Visual Field (HVF), and fundus photography. Sagittal ONA was measured on multiplanar T2-SPACE images on a DICOM viewer. Papilledema on the fundus photographs was graded using the Frisen scale. RESULTS Fifty-four patients with IIH and 30 unmatched controls were included. The IIH group was 6.3 years younger (95% CI 2.4-10.3, P = 0.002), had 8.7 kg/m2 higher body mass index (4.9-12.5, P < 0.001), and 26.3% more women (P = 0.011) compared with controls. In both eyes, the ONA was significantly smaller in patients with IIH by 12° compared with controls (7°-17°, P < 0.00001). In the IIH group, no correlation between ONA and the CSF opening pressure was present in either eye (right eye r = 0.19, P = 0.15; left eye r = 0.18, P = 0.19) The ONA did not correlate with logarithm of the minimum angle of resolution visual acuity (right eye r = 0.26, P = 0.063; left eye r = 0.15, P = 0.27), HVF mean deviation (right eye r = 0.0059, P = 0.97; left eye r = -0.069, P = 0.63), or Frisen grade (Spearman's rho right eye 0.058, P = 0.67; left eye 0.14, P = 0.30). CONCLUSIONS The ONA is significantly smaller in patients with IIH compared to controls, but does not correlate with CSF opening pressure, severity of papilledema, or visual function. The ONA may be useful in identifying patients with raised ICP, but not necessarily those with a poor visual prognosis.

Background and importance Idiopathic intracranial hypertension, also known as pseudotumor cerebri, often presents with severe headache and associated vision loss. Venous outflow obstruction has been noted as a prominent etiologic factor in many cases, and previous anatomic studies have shown that the internal jugular (IJ) vein at the skull base can be prone to compression by the neighboring bony structures. Clinical presentation We present a case of 13-yr-old male with a multifactorial intracranial hypertension including compression of the IJ vein by the transverse process of C1. Computerized tomography angiographic imaging revealed bilateral stenosis of the IJ veins due to compression from the transverse processes of C1. Medical management and shunt were attempted without resolution of symptoms. A hemodynamically significant stenosis at the right IJ was confirmed with manometry and so the C1 transverse process was resected and a stent placed endovascularly with resolution of pressure gradient and clinical symptoms. Conclusion Contribution of C1 compression to this patient's intracranial hypertension suggests that evaluation for IJ compression below the skull base may be needed to identify the underlying cause of intracranial hypertension in certain patients. Furthermore, surgical decompression of the IJ vein may be required as part of the treatment strategy. If venous stenting is being considered, this decompressive step must be taken before stenting is performed. We offer this case as evidence that decompression of the IJ vein by C1 lateral mass resection can be an effective and novel technique in the repertoire of neurosurgical management of intracranial hypertension.

BACKGROUND The continued increase in idiopathic intracranial hypertension (IIH) prevalence has many implications for societal health care. Its potential vision-threatening consequences make ophthalmologists key players in its diagnosis and management. Newer technology such as optical coherence tomography angiography (OCT-A) enables evaluation of the branching complexity of the peripapillary capillary plexus, a region where accurate imaging via fluorescein angiography was previously limited. METHODS A cross-sectional, observational study of 23 (46 eyes) consecutive patients with IIH. Peripapillary total vasculature was recorded using commercial OCT-A en face vessel density mapping. In addition, OCT-A blood flow slab was compared with papilledema grading. OCT-A images were analyzed using a customized image analysis protocol using ImageJ software (v1.51w) and Photoshop software (Adobe Systems, CA). SPSS software version 25 was used for statistical analysis (SPSS Inc, IBM, Chicago, IL). RESULTS Skeletonized vessel density peripapillary capillary plexus was significantly associated with Frisen papilledema grades, OCT retinal nerve fiber layer (RNFL), and macular ganglion cell layer (GCL) thickness with a P < 0.001, P = 0.022, and P = 0.006, respectively. Every point increase in grade was correlated with a decrease of 9.1 pixels/mm2 in vessel density (R = 0.512, β = -0.115 ± 0.029; P < 0.001). Increased papilledema was correlated with an increased retinal blood flow percentage (R = 0.300, β = 2.114 ± 1.013; P < 0.05) and decreased choroidal blood flow (CBF) percentage (R = 0.300, β = 2.114 ± 1.013; P < 0.05). Every point increase in grade was correlated with a decrease in CBF by 47.4%, as calculated using a linear best-fit line inclusive for all of the data points. CONCLUSIONS OCT-A allows for effective visualization and quantification of the peripapillary retinal vasculature. Our results demonstrate a correlation between skeletonized peripapillary density and papilledema grading, OCT RNFL thickness, and GCL thickness. In addition, we show a significant negative correlation between CBF and papilledema grading. These changes provide key findings regarding the pathophysiology of optic neuropathy in papilledema and highlight the potential of OCT-A as a diagnostic tool for papilledema and a clinical marker for detecting early optic nerve damage.

