Neurofilament light chain is elevated in patients with newly diagnosed idiopathic intracranial hypertension: A prospective study.

BACKGROUND: Idiopathic intracranial hypertension is a secondary headache disorder potentially causing visual loss. Neurofilament light chain is a candidate, prognostic biomarker, but further studies of neuronal biomarkers are needed. Our objective was to investigate neurofilament light chain in cerebrospinal fluid (cNfL) and plasma (pNfL), amyloid-beta 42 (Aß-42), total-tau and phosphorylated-tau in cerebrospinal fluid in new-onset idiopathic intracranial hypertension. METHODS: Prospective case-control study including new-onset idiopathic intracranial hypertension and age, sex and BMI matched controls. Biomarkers were compared between patients and controls and related to papilledema, visual fields and opening pressure. RESULTS: We included 37 patients and 35 controls. Patients had higher age-adjusted cNfL (1.4 vs. 0.6 pg/mL, p-adjusted < 0.001), pNfL (0.5 vs. 0.3 pg/mL, p-adjusted < 0.001) and total-tau/Aß-42 (0.12 vs. 0.11, p-adjusted = 0.039). Significant, positive linear correlations were found between cNfL, pNfL, total-tau/Aß-42 and opening pressure. Patients with severe papilledema had elevated cNfL compared to mild-moderate papilledema (median cNfL: 4.3 pg/mL (3.7) versus 1.0 pg/mL (1.4), p-adjusted = 0.009). cNFL was inversely associated with perimetric mean deviation (r = -0.47, p-adjusted < 0.001). CONCLUSIONS: cNfL, pNfL and total-tau/Aß-42 were elevated in new-onset idiopathic intracranial hypertension. cNfL was associated with severity of papilledema and visual field defects at diagnosis. This indicates early axonal damage. Neurofilament light chain is a candidate biomarker for disease severity.

Idiopathic intracranial hypertension secondary to fluoroquinolone therapy: French pharmacovigilance data review.

We investigate spontaneous reports of IIH related to fluoroquinolones recorded in the French national pharmacovigilance database in order to detect a possible pharmacovigilance signal. The association between IIH risk and fluoroquinolone exposure was assessed using a case/non-case study. Between 1985 and July 2023, 17 reports of IIH after fluoroquinolone exposure were recorded. No specific fluoroquinolone was predominant. IIH led to death in one case and blindness in one case. The Reporting Odds Ratio was 2.58 (95% confidence interval 1.59-4.19). We highlight statistically significant disproportionality, which constitutes a pharmacovigilance signal. IIH risk after fluoroquinolone exposure is a class effect.

Idiopathic Intracranial Hypertension After Abrupt Cessation of Medication: A Case Report of Abrupt Glucagon-Like Peptide-1 (GLP-1) Receptor Agonist Cessation and Review of the Literature.

PURPOSE OF REVIEW: The purpose of this review is two-fold: (1) to discuss a case report of idiopathic intracranial hypertension (IIH) after abrupt cessation of a glucagon-like peptide-1 (GLP-1) receptor agonist with resultant rapid weight gain and (2) to review the literature regarding the potential role of GLP-1 receptor agonists in the treatment of IIH as well as potential pitfalls. RECENT FINDINGS: GLP-1 receptor agonists have become widely used to treat obesity. Obesity is a known risk factor for the development of IIH, though the precise pathophysiology is unclear. GLP-1 receptor agonists may help treat IIH by promoting weight loss, lipolysis of adipose tissue, and potentially decreasing the secretion of CSF, as was seen in rat models. Abrupt cessation of GLP-1 receptor agonists can result in regaining lost weight rapidly. In the case that we present, the patient stopped duraglutide abruptly due to lack of insurance coverage and regained the weight she had lost within a month. She subsequently developed IIH. GLP-1 receptor agonists have the potential to help treat IIH; however, this class of medication needs to be used carefully, as cessation of the medication and resultant rapid weight gain can result in IIH.

