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British Journal of Neurosurgery Oct 2023Bariatric surgery is an effective treatment for patients with idiopathic intracranial hypertension (IIH), a condition that is associated with skull base defects. A...
Bariatric surgery is an effective treatment for patients with idiopathic intracranial hypertension (IIH), a condition that is associated with skull base defects. A 55-year-old woman presented with symptoms of intractable nausea and vomiting, followed by headache and confusion two weeks after an elective laparoscopic vertical sleeve gastrectomy procedure. She had a presumed diagnosis of IIH and a remote history of CSF oto/rhinorrhea treated with a lumbar peritoneal (LP) shunt. Computed tomography (CT) scan of the head revealed tension pneumocephalus with midline shift and dehiscence of the tegmen. The patient underwent emergent craniotomy for decompression of the air-filled temporal lobe, clamping of the LP shunt, and repair of the skull base defect. Caution should be exercised in obese patients with a history of CSF leak secondary to a middle fossa skull base defect when being evaluated for bariatric surgery.
Topics: Female; Humans; Middle Aged; Pneumocephalus; Cerebrospinal Fluid Rhinorrhea; Tomography, X-Ray Computed; Treatment Outcome; Bariatric Surgery; Pseudotumor Cerebri
PubMed: 33393846
DOI: 10.1080/02688697.2020.1866166 -
The Clinical Neuropsychologist Apr 2024Cognitive disturbance is not pathognomonic of idiopathic intracranial hypertension (IIH), and therefore is not routinely assessed unless it presents as a major... (Review)
Review
Cognitive disturbance is not pathognomonic of idiopathic intracranial hypertension (IIH), and therefore is not routinely assessed unless it presents as a major complaint. Consequently, cognition has been slow to gain substantial traction in IIH-related research, despite its notable impact on a subset of patients. We completed a systematic review of the literature examining the neurocognitive profile of patients with IIH. A PRISMA compliant literature search was conducted in Ovid Medline, PubMed, PsycInfo, Embase, Web of Science, Cochrane, Cinahl, and Scopus databases. The initial query yielded 1376 unique articles. These articles were narrowed to those including empirical analyses of cognitive assessment in adult patients with IIH. A final cohort of nine articles resulted, comprising the findings from 309 patients with IIH and 153 healthy control subjects. Although there was considerable variability in methodology particularly with respect to cognitive assessment, fairly consistent deficits were observed across studies in the domains of processing speed, working memory, sustained and complex attention, set-shifting, and confrontation naming. Body mass index and body weight were not associated with cognitive performance. Pertinent limitations of the literature were identified, most notably failure to report trial-level cognitive testing data, the need for more comprehensive testing batteries with less reliance on screening tools, and not controlling for variables that may impact cognition. A more complete understanding of the cognitive profile in patients with idiopathic intracranial hypertension could lead to the increased -relevance of cognitive screening in disease management, and therefore more appropriate neuropsychological referral, earlier identification of functional limitations, and targeted neurorehabilitation.
Topics: Adult; Humans; Pseudotumor Cerebri; Neuropsychological Tests; Cognition; Cognitive Dysfunction; Memory, Short-Term
PubMed: 37614079
DOI: 10.1080/13854046.2023.2249176 -
Journal of Neuro-ophthalmology : the... Mar 2024There are an increasing number of controlled clinical trials and prospective studies, ongoing and recently completed, regarding management options for idiopathic...
BACKGROUND
There are an increasing number of controlled clinical trials and prospective studies, ongoing and recently completed, regarding management options for idiopathic intracranial hypertension (IIH). We present a Common Design and Data Element (CDDE) analysis of controlled and prospective IIH studies with the aim of aligning essential design and recommending data elements in future trials and enhancing data synthesis potential in IIH trials.
METHODS
We used PubMed and ClinicalTrials.gov to screen for ongoing and published trials assessing treatment modalities in people with IIH. After our search, we used the Nested Knowledge AutoLit platform to extract pertinent information regarding each study. We examined outputs from each study and synthesized the data elements to determine the degree of homogeneity between studies.