Background Determining an optimal location within the right atrium (RA) for placement of the distal ventriculoatrial (VA) shunt catheter offer several operative challenges that place patients at risk for perioperative complications and downstream VA shunt failure. Utilizing transesophageal echocardiography (TEE) guidance to place distal VA shunt catheters may help to circumvent these risks. Objective To review our current practice of VA shunt insertion using TEE guidance. Methods A retrospective review of all consecutive patients who underwent VA shunt procedures between December 19, 2016 and January 22, 2019, during which time intraoperative TEE was used for shunt placement was performed. Data on the time required for shunt placement and total procedure time, baseline echocardiography findings, and short- and long-term complications of shunt placement were assessed. Results A total of 33 patients underwent VA shunt procedures, with a median follow-up time of 250 (88-412) d. The only immediate complication related to shunt placement or TEE use was transient ectopy in 1 patient. The mean time for atrial catheter insertion was 12.6 ± 4.8 min. Right-heart catheters were inserted between the RA-superior vena cava junction and 22 mm within the RA in all but 3 procedures. A total of 7/33 patients (21%) underwent shunt revision. Indications for revisions included distal clots, proximal obstruction, positive blood culture, and shunt valve revision. No other complications of VA shunt insertion were reported. Conclusion VA shunt insertion using TEE allows for precise distal catheter placement. Early patient experience confirms this technique has a low complication rate.

Optic nerve sheath fenestration (ONSF) is a surgical technique commonly and effectively employed in the treatment of recalcitrant, vision-threatening papilledema. Unilateral blindness, a known risk of unilateral ONSF widely cited with 1%-2% incidence. In this case, a 58-year-old patient who presented symptomatic with bilateral hemorrhagic papilledema unresponsive to medical therapy underwent uneventful unilateral ONSF via standard superomedial eyelid crease incision. The patient presented postoperatively with severe bilateral vision loss. Workup revealed widespread metastatic malignant disease involving the optic chiasm and optic nerve sheath.

Idiopathic intracranial hypertension is a syndrome of increased intracranial pressure of unclear etiology that most often occurs in obese women of childbearing age but can also occur in men, children, and older adults. This article reviews the diagnostic criteria, clinical features, neuroimaging findings, differential diagnosis, and management options for this condition.Recent population studies have found that the annual incidence of idiopathic intracranial hypertension is increasing in association with obesity rates, whereas recent scientific studies indicate a possible role for androgen sex hormones and adipose tissue in the pathogenesis of the disease. Prospective clinical trials have demonstrated a role for weight loss, acetazolamide, and topiramate in the management of mild disease. A recently begun randomized multicenter trial of surgical interventions will provide insight into the indications for surgical intervention, optimal timing and choice of intervention, and long-term outcomes.Idiopathic intracranial hypertension is a disorder producing symptoms and signs of increased intracranial pressure in the absence of an alternative cause. The main goals of treatment are to preserve visual function and alleviate symptoms, which can usually be achieved with a combination of weight loss, medical therapies, and surgical interventions depending on the severity of symptoms and vision loss, response to treatment, and subsequent clinical course.

