Definite Idiopathic Intracranial Hypertension Research Articles

The additive value of complementing diagnostic idiopathic intracranial hypertension criteria by MRI – an external validation study

BackgroundRecently, diagnostic criteria including a standardized MRI criterion were presented to identify patients suffering from idiopathic intracranial hypertension (IIH) proposing that IIH might be defined by two out of three objective findings (papilledema, ≥ 25 cm cerebrospinal fluid opening pressure (CSF-OP) and ≥ 3/4 neuroimaging signs).MethodsTo provide independent external validation, we retrospectively applied the proposed diagnostic criteria to our cohort of patients with clinical suspicion of IIH from the Vienna IIH database. Neuroimaging was reevaluated for IIH signs according to standardized definitions by a blinded expert neuroradiologist. We determined isolated diagnostic accuracy of the neuroimaging criterion (≥ 3/4 signs) as well as overall accuracy of the new proposed criteria.ResultsWe included patients with IIH (n = 102) and patients without IIH (no-IIH, n = 23). Baseline characteristics were balanced between IIH and no-IIH groups, but papilledema and CSF-OP were significantly higher in IIH. For the presence of ≥ 3/4 MRI signs, sensitivity was 39.2% and specificity was 91.3% with positive predictive value (PPV) of 95.2% and negative predictive value (NPV) 25.3%. Reclassifying our cohort according to the 2/3 IIH definition correctly identified 100% of patients without IIH, with definite IIH and suggested to have IIH without papilledema by Friedman criteria, respectively.ConclusionThe standardized neuroimaging criteria are easily applicable in clinical routine and provide moderate sensitivity and excellent specificity to identify patients with IIH. Defining IIH by 2/3 criteria significantly simplifies diagnosis without compromising accuracy.

Clinico demographic profile and short-term outcome of Pediatric Idiopathic Intracranial Hypertension in a tertiary care hospital of Bangladesh

Background: Diagnosis of IIH (idiopathic intracranial hypertension) in children is challenging. The pathogenesis of IIH is still largely unknown. The aim of this study to evaluate the clinico-demographic profile and short-term outcome of IIH among Bangladeshi children. Methodology: This observational cohort study was done in pediatric Neurology Department, National Institute of Neurosciences and Hospital Bangladesh from January 2018 to July 2019. Total 19 cases were enrolled who were clinically suspected and/or had normal CSF with opening pressure (OP) above 250 mmH2O. They were further categorized as “definite IIH”, definite IIH without papilledema, and “probable IIH”. Clinical, radiological profile and short-term outcome were analyzed by SPSS version 22. Result: Among them most were probable IIH (74%). Episodic (57.9%), moderate, diffuse headache was the predominant symptom along with vomiting, double vision, unilateral VI nerve palsy and papilledema. Obesity was found in 21% cases. Mean CSF pressure was 21.84+7.62cm H2O and found high in definite IIH. All the cases were treated with acetazolamide and serial LP was done in 4(15.8%) cases. Definite IIH requires longer hospital stay (6.42 ± 2.87days). Recurrence was observed in 10.5% cases and visual impairment was found in 15.8% cases. Conclusion: This study found wide range of diversity between clinical feature and CSF opening pressure in Pediatric IIH and diagnostic confirmation was challenging. Initial treatment response was satisfactory with a low recurrence rate. J Bangladesh Coll Phys Surg 2024; 42: 161-168

A New Perspective on the Pathophysiology of Idiopathic Intracranial Hypertension: Role of the Glia-Neuro-Vascular Interface.