RESULTS
The most CDDE for inclusion criteria was the modified Dandy criteria for diagnosis of IIH, used in 9/14 studies (64%). The most CDDE for outcomes was change in visual function, reported in 12/14 studies (86%). Evaluation of surgical procedures (venous sinus stenting, cerebrospinal fluid shunt placement, and others) was more common, seen in 9/14 studies (64%) as compared with interventions with medical therapy 6/14 (43%).
CONCLUSIONS
Although all studies have similar focus to improve patient care, there was a high degree of inconsistency among studies regarding inclusion criteria, exclusion criteria, and outcomes measures. Furthermore, studies used different time frames to assess outcome data elements. This heterogeneity will make it difficult to achieve a consistent standard, and thus, making secondary analyses and meta-analyses less effective in the future. Consensus on design of trials is an unmet research need for IIH.
Topics: Humans; Pseudotumor Cerebri; Prospective Studies; Neurosurgical Procedures; Stents; Intracranial Hypertension
PubMed: 37342870
DOI: 10.1097/WNO.0000000000001902 -
Interventional Neuroradiology : Journal... Aug 2023Venous sinus stenosis (VSS) stenting has emerged as an effective treatment for patients with Idiopathic Intracranial Hypertension (IIH). However, stenting carries risk...
BACKGROUND
Venous sinus stenosis (VSS) stenting has emerged as an effective treatment for patients with Idiopathic Intracranial Hypertension (IIH). However, stenting carries risk of in-stent stenosis/thrombosis and cumulative bleeding risk from long-term dual antiplatelet (DAPT) use. Thus, we investigated the potential safety and efficacy of primary balloon angioplasty as an alternative to stenting in IIH.
METHODS
A prospectively maintained single-center registry of IIH patients undergoing endovascular procedures was queried. Inclusion criteria included patients with confirmed IIH and angiographically demonstrable VSS who underwent interventions from 2012- 2021. Patients were dichotomized into primary balloon angioplasty (Group A) and primary stenting (Group S), comparing clinical outcomes using bivariate analyses.
RESULTS
62 patients were included with median age of 33 [IQR 26-37], 74% females. Group A (9/62) and Group S (53/62) had similar baseline characteristics. Papilledema improvement was higher in Group S at 6 weeks and 6 months (44 vs. 93, p = 0.002 and 44 vs. 92%, p = 0.004), with similar improvements across all symptoms. Group S had higher mean post-procedure venous pressure gradient change (8 vs. 3 mmHg, p = 0.02) and a lower CSF opening pressure at 6 months (23 vs. 36 cmH2O, p < 0.001). VPS rescue rate was higher in Group A (44 vs. 2%, p = 0.001). There was only one procedural complications; a subdural hematoma in Group A.
CONCLUSIONS
Primary VSS balloon angioplasty provides a marginal and short-lived improvement of IIH symptoms compared to stenting. These findings suggest a cautious and limited role for short-term rescue angioplasty in poor shunting and stenting candidates with refractory IIH.
Topics: Female; Humans; Male; Pseudotumor Cerebri; Constriction, Pathologic; Cranial Sinuses; Treatment Outcome; Angioplasty, Balloon; Stents; Intracranial Hypertension; Retrospective Studies
PubMed: 35323053
DOI: 10.1177/15910199221089446 -
American Journal of Ophthalmology Sep 2023To describe the racial distribution of patients diagnosed with nonarteritic anterior ischemic optic neuropathy (NAION) in 2 large urban academic centers in the United...
PURPOSE
To describe the racial distribution of patients diagnosed with nonarteritic anterior ischemic optic neuropathy (NAION) in 2 large urban academic centers in the United States.
DESIGN
Retrospective cross-sectional study.
METHODS
All patients with NAION evaluated between 2014 and 2022 in tertiary neuro-ophthalmology services in Atlanta, GA, and New York, NY, respectively, were included. In order to assess the racial accessibility to our services, similar numbers of patients with idiopathic intracranial hypertension seen in the same services were included. Self-reported race at the time of neuro-ophthalmologic examination was collected for all patients. Results were compared with data published by the US Bureau of Census for Decennial Census in 2020 for the states of Georgia and New York, and for the total population of the United States.