The diagnostic value of MRI findings in pediatric idiopathic intracranial hypertension: a case-control study.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a rare medical condition in children. Based on the different radiological findings reported in various studies in pediatric IIH, this study was conducted to determine the diagnostic value of MRI findings in diagnosing IIH in children. METHODS: In this retrospective study, the medical records of all children aged 1 to 18 years who visited Ghaem Hospital in Mashhad, Iran, between 2012 and 2022 and were diagnosed with IIH were gathered. Forty-nine cases of children with IIH and 48 control cases of children with the first unprovoked seizure with no indications of increased intracranial pressure for comparison were selected. Patient demographic information and MRI findings were extracted. The comparison between different MRI findings in the case and control groups was conducted using statistical tests. RESULTS: In the case group, the mean diameter of the subarachnoid space expansion around the optic nerve was 5.96 ± 1.21, compared to 4.79 ± 0.33 in the control group, with statistically significant difference (P < 0.001). All the patients with flattening of the posterior globe or transverse sinus stenosis were in the case group, and the frequency of these findings in the case group was significantly higher than in the control group (P < 0.001). The majority of patients (95.5%) classified under category 3 and 4 of empty sella were part of the case group, and the statistical test results indicated a significant difference between the two groups (P < 0.001). The optic nerve sheath diameter cut-off of 5.35 mm, when used for expansion of the subarachnoid space around the optic nerve, with a sensitivity of 82% and a specificity of 100% in diagnosing IIH. CONCLUSION: The most reliable diagnostic indicators for diagnosing IIH in children are perioptic subarachnoid space expansion with high sensitivity, and posterior globe flattening and transverse sinus stenosis with high specificity.

Intraoperative cone-beam computed tomography for catheter placement verification in pediatric hydrocephalus: technical note.

Ventriculoperitoneal (VP) shunt placement, essential for managing hydrocephalus, often risks catheter malpositioning, especially in patients with small ventricles. We present a novel technique combining neuronavigation with intraoperative cone-beam computed tomography using the BrainLab system and Loop-X mobile imaging unit. This approach enables real-time verification of catheter placement by integrating preoperative MRI data with intraoperative CT imaging. In a 12-year-old boy with therapy-refractory idiopathic intracranial hypertension, neuronavigation was guided by the BrainLab Skull Fix and Cushing canula, ensuring precise catheter insertion into the right frontal horn. Post-placement, Loop-X facilitated immediate verification of the catheter's trajectory and positioning, corroborated by postoperative MRI. This technique demonstrated high precision and minimized radiation exposure, emphasizing its utility in reducing revision rates due to suboptimal catheter placement.

Role of neuroimaging markers on predicting of idiopathic intracranial hypertension.

BACKGROUND: The goals of neuroimaging in idiopathic intracranial hypertension (IIH) are the exclusion of mimickers and effective management of disease. In recent studies, several imaging markers have been identified as potential predictors of IIH. PURPOSE: To investigate the predictive roles of novel radiological markers as the Meckel's cave area, alongside classical radiologic markers in identifying IIH such as the empty sella. MATERIAL AND METHODS: The patients were classified according to cerebrospinal fluid (CSF) opening pressure as the IIH group and control group. The observational, case-control study included 22 patients with IIH and 22 controls. Groups were compared for presence of empty sella, Meckel's cave area, fat area of posterior neck, fat thickness of scalp, presence of transverse sinus stenosis, and ophthalmic markers, such as increase of optic nerve (ON) sheath diameter. RESULTS: In the IHH group, higher occurrences of increased ON sheath diameter, ON tortuosity, flattening of the scleral surface, and transverse sinus stenosis were observed (P < 0.001, P < 0.001, P = 0.046, and P = 0.021, respectively). Meckel's cave area and fat area of posterior neck were similar in both groups (P = 0.444 and P = 0.794). CONCLUSION: Ophthalmic markers and transverse sinus stenosis could be utilized as radiologic features supporting early and precise diagnosis of IIH. However, enlargement of Meckel's cave area and measurements of fatty area of posterior neck are not helpful for diagnosis of IIH.

Ultrasound-guided initial diagnosis and follow-up of pediatric idiopathic intracranial hypertension.

BACKGROUND: Idiopathic intracranial hypertension in children often presents with non-specific symptoms found in conditions such as hydrocephalus. For definite diagnosis, invasive intracranial pressure measurement is usually required. Ultrasound (US) of the optic nerve sheath diameter provides a non-invasive method to assess intracranial pressure. Transtemporal US allows imaging of the third ventricle and thus assessment for hydrocephalus. OBJECTIVE: To investigate whether the combination of US optic nerve sheath and third ventricle diameter can be used as a screening tool in pediatric idiopathic intracranial hypertension to indicate elevated intracranial pressure and exclude hydrocephalus as an underlying pathology. Further, to analyze whether both parameters can be used to monitor treatment outcome. MATERIALS AND METHODS: We prospectively included 36 children with idiopathic intracranial hypertension and 32 controls. Using a 12-Mhz linear transducer and a 1-4-Mhz phased-array transducer, respectively, optic nerve sheath and third ventricle diameters were determined initially and during the course of treatment. RESULTS: In patients, the mean optic nerve sheath diameter was significantly larger (6.45±0.65 mm, controls: 4.96±0.32 mm) and the mean third ventricle diameter (1.69±0.65 mm, controls: 2.99±1.31 mm) was significantly smaller compared to the control group, P<0.001. Optimal cut-off values were 5.55 mm for the optic nerve sheath and 1.83 mm for the third ventricle diameter. CONCLUSIONS: The combined use of US optic nerve sheath and third ventricle diameter is an ideal non-invasive screening tool in pediatric idiopathic intracranial hypertension to indicate elevated intracranial pressure while ruling out hydrocephalus. Treatment can effectively be monitored by repeated US, which also reliably indicates relapse.