The aims of our study were to refer to the complex relationship between idiopathic intracranial hypertension (IIHT) and cerebral sinovenous thrombosis (CSVT), and to determine the differences and commonalities between the patients with and without CSVT in their etiology, along with documenting the uncertainties in concluding on the diagnosis and treatment of these patients.IIHT was diagnosed according to Dandy criteria, while CSVT was screened for by way of a cranial magnetic resonance imaging for all patients and cranial magnetic resonance venography only if the magnetic resonance imaging was nebulous or there was a family history.We retrospectively evaluated a total of 26 patients (9 of whom had CSVT) diagnosed with IIHT between 2014 and 2018. A total of 9 patients with concurrent CSVT were described as suffering from vascular IIHT, while the remaining 17 were described as suffering from other IIHT. Demographic characteristics were similar in both groups (mean age: 12 vs. 11; male/female ratio: 2/7 vs. 5/12 in vascular IIHT and other IIHT, respectively). Clinical findings, cerebrospinal fluid-opening pressure values, and pathologies of etiology were also similar (vitamin D deficiency: 66% vs. 52%; vitamin B12 deficiency: 11% vs. none; iron deficiency: 22% vs. 11%; obesity: 22% vs. 23%). A mixture of acetazolamide, topiramate, anticoagulant therapy, and acetylsalicylic acid were given according to the diagnoses.CSVT is a common clinical entity among the causes of IIHT, and it should be taken into consideration in this patient group. However, there is a need for a common guideline for laboratory and imaging methods to understand the etiopathogenesis of childhood IIHT and determine the patients at risk.

BACKGROUND The resistivity index (RI) in cerebral venous sinus stenosis (VSS) has not been studied in patients with idiopathic intracranial hypertension (IIH). OBJECTIVE To evaluate the role of RI measured by quantitative magnetic resonance venogram (QMRV) as a noninvasive tool in the diagnosis of venous hypertension associated with VSS in IIH. METHODS Retrospective evaluation of 13 consecutive IIH patients who underwent venous sinus stenting at our institution between 2013 and 2018.Patients' demographics, clinical presentation, cerebral mean venous sinus pressure (MVP), and RI both pre- and poststenting were recorded. The baseline RI was also compared to a control group. RESULTS Among 13 patients of IIH, 11 had unilateral VSS in dominant sinus, whereas 2 had bilateral VSS. RI was significantly higher in IIH patients compared to the control group in the superior sagittal (SSS) and transverse sinuses (TS) (0.21 vs 0.11, P = .01 and 0.22 vs 0.13, P = .03, respectively). The MVP (in mm Hg) decreased significantly after venous sinus stenting in the SSS (41.9 to 22.5, P

To explore the incidence of and potential risk factors for developing persistent low-pressure syndrome after lumbar puncture (LP) in patients with idiopathic intracranial hypertension (IIH), as measured by use of blood patches.A retrospective chart review was conducted of patients with definitively diagnosed IIH by clinical examination and LP, comparing them to patients with multiple sclerosis (MS) as controls who also received diagnostic LPs. Demographic, clinical, and radiological data were collected for each patient. The main outcome measure was the rate of post-LP blood patches in IIH patients compared with MS patients. Secondary outcome measures were the likelihood of undergoing an epidural blood patch related to age, body mass index, volume removed, opening pressure, the difference between opening and closing pressure, and the level of puncture within the IIH cohort.One hundred four IIH patients and 149 MS patients were included in the study. Among IIH patients, 12/104 (11.5%) underwent an epidural blood patch after LP as compared to 8/149 (5.4%) of the MS control patients (P = 0.086). Within the IIH population, none of the clinical or LP parameters were significantly correlated with increased risk of needing a blood patch.The incidence of low-pressure syndrome, as measured by blood patches, is similar in IIH patients and MS controls. This suggests that having elevated intracranial pressure before an LP is not protective against developing postpuncture low-pressure syndrome, contrary to common assumptions.