Idiopathic intracranial hypertension (IIH) is a neurological disease characterized by symptoms and signs of increased intracranial pressure (ICP) of unknown cause. Most attention has been given to the role of cerebrospinal fluid (CSF) disturbance and intracranial venous hypertension caused by sinus vein stenosis. We previously proposed that key pathophysiological processes take place within the brain at the glia-neuro-vascular interface. However, the relative importance of the proposed mechanisms in IIH disease remains unknown. Modern treatment regimens aim to reduce intracranial CSF and venous pressures, but a substantial proportion of patients experience lasting complaints. In 2010, the first author established a database for the prospective collection of information from individuals being assessed for IIH. The database incorporates clinical, imaging, physiological, and biological data, and information about treatment/outcome. This study retrieved information from the database, asking the following research questions: In IIH subjects responding to shunt surgery, what is the occurrence of signs of CSF disturbance, sinus vein stenosis, intracranial hypertension, and microscopic evidence of structural abnormalities at the glia-neuro-vascular interface? Secondarily, do semi-quantitative measures of abnormal ultrastructure at the glia-neurovascular differ between subjects with definite IIH and non-IIH (reference) subjects? The study included 13 patients with IIH who fulfilled the diagnostic criteria and who improved following shunt surgery, i.e., patients with definite IIH. Comparisons were done regarding magnetic resonance imaging (MRI) findings, pulsatile and static ICP scores, and immune-histochemistry microscopy. Among these 13 IIH subjects, 6/13 (46%) of patients presented with magnetic resonance imaging (MRI) signs of CSF disturbance (empty sella and/or distended perioptic subarachnoid spaces), 0/13 (0%) of patients with IIH had MRI signs of sinus vein stenosis, 13/13 (100%) of patients with IIH presented with abnormal preoperative pulsatile ICP [overnight mean ICP wave amplitude (MWA) above thresholds], 3/13 (23%) patients showed abnormal static ICP (overnight mean ICP above threshold), and 12/13 (92%) of patients with IIH showed abnormal structural changes at the glia-neuro-vascular interface. Comparisons of semi-quantitative structural variables between IIH and aged- and gender-matched reference (REF) subjects showed IIH abnormalities in glial cells, neurons, and capillaries. The present data suggest a key role of disease processes affecting the glia-neuro-vascular interface.

Predictive Value of International Classification of Diseases Codes for Idiopathic Intracranial Hypertension in a University Health System.

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. 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. 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. 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.

Apparent diffusion coefficient echoplanar imaging maps of the optic nerves in childhood idiopathic intracranial hypertension.

Dueto motion artifacts, optic nerve (ON) findings of idiopathic intracranial hypertension (IIH) can easily be overlooked on T2-weighted (T2w) turbo spin-echo sequence. This study aimed to investigate the contribution of the apparent diffusion coefficient (ADC) map derived from the interleaved multi-shot (IMS) echoplanar imaging (EPI) to the ON findings of IIH in children. MRIs of 42 pediatric patients aged 3-17 years diagnosed with definite IIH according to modified Dandy criteria were retrospectively re-evaluated, between April 2018 and January 2021. Forty-two age- and sex-matched subjects with no IIH symptoms and reported as normal were included as a control group. ON sheath distance (ONSD) on the ADC map (p = .005) and vertical tortuosity (p = .030) were significant single MRI parameters for predicting IIH. Other single parameters were not statistically significant. Flattening of the posterior sclera (FPS) and ON protrusion (ONP) were observed on ADC maps more frequently than T2w (42.8% vs. 19% and 19% vs. 4.7%, respectively). From combined MRI parameters, the presence of at least one of ONP, FPS, or ONSD on ADC maps (p = .001) showed greater significance than the presence of T2w (p = .048). The predictive values of other MRI findings evaluated together were not statistically significant (p> .05). This study's results show that due to the short readout time and less sensitivity to motion, the ADC map obtained from IMS-EPI can contribute to orbital findings of IIH, in addition to T2w.

Visual outcomes in idiopathic intracranial hypertension in children

ObjectiveThe purpose of this study was to report the clinical characteristics and long-term visual outcomes in a cohort of children with idiopathic intracranial hypertension (IIH). DesignRetrospective, observational cohort study. ParticipantsConsecutive children who met the diagnostic criteria for definite IIH at a tertiary care pediatric hospital between 2009 and 2020. MethodsThe charts of pediatric patients with IIH were reviewed. The main outcome measure was long-term visual impairment, with an analysis of clinical features by age and risk factors for a poor visual outcome. ResultsThere were 110 children (75 females) with IIH. At presentation, younger children with IIH were less likely to present with headaches (p = 0.01) and more likely to be asymptomatic (p = 0.03). There was a strong association with female sex (p < 0.001) and higher body mass index (p < 0.001) in adolescents in comparison to younger children. Of the 90 patients with long-term visual outcome data, only 8 (9%) had evidence of mild visual impairment (1 loss of visual acuity, 7 loss of visual field) with no cases of severe visual impairment. On risk factor analysis, the only variable associated with a poor visual outcome was greater severity of papilledema at diagnosis. ConclusionsIn this large series of pediatric IIH, the long-term visual outcomes were favourable, with evidence of mild visual impairment in less than 10% of patients.