RESULTS
In both locations, the vast majority of patients with NAION reported being White (91.06% in Atlanta and 78.9% in New York). Only 7.27% of patients in Atlanta and 3% in New York reported being Black, and 1.24% of patients in Atlanta and 5.9% in New York reported being Asian. There were a much higher proportion of White people among NAION patients than in the group of patients with idiopathic intracranial hypertension and in each respective state and in the United States.
CONCLUSIONS
Our results show that the majority of patients with NAION are White, followed by Black and Asian races, respectively, thus confirming the NAION race distribution reported by past clinical trials.
Topics: Humans; Optic Neuropathy, Ischemic; Retrospective Studies; Cross-Sectional Studies; Pseudotumor Cerebri; New York
PubMed: 36963603
DOI: 10.1016/j.ajo.2023.03.013 -
Quality, Reliability, and Readability of Online Information on Idiopathic Intracranial Hypertension.Journal of Neuro-ophthalmology : the... Mar 2024To evaluate the quality & reliability, technical quality, and readability of patient-targeted online information on idiopathic intracranial hypertension.
BACKGROUND
To evaluate the quality & reliability, technical quality, and readability of patient-targeted online information on idiopathic intracranial hypertension.
METHODS
In this cross-sectional study, we searched Google and Bing search engines for the terms "idiopathic intracranial hypertension" and "pseudotumor cerebri." We evaluated the first 50 search outcomes for each search term. Peer-reviewed articles, targeted advertisements, book chapters, videos, personal blogs, websites intended for healthcare professionals, and non-English websites were excluded. Websites were classified as institutional or private. Two independent reviewers assessed each website for 1) quality and reliability using DISCERN, Health on the Net Code of Conduct (HONcode), and JAMA criteria and 2) technical quality based on 11 criteria. Readability was evaluated using 6 measures (Flesch-Kincaid Reading Ease score and grade level, Gunning Fog, Simple Measure of Gobbledygook (Smog) index, Coleman-Liau index, and automated readability index).
RESULTS
Sixty-three websites (37 institutional) were included. The mean scores for the DISCERN, HONcode, and JAMA criteria were 3.6 ± 1 (1-4.8; 1: worse, 5: best), 10.3 ± 2.8 (2-16; 0: worse, 16: best), and 2 ± 1.3 (0-4; 0: worse, 4: best), respectively. The mean technical quality score was 0.8 ± 0.1 (0.5-1). The mean Flesch-Kincaid grade level score was 8.9 ± 1.8 (3.3-13.3). For Flesch-Kincaid grade level, 47 (74.6%) websites scored a grade level of 10 or less. For Gunning Fog Score, 35 websites (55.6%) scored from 7.3 to 10.8. For the Smog Index, 46 (73%) websites scored 5.7-8. The mean Coleman Liau Index was 16 ± 2.1 (9.6-22.2). For the automated readability index, 30 (50.7%) websites scored less than the eighth grade. No statistically significant difference was present between institutional and private websites except for JAMA, which scored higher in private websites (2.4 vs 1.7, P = 0.03).
CONCLUSIONS
Our findings suggest that online information on idiopathic intracranial hypertension/pseudotumor cerebri for patients generally demonstrates moderate to good quality and reliability. Although most websites maintain credible technical quality, the readability exceeds recommended levels for the average American reader. This may impede understanding, emphasizing the need for future improvements.
PubMed: 38527126
DOI: 10.1097/WNO.0000000000002130 -
The Journal of Headache and Pain Jul 2023In idiopathic intracranial hypertension (IIH), certain MRI features are promising diagnostic markers, but whether these have prognostic value is currently unknown.
BACKGROUND
In idiopathic intracranial hypertension (IIH), certain MRI features are promising diagnostic markers, but whether these have prognostic value is currently unknown.
METHODS
We included patients from the Vienna-Idiopathic-Intracranial-Hypertension (VIIH) database with IIH according to Friedman criteria and cranial MRI performed at diagnosis. Presence of empty sella (ES), perioptic subarachnoid space distension (POSD) with or without optic nerve tortuosity (ONT), posterior globe flattening (PGF) and transverse sinus stenosis (TSS) was assessed and multivariable regression models regarding visual outcome (persistent visual impairment/visual worsening) and headache outcome (headache improvement/freedom of headache) were fitted.