A 55-year-old obese woman with a history of migraine and tension-type headaches presented with headache for 5 days with acute worsening for 1 day. The headache was throbbing, holocephalic, and maximal at the left temple, and worsened with both standing and lying down and with exertion. The headache was initially mild for 4 days before acutely worsening in severity and waking her from sleep with associated nausea and photophobia. Unlike her typical headaches, it did not improve with over-the-counter analgesics and was more prolonged. There were no associated visual complaints, hearing changes or phonophobia, jaw claudication, weakness, numbness, or paresthesias.

Purpose The clinical significance of postoperative pseudomeningocele formation following optic nerve sheath fenestration (ONSF) has not been fully characterized. A literature review identifies 9 previously published cases the authors believe demonstrate pseudomeningocele formation and approximately 19 other similar findings that were either transient or less defined blebs. This study was undertaken to more clearly define the clinical, radiographic, and histopathologic features associated with this entity. Methods Sixteen-year, single-center, retrospective chart review of all ONSF cases performed by 2 surgeons. Clinical data, intracranial pressure, radiographic imaging, and histopathology of clinically detected pseudomeningoceles after ONSF were reviewed. Results Eighty-six eyes in 57 patients underwent ONSF (28 unilateral, 12 bilateral sequential, 17 bilateral simultaneous). Forty-nine of 57 patients had elevated intracranial pressure preoperatively (41 idiopathic intracranial hypertension, 4 venous thrombosis, 2 meningitis, 1 arteriovenous malformation, and 1 sarcoid). In 32 patients undergoing postoperative imaging, 4 eyes (4.7%) in 4 patients developed well-defined pseudomeningoceles, of which 3 were symptomatic and 2 required surgical revision. Each pseudomeningocele developed in the setting of elevated preoperative intracranial pressure (350, 360, 430, 500 mm H20). Magnetic resonance imaging and/or computed tomography revealed sharply demarcated fluid-filled sacs adjacent to the optic nerve. The contents of these sacs were hypointense on T1-weighted imaging, hyperintense on T2-weighting, variably enhanced with contrast, and hypointense on fluid attenuated inversion recovery, and were thus consistent with cerebrospinal fluid. Histopathologic analysis of one of these outpouchings demonstrated an acellular, fibrocollagenized lining consistent with pseudomeningocele. Three eyes in 3 additional patients had less well-defined findings on imaging interpreted as bleb-like or cyst-like change. Conclusions Pseudomeningoceles following ONSF may be asymptomatic or may cause symptomatic orbital mass effect and rarely visual loss, amendable to surgical excision. Post-ONSF pseudomeningoceles are identified on computed tomography or magnetic resonance imaging to occur at the locations of fenestration sites and contain cerebrospinal fluid communicating with the subdural space that may act as a "filtration" bleb in some cases. Imaging findings may represent a spectrum spanning intraorbital cerebrospinal fluid leakage, partial walling off of bleb, or fully developed cysts. Resection of optic nerve pseudomeningoceles is considered in symptomatic cysts or eyes with papilledema that fails to improve.

This review summarizes the recent literature on diagnostic error relevant to the practice of neuro-ophthalmology, and evaluates the potential for increased access to neuro-ophthalmology to decrease rates of diagnostic error, thereby reducing medical harm and spending on unnecessary tests and treatments.Despite medical advances, current research continues to show alarmingly high rates of diagnostic error. Research into diagnostic error shows that many of these errors can be traced to cognitive errors. Recent studies on diagnostic problems relevant to neuro-ophthalmology, including studies of misdiagnosis of posterior communicating artery aneurysms, idiopathic intracranial hypertension, optic neuritis, and optic nerve sheath meningiomas, have identified major causes of diagnostic error in these conditions.Studies on diagnostic error in neuro-ophthalmologic conditions show that prior to evaluation by a neuro-ophthalmologist, patients may undergo unnecessary tests and treatments that are costly and potentially harmful. Further research on diagnostic error will inform better utilization of neuro-ophthalmologists as a resource to decrease diagnostic error.