Unilateral papilledema in idiopathic intracranial hypertension: A rare entity.

Idiopathic Intracranial Hypertension (IIH), also known as pseudotumor cerebri is a clinical syndrome of unknown etiology, which typically affects young, obese women of childbearing age and is characterized by increased intracranial pressure and bilateral papilledema. Unilateral and highly asymmetrical papilledema is a rare presentation in IIH occurring in less than 4% of patients with definite IIH and can poses a diagnostic challenge for the treating physician as it usually raises the suspicion of localized ocular pathology. As per current consensus, papilledema results in stasis of axoplasmic transport due to mechanical compression, leading to secondary vascular changes of venous dilation and hemorrhage. Given this mechanism, the underlying reasons for unilateral and asymmetrical papilledema remain unclear.

Spontaneous Skull Base Cerebrospinal Fluid Leaks and Their Relationship to Idiopathic Intracranial Hypertension.

The association between spontaneous skull base cerebrospinal fluid (CSF) leaks and idiopathic intracranial hypertension (IIH) has been suggested, but its significance remains unclear. To estimate the prevalence of IIH in spontaneous skull base CSF leak patients. Systematic collection of demographics, neuro-ophthalmic and magnetic resonance imaging evaluation of spontaneous skull base CSF leak patients seen pre- and post-leak repair in one neuro-ophthalmology service. Patients with preexisting IIH were diagnosed with definite IIH if adequate documentation was provided; otherwise, they were categorized with presumed IIH. Classic radiographic signs of intracranial hypertension and bilateral transverse venous sinus stenosis were recorded. Thirty six patients were included (age [interquartile range]: 50 [45;54] years; 94% women; body mass index: 36.8 [30.5;39.9] kg/m2), among whom six (16.7%, [95% confidence interval, CI]: [6.4;32.8]) had a preexisting diagnosis of definite or presumed IIH. Of the remaining 30 patients, four (13.3%, 95%CI: [3.8;30.7]) had optic nerve head changes suggesting previously undiagnosed IIH, while one was newly diagnosed with definite IIH at initial consultation. One out of 29 patients with normal findings of the optic nerve head at presentation developed new onset papilledema following surgery (3.4%, 95%CI: [0.1;17.8]) and was ultimately diagnosed with definite IIH. Overall, the prevalence of definite IIH was 19.4% (95%CI: [8.2;36.0]). Striking demographic overlap exists between IIH patients and those with spontaneous CSF leak. Definite IIH was present in approximately 20% of our patients. However, its true prevalence is likely higher than identified by using classic criteria. We therefore hypothesize that an active CSF leak serves as an auto-diversion for CSF, thereby "treating" the intracranial hypertension and eliminating characteristic signs and symptoms at initial presentation.

Predictive Value of International Classification of Diseases Codes for Idiopathic Intracranial Hypertension in a University Health System.

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. 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. 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. 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.

Self-Reported Weight and Height Among Idiopathic Intracranial Hypertension Patients.

Idiopathic intracranial hypertension (IIH) mostly affects overweight and obese women. Severe obesity is associated with poorer visual outcomes in IIH, and weight gain can precipitate IIH and increase the risk of recurrence. Conversely, weight loss can decrease intracranial pressure and is an effective IIH treatment. Therefore, accurate monitoring of weight and body mass index (BMI) is important to help guide the management of IIH patients. Our goal was to compare estimated and measured BMI among patients with presumed IIH and non-IIH controls to determine whether these vital signs should be systematically measured when evaluating patients for IIH. A retrospective chart review was performed of consecutive patients with reported and measured weight and height seen in one ophthalmology-based neuro-ophthalmology clinic for IIH between January 2, 2018 and September 10, 2018. Patients with IIH or presumed IIH were compared to non-IIH controls, matched according to age (±5 years), BMI (±5 kg/m unless ≥40 kg/m), sex, and race. Patients with confirmed IIH were asked to self-report their weight when seen in follow-up and they were weighed to compare their self-reported vs measured percent weight change. We included 379 subjects (140 patients; 239 controls) among whom 75 of the IIH patients were matched to non-IIH controls. Patients with presumed or definite IIH accurately estimated their height and generally underestimated their weight by a median of 1.8 kg (4 lb), resulting in a median BMI underestimate of 0.9 kg/m. There was no difference in BMI underestimation when comparing presumed or definite IIH patients to matched non-IIH controls while controlling for insurance status, smoking, diabetes, and vascular disease (P = 0.66). As BMI increased, all subjects underestimated their BMI more (by 0.9% per 10 measured BMI unit increase), when controlling for age, sex, and race (P < 0.003). Sixteen confirmed IIH patients were seen in follow-up. At initial neuro-ophthalmology consultation, these subjects underestimated their weight by a mean of 3.2%. At last follow-up they underestimated their weight by only 1.2% (P = 0.03). There was no evidence that IIH or presumed IIH patients had a different perception of their weight than non-IIH controls at initial neuro-ophthalmology consultation. Both patients and matched controls tended to underestimate their weight by the same amount, resulting in an overall BMI underestimation of approximately 1% per 10 measured BMI unit increase. Heavier subjects tended to underestimate their body weight and resultant BMI more, and IIH patients tended to estimate their weight more accurately at follow-up. Our results emphasize the need to systematically objectively measure the weight of presumed IIH patients seen in an ophthalmology clinic.