Spontaneous Cerebrospinal Fluid Leak in a Patient with Asymptomatic Idiopathic Intracranial Hypertension.

BACKGROUND: Spontaneous cerebrospinal fluid (CSF) leaks occur when there is a tear in the dura mater. Spontaneous CSF leaks are rare, and often associated with conditions like intracranial hypertension, connective tissue disorders, or congenital defects in the dura mater. CASE REPORT: The patient was a 66-year-old woman who presented to the Emergency Department with clear, positional nasal discharge from her left nostril for 1 week. She had a history of chronic headaches, which seemed to have been relieved around the time of onset of her rhinorrhea. Diagnostic imaging, including computed tomography and magnetic resonance imaging scans, confirmed the presence of a CSF leak and a left temporal meningoencephalocele. The patient subsequently underwent surgical repair of the leak and ventriculoperitoneal shunt placement, and was discharged home in stable condition. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Early detection of CSF leaks require a thorough history and physical examination, and is crucial in preventing potentially life-threatening complications such as meningitis and intracranial abscesses.

National trends in the endovascular and surgical treatment of idiopathic intracranial hypertension.

BACKGROUND: The pattern of surgical treatments for Idiopathic Intracranial Hypertension (IIH) in the United States is not well-studied, specifically the trend of utilizing endovascular venous stenting (EVS) as an emerging technique. METHODS: In this cross-sectional study, we aimed to explore the national trend of utilizing different procedures for the treatment of IIH including EVS, Optic Nerve Sheath Fenestration (ONSF), and CSF Shunting, with a focus on novel endovascular procedures. Moreover, we explored rates of 90-day readmission and length of hospital stay following different procedures, besides the effects of sociodemographic and clinical parameters on procedure choice. RESULTS: 36,437 IIH patients were identified from records between 2010 and 2018. Those in the EVS group were 29 years old on average, and 93.4 % were female. Large academic hospital setting was independently associated with higher EVS utilization, while other factors were not predictive of procedure choice. The proportion of EVS use in IIH hospitalizations increased significantly from 2010 to 2018 (P < 0.001), while there was a relative decline in the frequency of shunting procedures (P = 0.001), with ONSF utilization remaining stable (P = 0.39). The rate of 90-day readmission and length of hospital stay were considerably lower following EVS compared to other procedures (Ps < 0.001). CONCLUSION: This study presents novel population-level data on national trends in the frequency and outcome of EVS for IIH therapy. EVS was associated with shorter length of hospital stays and fewer readmission rates. In addition, a continuous increase in venous stenting compared to other procedures suggests an increasing role for endovascular therapies in IIH.

Clinical profile and treatment outcomes of idiopathic intracranial hypertension: a multicenter study from Korea.

BACKGROUND: Currently, there is a relative lack of detailed reports regarding clinical presentation and outcome of idiopathic intracranial hypertension in Asians. This study aims to describe the clinical features and treatment outcomes of Korean patients with idiopathic intracranial hypertension. METHODS: We prospectively recruited patients with idiopathic intracranial hypertension from one hospital and retrospectively analyzed the medical records of 11 hospitals in Korea. We collected data regarding preceding medical conditions or suspected medication exposure, headache phenotypes, other associated symptoms, detailed neuroimaging findings, treatments, and outcomes after 1-2 and 3-6 months of treatment. RESULTS: Fifty-nine (83.1% women) patients were included. The mean body mass index was 29.11 (standard deviation, 5.87) kg/m2; only 27 patients (45.8%) had a body mass index of ≥ 30 kg/m2. Fifty-one (86.4%) patients experienced headaches, patterns of which included chronic migraine (15/51 [29.4%]), episodic migraine (8/51 [15.7%]), probable migraine (4/51 [7.8%]), chronic tension-type headache (3/51 [5.9%]), episodic tension-type headache (2/51 [3.9%]), probable tension-type headache (2/51 [3.9%]), and unclassified (17/51 [33.3%]). Medication overuse headache was diagnosed in 4/51 (7.8%) patients. After 3-6 months of treatment, the intracranial pressure normalized in 8/32 (25.0%), improved in 17/32 (53.1%), no changed in 7/32 (21.9%), and worsened in none. Over the same period, headaches remitted or significantly improved by more than 50% in 24/39 patients (61.5%), improved less than 50% in 9/39 (23.1%), and persisted or worsened in 6/39 (15.4%) patients. CONCLUSION: Our findings suggest that the features of Asian patients with idiopathic intracranial hypertension may be atypical (i.e., less likely obese, less female predominance). A wide spectrum of headache phenotypes was observed. Medical treatment resulted in overall favorable short-term outcomes; however, the headaches did not improve in a small proportion of patients.