We aim to provide a comprehensive and updated review on idiopathic intracranial hypertension (IIH), including the most current studies and treatment options. Special focus will be put on recent theories about the pathophysiology, and on newer prospective studies on treatment modalities.The Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) provided evidence supporting acetazolamide as a well tolerated first-line therapy in IIH patients with mild vision loss. Recent studies have shown venous sinus stenting as a well tolerated and effective surgical alternative for patients with refractory IIH.Idiopathic intracranial hypertension is a vision-threatening disorder that predominantly affects obese women of childbearing age. This disorder is becoming more prevalent as the obesity epidemic continues to increase. As our understanding of this disorder continues to evolve, diagnosis and management approaches have changed over time. However, the pathogenesis for IIH remains unclear. Several theories have been proposed, including abnormalities in cerebrospinal dynamics, metabolic causes and genetics. The diagnostic criteria are based on the revised Dandy criteria. Traditionally, treatment was based on clinical experiences and retrospective studies. However, a new, prospective, randomized, controlled trial, the IIHTT, provided evidence-based data to help guide medical therapy. Additionally new, prospective studies are underway for the different surgical alternatives to treat IIH.

The recent discoveries of the glymphatic and lymphatic systems of the brain have helped advance our understanding of CSF physiology and may allow new insights in the understanding of idiopathic intracranial hypertension (IIH). The clinical and radiologic presentations of IIH appear to be related to congestion of the glymphatic system associated with an overflow of the lymphatic CSF outflow pathway. By revisiting the role of “vascular arachnoid granulations” in the brain, we hypothesize that an initial impairment of the transport of interstitial fluid from the glymphatic system to the venous blood of the dural sinuses may trigger the hydrodynamic cascade of IIH. Furthermore, we speculate that, similar to other water-exchange systems in the brain, a specific subtype of aquaporin is involved in this transport. This theory may eventually help to provide an underlying explanation for IIH and its associated conditions, since in most of them, the expression of several aquaporins is altered.

ObjectiveTo report the clinical and radiologic features of 3 patients in whom prolonged vigorous coughing/Valsalva triggered the development of pseudotumor cerebri syndrome (PTCS) that persisted even after the coughing attacks had stopped and to discuss how this novel proximate trigger supports a “hydraulic model” of PTCS pathogenesis.MethodsThis was a retrospective case series of 3 patients seen between January 2011 and July 2017 in a tertiary care neuro-ophthalmology clinic.ResultsThree female patients (ages 13, 28, and 21 years) were asymptomatic until developing prolonged fits of profuse coughing. Two patients had upper respiratory tract infections, and 1 patient had a prolonged asthma exacerbation. Symptoms typical of PTCS began 2 weeks into the coughing attacks, and patients were examined 1 to 2 weeks after coughing had ceased. One patient was overweight and 2 were obese. All 3 patients had papilledema, and 1 of 3 patients had lateral rectus palsy. Two of 3 patients had visual field defects, and all 3 patients had dural venous sinus stenoses (DVSS) on venous imaging. Lumbar puncture (LP) confirmed elevated opening pressures in 2 of 3 patients and brought immediate and sustained relief of symptoms; LP attempts failed in the third patient because of body habitus (body mass index 68 kg/m2). All patients were given acetazolamide and advised to work on weight loss. At follow-up 3 to 6 months later, all 3 patients had complete remission of their clinical syndromes and resolution of their DVSS on neuroimaging.ConclusionsIn susceptible individuals, prolonged coughing/Valsalva can trigger PTCS in the setting of collapsible (nonfixed) DVSS. This new observation supports a hydraulic model of PTCS pathogenesis.

Idiopathic intracranial hypertension (IIH) is a syndrome characterized by increased intracranial pressure (ICP), the absence of structural lesions on neuroimaging, and normal cerebrospinal fluid composition. Cerebral venous sinus thrombosis (CVST) is a common cause of increased ICP and can be differentiated from IIH with magnetic resonance venography. We describe a young woman with typical IIH who underwent lumbar puncture and was treated with a short course of high-dose corticosteroids followed by acetazolamide. She subsequently developed CVST, subarachnoid hemorrhage, and stroke. Risk factors that may have resulted in CVST are discussed.