Pediatric Idiopathic Intracranial Hypertension: Age, Gender, and Anthropometric Features at Diagnosis in a Large, Retrospective, Multisite Cohort

To examine anthropometric and maturational characteristics at diagnosis in pediatric idiopathic intracranial hypertension (IIH). Retrospective, international, multisite study. Pediatric patients (2-18 years of age at diagnosis) with IIH. Body mass index (BMI), height, and weight Z-scores; sexual maturation. Cases of IIH were identified retrospectively based on diagnostic code, pediatric neuro-ophthalmologist databases, or both and updated diagnostic criteria (2013) were applied to confirm definite IIH. Anthropometric measurements were converted into age- and gender-specific height, weight, and BMI Z-scores CDC 2000 growth charts. When available, sexual maturation was noted. Two hundred thirty-three cases of definite IIH were identified across 8 sites. In boys, a moderate association between age and BMI Z-scores was noted (Pearson's correlation coefficient, 0.50; 95% confidence interval [CI], 0.30-0.66; P < 0.001; n= 72), and in girls, a weak association was noted (Pearson's correlation coefficient, 0.34; 95% CI, 0.20-0.47; P < 0.001; n= 161). The average patient was more likely to be overweight at diagnosis at age 6.7 years in girls and 8.7 years in boys, and obese at diagnosis at age 12.5 years in girls and 12.4 years in boys. Compared with age- and gender-matched reference values, early adolescent patients were taller for age (P= 0.002 in girls and P= 0.02 in boys). Data on Tanner staging, menarchal status, or both were available in 25% of cases (n= 57/233). Prepubertal participants (n= 12) had lower average BMI Z-scores (0.95±1.98) compared with pubertal participants (n= 45; 1.92±0.60), but this result did not reach statistical significance (P=0.09). With updated diagnostic criteria and pediatric-specific assessments, the present study identifies 3 subgroups of pediatric IIH: a young group that is not overweight, an early adolescent group that is either overweight or obese, and a late adolescent group that is mostly obese. Data also suggest that the early adolescent group with IIH may be taller than age- and gender-matched reference values. Understanding these features of pediatric IIH may help to illuminate the complex pathogenesis of this condition.

Brain Imaging in Idiopathic Intracranial Hypertension.

The primary role of brain imaging in idiopathic intracranial hypertension (IIH) is to exclude other pathologies causing intracranial hypertension. However, subtle radiologic findings suggestive of IIH have emerged with modern neuroimaging. This review provides a detailed description of the imaging findings reported in IIH and discusses their possible roles in the pathophysiology and the diagnosis of IIH. References were identified by searches of PubMed from 1955 to January 2015, with the terms "idiopathic intracranial hypertension," "pseudotumor cerebri," "intracranial hypertension," "benign intracranial hypertension," "magnetic resonance imaging," "magnetic resonance venography," "computed tomography (CT)," "CT venography," "imaging," and "cerebrospinal fluid (CSF) leak." Additional references were identified by hand search of relevant articles. When possible, we extracted the number of patients and control subjects from each study for each radiological finding. When at least 2 studies used the same criteria to define a radiological finding, all patients from these studies were pooled to obtain a mean sensitivity and specificity with 95% confidence interval. Specific neuroimaging findings may suggest long-standing IIH, including empty sella, flattening of the posterior globes, optic nerve head protrusion, distention of the optic nerve sheaths, tortuosity of the optic nerve, cerebellar tonsillar herniation, meningoceles, CSF leaks, and transverse venous sinus stenosis. Although IIH remains a diagnosis of exclusion, the most recently proposed diagnostic criteria have included neuroimaging findings to suggest IIH when major diagnostic criteria are not fulfilled. However, these findings are not diagnostic of IIH, and their presence is not required for the diagnosis of definite IIH. Their incidental discovery on brain imaging should not prompt invasive procedures, unless other signs of IIH, such as papilledema, are present.