RESULTS
We included 84 IIH patients (88.1% female, mean age 33.5 years, median body mass index 33.7). At baseline, visual impairment was present in 70.2% and headache in 84.5% (54.8% chronic). Persistent visual impairment occurred in 58.3%, visual worsening in 13.1%, headache improvement was achieved in 83.8%, freedom of headache in 26.2%. At least one MRI feature was found in 78.6% and 60.0% had ≥3 features with POSD most frequent (64.3%) followed by TSS (60.0%), ONT (46.4%), ES (44.0%) and PGF (23.8%). In multivariable models, there was no association of any single MRI feature or their number with visual impairment, visual worsening, headache improvement or freedom. Visual impairment at baseline predicted persistent visual impairment (odds ratio 6.3, p<0.001), but not visual worsening. Chronic headache at baseline was significantly associated with lower likelihood of headache freedom (odds ratio 0.48, p=0.013), but not with headache improvement.
CONCLUSIONS
MRI features of IIH are neither prognostic of visual nor headache outcome.
Topics: Humans; Female; Adult; Male; Pseudotumor Cerebri; Magnetic Resonance Imaging; Optic Nerve; Headache; Vision Disorders
PubMed: 37507663
DOI: 10.1186/s10194-023-01641-x -
Wiener Klinische Wochenschrift Jan 2024Idiopathic intracranial hypertension (IIH) is becoming increasingly more prevalent bearing the risk of visual impairment and affecting the quality of life. Clinical...
BACKGROUND
Idiopathic intracranial hypertension (IIH) is becoming increasingly more prevalent bearing the risk of visual impairment and affecting the quality of life. Clinical presentation and outcome are heterogeneous. Large, well-characterized cohorts are scarce.
OBJECTIVE
To characterize the clinical spectrum, diagnostic findings, therapeutic management, and outcome of IIH.
METHODS
We identified patients with IIH according to modified Friedman criteria treated at our center between 2014 and 2021. The Vienna IIH database is described in detail.
RESULTS
Of 113 patients 89% were female (mean age 32.3 years). Median body mass index (BMI) was 31.8, with 85% overweight (BMI > 25) and 5% were classified as IIH without papilledema. Headache was present in 84% and showed migraine features in 43%. Median opening pressure in lumbar puncture was 31 cmHO. Pharmacotherapy (predominantly acetazolamide) was established in 99%, 56% required at least 1 therapeutic lumbar puncture and 13% a surgical intervention. After a median 3.7 years follow-up, 57% had achieved significant weight loss, papilledema was present in 59% and headache in 76% (58% improved). Comparing initial presentation to follow-up, perimetry was abnormal in 67% vs. 50% (8% worsened, 24% improved) and transorbital sonography in 87% vs. 65% with a median optic nerve sheath diameter of 5.4 mm vs. 4.9 mm. Median peripapillary retinal nerve fiber layer thickness decreased from 199 µm to 99 µm and ganglion cell layer volume from 1.13 mm to 1.05 mm.
CONCLUSION
The large representative Vienna IIH cohort characterizes IIH-related symptoms, diagnostic findings, treatment, and outcome emphasizing substantial long-term sequelae of IIH. Future analyses will aim to refine phenotyping and identify factors predicting outcome.
Topics: Humans; Female; Adult; Male; Pseudotumor Cerebri; Papilledema; Austria; Quality of Life; Headache
PubMed: 37650963
DOI: 10.1007/s00508-023-02252-x -
Neurosurgery Apr 2024Although venous sinus stenting (VSS) improves cerebrospinal fluid reabsorption and decreases intracranial pressure in patients with idiopathic intracranial hypertension... (Meta-Analysis)
Meta-Analysis
BACKGROUND AND OBJECTIVES
Although venous sinus stenting (VSS) improves cerebrospinal fluid reabsorption and decreases intracranial pressure in patients with idiopathic intracranial hypertension (IIH), the underlying pathophysiology of IIH is not well understood. We present a review and meta-analysis of the literature on VSS for IIH treatment, focusing on the rates of restenosis and symptom recurrence.