Pseudotumor cerebri syndrome (PTCS) is a disorder defined by increased intracranial pressure in the absence of an intracranial space-occupying lesion. This retrospective study aimed to examine the outcomes in children with PTCS.Data was collected retrospectively from the charts of consecutive pediatric patients treated for PTCS at our hospital between 2000 and 2007 (60 patients; 36 females, 24 males).Forty-six patients (76.6%) responded well to acetazolamide therapy, with full resolution of symptoms, including papilledema (average treatment duration 1 year; range: 1 month-5 years). Of the 14 patients with no response to treatment, 9 (23.4%) required surgical intervention. Nonresponders tended to be younger at presentation (8.7 vs 11.5 years, P = 0.04). Twelve patients (26%) experienced relapse after acetazolamide was discontinued. The group that experienced relapse was significantly younger than the nonrelapsers (8.9 vs 12.1 years, P0.05).Younger age at presentation with PTCS was found to be a risk factor for treatment failure or relapse.

Idiopathic intracranial hypertension, otherwise known as primary pseudotumor cerebri syndrome (PTCS), most frequently occurs in obese women of childbearing age. However, children may be affected as well. This review will address recent findings regarding demographics, diagnosis, and treatment of pediatric PTCS. Prepubertal children with primary PTCS have an equal sex distribution and less frequent obesity compared with adult patients. However, female gender and obesity are risk factors for primary PTCS in postpubertal children. Compared with adults, children with PTCS more frequently present with ocular motility deficits and more often have associated medical conditions that increase the risk of developing PTCS. Visual field testing may be unreliable, and the optimal modality to monitor visual function is unknown. MRI shows signs of elevated intracranial pressure (ICP) in children with PTCS similar to that of adults. It has now been established that elevated ICP in children ≤18 years old is greater than 25 cm H20 in nonobese, nonsedated children, and greater than 28 cm H2O in the remainder. Optical coherence tomography (OCT) may be used to distinguish pseudopapilledema from papilledema, monitor response to treatment in preverbal children, and identify patients with PTCS at risk for permanent visual loss. However, the precise role of OCT in the management of pediatric PTCS remains to be determined.

Objective:To quantify the risk of secondary pseudotumor cerebri syndrome (PTCS) with fluoroquinolones.Methods:A case-control study of people 15–60 years of age from the LifeLink Database (QuintilesIMS, Parsippany, NJ) was conducted. Cases had the first ICD-9-CM code for benign intracranial hypertension (BIH) as well as having received a procedure code for an MRI or CT scan and a lumbar puncture within 15 days or 30 days of the BIH code. For each case, 10 controls were selected using density-based sampling. Current users of fluoroquinolones received a prescription within 15 days or 30 days of the date of the diagnosis. For the sensitivity analysis, risk periods for 30 and 60 days were also examined. Adjusted rate ratios (RRs) were computed from a conditional logistic regression model.Results:From a cohort of 6,110,723 people, there were 339 cases of PTCS and 3,390 corresponding controls. In the primary analysis, the adjusted RR for current users of fluoroquinolones for both the 15-day and 30-day definitions were 5.67 (95% confidence interval [CI] 2.72–11.83) and 4.15 (95% CI 2.29–7.50), respectively. The risk with tetracycline antibiotics was also increased, with RRs for 15 and 30 days of current use of 2.68 (0.89–8.11) and 3.64 (1.67–7.91), respectively.Conclusion:Our study suggests an increase in the risk of PTCS with current users of fluoroquinolones. Although this adverse event is rare, patients who experience symptoms of raised intracranial pressure including headaches, tinnitus, and double vision while taking fluoroquinolones should seek medical attention.

Fulminant idiopathic intracranial hypertension (FIIH) is a subtype of idiopathic intracranial hypertension (IIH) characterized by rapid, severe, progressive vision loss. Surgical intervention is often performed either as a cerebrospinal fluid (CSF) shunt procedure or an optic nerve sheath fenestration or, at times, both. These surgical procedures carry a significant risk of morbidity and failure. We present 2 patients in whom a temporary lumbar drain was successfully used in the management of medically undertreated pediatric FIIH, and circumvented the need for surgical intervention.