Asymmetric papilledema in idiopathic intracranial hypertension.

Very asymmetric papilledema in idiopathic intracranial hypertension (IIH) is rare, and few studies have dealt with this atypical presentation of IIH. Our aim was to describe the clinical and radiologic features of patients with IIH and very asymmetric papilledema. We identified all adult patients from our IIH database with very asymmetric papilledema defined as a ≥2 modified Frisén grade difference between the 2 eyes. Demographic data and initial symptoms were collected, and all brain imaging studies performed at our institution were reviewed. Of the 559 adult patients with definite IIH, 20 (3.6%; 95% confidence interval [CI], 2.3-5.6) had very asymmetric papilledema at initial evaluation. They were older (39 vs 30 years; P < 0.001), had lower cerebrospinal opening pressure (35.5 vs 36 cm of water; P = 0.03), and were more likely to be asymptomatic compared with patients with symmetric papilledema (27% vs 3%; P < 0.001). Visual fields were worse on the side of the highest-grade papilledema (P = 0.02). The bony optic canal was smaller on the side of the lowest-grade edema in all 8 patients (100%) in whom the imaging was sufficient for reliable measurements (P = 0.008). IIH with very asymmetric papilledema is uncommon. Very asymmetric papilledema may result from differences in size of the bony optic canals, supporting the concept of compartmentation of the perioptic subarachnoid spaces.

Cognitive function in idiopathic intracranial hypertension: a prospective case–control study

ObjectiveTo explore the extent and nature of cognitive deficits in patients with idiopathic intracranial hypertension (IIH) at the time of diagnosis and after 3 months of treatment.DesignProspective case–control study.SettingNeurological department,...

Factors Determining the Clinical Significance of an “Empty” Sella Turcica

Although often incidental, the "empty" sella turcica can reflect chronically elevated intracranial pressure (ICP). It is particularly common in the setting of idiopathic intracranial hypertension (IIH). This study evaluated which clinical and MRI findings could be used to differentiate patients with chronically elevated ICP from those with incidental empty sella turcica. Forty-five patients with definite IIH and 92 patients with "empty sella" reported on brain MRI were evaluated. Measurements of the sella turcica, diaphragm sella, pituitary gland, infundibulum, and scalp and neck soft tissues were made on MR images. These measurements, age, sex, clinical symptoms, and frequency of previously reported orbital findings of IIH were compared between the IIH and incidental empty sella turcica groups. Measurements on MRI were correlated with patient age in each group. The IIH and incidental empty sella turcica groups had statistically similar sellar, pituitary, and infundibular measurements. The patients with IIH were significantly younger than the patients with incidental empty sella turcica (mean age, 36.1 vs 54.3 years, respectively; p < 0.05); were more likely to report headache (93.3% vs 32.6%; p < 0.05) and visual complaints (66.2% vs 28.3%; p < 0.05); showed greater mean scalp thickness (9.0 vs 6.4 mm; p < 0.05) and neck soft-tissue thickness (19.5 vs 13.8 mm; p < 0.05); and were more likely to have an orbital finding suggestive of IIH (93% vs 14%). Age modestly correlated with the width of the diaphragm sella (r = 0.53) in the IIH group only. The significance of the MRI finding of an empty sella turcica can be determined using a combination of clinical and imaging findings.