Characterisation and Visual Outcomes of Fulminant Idiopathic Intracranial Hypertension: A Narrative Review.

Fulminant idiopathic intracranial hypertension (IIH) is a rapid vision-degrading presentation of IIH with limited published studies. This study composed a narrative review of fulminant IIH with the aim of better characterising fulminant IIH presentation and visual outcomes. SCOPUS and PubMed were searched for papers referencing IIH, benign intracranial hypertension, or pseudotumour cerebri. Abstracts were screened for rapid degradation in vision. All studies were required to meet both the modified Dandy and fulminant IIH criteria. Thirty-six studies met the inclusion criteria. Demographics, treatments, and visual outcome data were collected. Case studies made up 69% of the studies and 31% were case series. In total, 72 patients with fulminant IIH were reported, of which 23.6% were paediatric and 96% were female. Surgical intervention occurred in 85% of patients. Anaemia was present in 11% of patients and 85.7% of paediatric patients had a sixth cranial nerve palsy. In conclusion, we propose the following practice guidelines to assist in diagnosing and treating fulminant IIH patients: 1) patients who present with optic disc oedema require urgent visual field testing to evaluate for vision loss; 2) a paediatric patient presenting with a sixth cranial nerve palsy should have a comprehensive eye examination; 3) fulminant IIH can occur in patients with a normal body mass index; and 4) anaemia should be tested for in the setting of fulminant IIH. As little is known about the optimal treatment mechanisms for this presentation, multi-institutional and international collaborations will be a critical step for future research.

Effect of General Anesthesia on MR Optic Nerve Sheath Diameter in the Pediatric Population.

BACKGROUND: Papilledema is thought to be the hallmark sign of increased intracranial pressure (ICP). Distension of the subarachnoid space within the optic nerve sheath is also commonly reported in MR studies as an indirect sign of increased ICP. HYPOTHESIS: General anesthesia and positive pressure ventilation might result in changes in optic sheath diameter (OSD) observed on clinical brain MRI. STUDY TYPE: Retrospective. POPULATION: One hundred forty-five  patients (154 MRI scans, 7.3 years ± 5.1); 97 studies in the anesthesia group (4.4 years ± 3.4) of which 22 had papilledema, and 57 in the non-anesthesia group (12.3 years ± 3.2), of which 28 had papilledema. FIELD STRENGTH/SEQUENCE: 1.5T or 3.0T volumetric T2 images. T2 images were obtained from different vendors. ASSESSMENT: OSD, optic nerve diameter (OND), and peri-optic cerebrospinal fluid (CSF) were measured manually on T2-weighted MR images for various population subgroups (with and without anesthesia; with or without papilledema). The correlation between these measurements and the clinical diagnosis of papilledema was evaluated via receiver operating characteristic (ROC) analysis. STATISTICAL TESTS: Chi-square test; Mann-Whitney Test; Spearman's test and ROCs; Interclass correlation coefficient, P = 0.05. RESULTS: General anesthesia resulted in significantly larger mean OSD in patients with or without papilledema (7.3 ± 1.0 mm vs. 6.1 ± 1.1 mm and 6.7 ± 1.0 mm vs. 5.4 ± 0.9 mm, respectively). In the non-anesthesia group, the average OSD values (6.1 ± 1.1 mm) were significantly higher in papilledema patients compared to non-papilledema patients (5.4 ± 0.9 mm), with larger peri-optic CSF rim (1.6 ± 0.4 mm vs. 1.3 ± 0.3 mm). In the anesthesia group, OND was significantly larger in papilledema patients (3.4 ± 0.4 mm vs. 3.1 ± 0.5 mm), though the average peri-optic CSF rim did not reach a significance in papilledema compared with non-papilledema patients (2.0 ± 0.3 mm vs. 1.8 ± 0.4 mm, P = 0.06). In patients with general anesthesia, peri-optic CSF rim had a limited correlation with increased ICP. DATA CONCLUSION: In the pediatric population, imaging findings of increased OSD on brain MRI might be related to general anesthesia rather than increased ICP. The interpretation of optic nerve sheath distention should be reported cautiously in conjunction with anesthesia status, especially in the pediatric population. EVIDENCE LEVEL: 4 Technical Efficacy: 5.