METHODS
We performed a systematic review of PubMed and Embase databases between January 1, 2011, and December 31, 2021. Articles including ≥5 patients with IIH and venous sinus stenosis treated with VSS and post-treatment rates of restenosis (de novo stenosis at a different anatomic location along the dural sinuses or restenosis within or adjacent to the stent) were selected. Demographic, procedural, and outcomes data were collected and analyzed. Mean values for variables collected were pooled, and a mean value was calculated with a 95% CI.
RESULTS
Twenty-four articles were included, comprising 694 patients and 781 VSS cases. The mean age was 33.9 (CI, 31.5-36.2) years. The mean body mass index was 35.3 (CI, 32.9-37.7) kg/m 2 . Before VSS, 98.8% (CI, 96.8%-100.0%) of patients experienced headaches, 87.7% (CI, 80.6%-95.5%) had visual acuity issues, 78.7% (CI, 69.9%-88.5%) had papilledema, 58.3% (CI, 46.0%-73.9%) had tinnitus, and 98.8% (96.4%-100.0%) had symptoms refractory to previous therapies. After VSS, 77.7% (CI, 71.1%-84.95%) experienced symptom improvement and 22.3% (CI, 15.1%-29.0%) had persistent or worsened symptoms. Pooled restenosis rate was 17.7% (CI, 14.9%-20.9%).
CONCLUSION
VSS is effective in alleviating IIH signs and symptoms, but the associated high rates of restenosis and persistent symptoms highlight the need for further investigation of this procedure and other adjunctive treatments for IIH.
Topics: Humans; Adult; Pseudotumor Cerebri; Constriction, Pathologic; Treatment Outcome; Intracranial Pressure; Cranial Sinuses; Stents; Intracranial Hypertension; Retrospective Studies
PubMed: 37830801
DOI: 10.1227/neu.0000000000002718 -
Medicina (Kaunas, Lithuania) Aug 2023: Aside from primary pseudotumor cerebri syndrome (PTCS) with an unknown etiology (i.e., idiopathic intracranial hypertension), which typically occurs in association...
: Aside from primary pseudotumor cerebri syndrome (PTCS) with an unknown etiology (i.e., idiopathic intracranial hypertension), which typically occurs in association with obesity, several conditions including cerebral venous abnormalities, drug use, and hormonal imbalance may be a secondary cause of PTCS. However, a focal space-occupying lesion outside of the brain as a cause of PTCS has rarely been reported. : A previously healthy 34-year-old man presented with blurred vision for three weeks. The patient had a three-month preceding history of worsening headache. On admission, he was hypertensive (160/90 mmHg) and underweight with a body mass index of 18.4 kg/m. Fundus examination documented papilledema in both eyes. Neurological examination was unremarkable except for mild nuchal rigidity, and results of routine serologic testing were normal. Gadolinium-enhanced brain magnetic resonance imaging revealed bilateral posterior scleral flattening, suggesting intracranial hypertension. There was no other abnormal brain parenchymal lesion or meningeal enhancement. Cerebrospinal fluid (CSF) assay showed a markedly increased opening pressure (30.0 cmHO) with normal CSF composition. A tentative diagnosis of PTCS was made based on ophthalmological, neuroradiological, and laboratory findings. During differential diagnosis, abdomen computed tomography demonstrated a huge benign cystic lesion (14.7 × 10.6 × 16.4 cm) in the right retroperitoneal space, which originated from the mesentery and resulted in hydronephrosis and renovascular hypertension due to external compression of the right kidney. Other evaluations were unremarkable. After successful surgical removal of the cyst, clinical symptoms such as headache, blurred vision, and papilledema on fundus examination were markedly improved, and blood pressure was normalized during the three-month follow-up period. : A large retroperitoneal cyst that can increase intra-abdominal pressure could be a rare cause of PTCS. Therefore, meticulous evaluation is warranted for patients with PTCS, especially those without known risk factors.
Topics: Male; Humans; Adult; Pseudotumor Cerebri; Papilledema; Intracranial Hypertension; Cysts; Risk Factors
PubMed: 37763692
DOI: 10.3390/medicina59091573