MRI Findings in Idiopathic Intracranial Hypertension (IIH) Compared with Cerebral Venous Thrombosis (CVT) (P07.267)

Objective: To compare MRI findings suggestive of elevated ICP in IIH patients with transverse sinus stenosis (TSS) versus CVT patients. Background Numerous publications have emphasized that IIH patients often have MRI findings suggestive of raised ICP in addition to TSS on vascular imaging. Design/Methods: Among 240 patients who underwent standardized contrast-enhanced brain MRI and contrast-enhanced MRV at our institution between 9/2009 and 9/2011, 60 with abnormal imaging findings on MRV were included: 29 patients with definite IIH, and 31 with definite CVT. Medical records were reviewed, and imaging studies were prospectively evaluated by the same neuroradiologist to assess for presence or absence of TSS, site of CVT if present, orbital findings of posterior globe flattening, optic nerve sheath dilation/tortuosity, and the size/appearance of the sella turcica. Results: 29 IIH patients (28 women, 19 African-Americans, mean-age 30.6, mean-BMI 35.2) had bilateral (28) or unilateral (1) TSS. 31 CVT patients (19 women, 13 African-Americans, mean-age 45.6, mean-BMI 28.6) had thrombosis of the sagittal (3), sigmoid (3), cavernous (1), unilateral transverse (7), or multiple (16) sinuses or cortical veins (1). 14 CVT patients presented with raised ICP, 15 had focal symptoms, 2 were asymptomatic. IIH patients had larger sella turcicas than CVT patients (p Conclusions: Although abnormal imaging findings involving the globes, optic nerves, and sella turcica are more common in IIH patients with TSS than in CVT patients, these findings are not specific for IIH and are found in patients with raised ICP from other causes such as CVT, along with other signs suggestive of raised ICP. Disclosure: Dr. Ridha has nothing to disclose. Dr. Saindane has nothing to disclose. Dr. Bruce has nothing to disclose. Dr. Riggeal has nothing to disclose. Dr. Kelly has nothing to disclose. Dr. Newman has received personal compensation for activities with Biogen Idec. Dr. Biousse has nothing to disclose.

Traitement et pronostic de l’hypertension intracrânienne idiopathique de l’enfant. Étude rétrospective (1995–2009) et revue de la littérature

Idiopathic intracranial hypertension (IIH) may cause severe visual loss due to the optic nerve damage. Routine management involves mainly medical treatment. The aim of this study was to improve diagnosis and management of IIH in children. The medical records of all patients with definite IIH seen at the children's hospital of Toulouse between 1995 and 2009 were reviewed. Cases of secondary intracranial hypertension were included because they did not present any cerebral lesions and underwent a similar therapeutic approach. The clinical and ophthalmological data at the beginning and at the end of their treatment was collected. Eighteen children were included in this study. The average age was 10 years and the sex-ratio was equal to 1. There were 3 cases of secondary idiopathic intracranial hypertension in this pediatric group. The main features encountered were headache (15 children) and diplopia (8 children). Abnormal neurological examination was found for 11 patients with abducens nerve paresis in 8 cases, rachialgia in 6 cases, and neurogenic pains (neuralgia, dysesthesia, paresthesia, hyperesthesia) in the other cases. Papilledema was noted in 16 patients. At the initial phase, loss of visual acuity was documented in 6 patients and altered visual field in nine patients. All patients had a medical treatment. When recurrence occurred, each new treatment was documented, for a total of 23 treatments analyzed. Lumbar puncture was the only treatment for 2 patients. In 16 cases, first-line treatment was acetazolamide and it was the second choice in 1 case, with an average dosage of 11.2mg/kg and a mean duration of 2.5 months (15 treatments could be analyzed). This treatment was effective in 11 cases out of 15. Steroids were the initial treatment in 4 cases and second-line treatment in 4 cases (after failed acetazolamide therapy). The dosage was 1.5-2mg/kg for a mean duration of 1.5 months (6 treatments could be analyzed). This treatment was effective in 5 patients out of 6. One patient had dual therapy. No surgical procedure was necessary in this pediatric cohort. Three patients presented relapses of IIH. The outcome was good with no residual visual impairment in the 13 patients analyzed. One patient was still under medication. Therapeutic management of IIH in a pediatric population is essentially medical, in some cases limited to lumbar puncture. The first-line treatment is acetazolamide, but this study shows that low doses and short duration are usually chosen. Doses must be increased and treatment prolonged to avoid the use of corticosteroids as a second-line treatment and prevent possible relapses that require close monitoring of visual function. The visual prognosis is generally better for this age group compared to adults and no risk factors for visual sequelae were identified. A standardized protocol for management of IIH was proposed.

Long-term visual outcome in idiopathic intracranial hypertension

Objective:To characterize the course, outcome, and risk of relapse or late worsening in a clearly defined cohort of idiopathic intracranial hypertension (IIH) over a long period of follow-up.Materials and Methods:Retrospective chart review of patients with definite IIH was evaluated at the Sree Chitra Tirunal Institute for Medical Sciences and Technology between 1998 and 2006. Patients’ demographic data, clinical, neuro-ophthalmic examinations, and treatment details were abstracted. Patients were further categorized into three groups based on whether they improved, worsened, or relapsed on follow-up. Final visual outcome of each patient was defined according to grading of the worse eye at the last visit. Statistical analysis included t test to compare group means and chi-square test to compare proportions.Results:Of the 43 women included, visual impairment was observed in 80 eyes (93%) at presentation and it was moderate to severe in 14%. The mean CSF opening pressure at presentation did not differ significantly in those with visual impairment compared to those with normal vision. Those having early severe visual impairment had significantly higher (P = 0.015) likelihood of severe visual impairment on last follow-up. Of the total, 34 patients (79%) improved, 4 (9.3%) relapsed on follow-up after period of stability, and 5 (11.6%) worsened over 56 months follow-up (range, 26-132 months). The groups were comparable, except those who improved were younger (P<0.05). At last examination, 9% had significant vision loss.Conclusion:IIH patients can have delayed worsening or relapses and about tenth of patients can have permanent visual loss early or late in the course of the disease. All patients with IIH need to be kept under long-term follow-up, with regular monitoring of visual functions.

Idiopathic intracranial hypertension: A comparison between French and North-American white patients

Objective To compare French and American white patients with idiopathic intracranial hypertension (IIH), and to determine prognostic factors associated with visual loss. Methods Medical records of all consecutive white patients with definite IIH seen between 2001 and 2006 in three French tertiary care medical centers and one American tertiary medical center were reviewed. Demographics, associated clinical features, and visual function at presentation and follow-up were collected. French white patients were compared to American white patients. Results One hundred and thirty-four patients (66 French, 68 American) were included. American patients were 8.7 times more likely than French patients to have visual acuity 20/60 or worse or visual field constriction (95% CI: 2.1–36.1, p = 0.0001). American patients were treated more aggressively than French patients. French patients were older (31 vs. 28 years, p = 0.02) and more likely to have anemia (20 vs. 2%, p < 0.001). American patients had a longer duration of symptoms prior to diagnosis (12 vs. 4 weeks, p = 0.01) and longer follow-up than French patients (26 vs. 11 months, p = 0.001). Multivariable analysis found that nationality was an independent risk factor for visual loss. French and American patients did not differ regarding gender proportion, frequency of obesity, sleep apnea, endocrine diseases, or systemic hypertension. Cerebrospinal fluid (CSF) opening pressures were similar in both groups. Conclusion American patients with IIH had worse visual outcomes than French patients despite more aggressive treatment. These differences are not explained by differences in previously known risk factors.

Idiopathic intracranial hypertension in men

To compare the characteristics of idiopathic intracranial hypertension (IIH) in men vs women in a multicenter study. Medical records of all consecutive patients with definite IIH seen at three university hospitals were reviewed. Demographics, associated factors, and visual function at presentation and follow-up were collected. Patients were divided into two groups based on sex for statistical comparisons. We included 721 consecutive patients, including 66 men (9%) and 655 women (91%). Men were more likely to have sleep apnea (24% vs 4%, p < 0.001) and were older (37 vs 28 years, p = 0.02). As their first symptom of IIH, men were less likely to report headache (55% vs 75%, p < 0.001) but more likely to report visual disturbances (35% vs 20%, p = 0.005). Men continued to have less headache (79% vs 89%, p = 0.01) at initial neuro-ophthalmologic assessment. Visual acuity and visual fields at presentation and last follow-up were significantly worse among men. The relative risk of severe visual loss for men compared with women was 2.1 (95% CI 1.4-3.3, p = 0.002) for at least one eye and 2.1 (95% CI 1.1-3.7, p = 0.03) for both eyes. Logistic regression supported sex as an independent risk factor for severe visual loss. Men with idiopathic intracranial hypertension (IIH) are twice as likely as women to develop severe visual loss. Men and women have different symptom profiles, which could represent differences in symptom expression or symptom thresholds between the sexes. Men with IIH likely need to be followed more closely regarding visual function because they may not reliably experience or report other symptoms of increased intracranial pressure.