Diagnosis of idiopathic intracranial hypertension: A proposal for evidence-based diagnostic criteria.

BACKGROUND: Based on expert opinion, abducens nerve palsy and a neuroimaging criterion (≥3 neuroimaging signs suggestive of elevated intracranial pressure) were added to the diagnostic criteria for idiopathic intracranial hypertension. Our objective was to validate this. METHODS: This prospective study included patients with new-onset idiopathic intracranial hypertension for a standardized work-up: interview, neuro-ophthalmological exam, lumbar puncture, neuroimaging. Neuroimaging was evaluated by a blinded neuroradiologist. RESULTS: We included 157 patients classified as idiopathic intracranial hypertension (56.7%), probable idiopathic intracranial hypertension (1.9%), idiopathic intracranial hypertension without papilledema (idiopathic intracranial hypertension-without papill edema; 0%), suggested idiopathic intracranial hypertension-without papill edema (4.5%), or non-idiopathic intracranial hypertension (36.9%). Moderate suprasellar herniation was more common in idiopathic intracranial hypertension than non-idiopathic intracranial hypertension (71.4% versus 47.4%, p < 0.01), as was perioptic nerve sheath distension (69.8% versus 29.3%, p < 0.001), flattening of the globe (67.1% versus 11.1%, p < 0.001) and transverse sinus stenosis (60.2% versus 18.9%, p < 0.001). Abducens nerve palsy was of no diagnostic significance. Sensitivity of ≥3 neuroimaging signs was 59.5% and specificity was 93.5%. CONCLUSION: Moderate suprasellar herniation, distension of the perioptic nerve sheath, flattening of the globe and transverse sinus stenosis were associated with idiopathic intracranial hypertension. We propose that idiopathic intracranial hypertension can be defined by two out of three objective findings (papilledema, opening pressure ≥25 cm cerebrospinal fluid and ≥3 neuroimaging signs).

Guidelines of the International Headache Society for Controlled Clinical Trials in Idiopathic Intracranial Hypertension.

The quality of clinical trials is essential to advance treatment, inform regulatory decisions and meta-analysis. With the increased incidence of idiopathic intracranial hypertension and the emergence of clinical trials for novel therapies in this condition, the International Headache Society Guidelines for Controlled Clinical Trials in Idiopathic Intracranial Hypertension aims to establish guidelines for designing state-of-the-art controlled clinical trials for idiopathic intracranial hypertension.

Predictors of disease course and long-term outcomes of idiopathic intracranial hypertension in children and adolescents.

This study aimed to identify predictors for unfavorable disease course and clinical and visual outcomes in pediatric patients with idiopathic intracranial hypertension (IIH). Employing a multi-tiered approach, we retrospectively analyzed clinical, ophthalmic, and neuroimaging data from patients diagnosed with IIH between 2003 and 2021. Of the 97 patients included, 56 (58%) were females. The median age was 12 years [Interquartile range (IQR) 9, 14], and the median follow-up time was 39.0 months (IQR 14.8, 90.9). Forty-two (43%) patients had an unfavorable disease course, 28 (29%) had persistence of headache at last follow-up, and 16 (18%) had a poor visual outcome, most of them with mild visual disturbances. Poor visual outcome was more common in females compared to males [16/47 (34%) vs. 0/39, p < 0.001)]. On multivariate regression analysis, female sex and disease recurrence were significantly associated with poor visual outcomes (OR: 18.5, CI:1.3-270, P = 0.03, and OR: 5.1, CI: 1.2-22.5, P = 0.03, respectively). Patients with persistent headaches exhibited lower incidence of papilledema, lower opening pressure, and fewer neuroimaging markers indicating elevated intracranial pressure. CONCLUSIONS: This study provides insights into predictive factors for an unfavorable disease course, persistent headaches, and poor visual outcomes in patients with childhood IIH. Patients with persistent headaches may have a variant of a chronic pain syndrome warranting a different therapeutic approach. WHAT IS KNOWN: • Childhood-onset Idiopathic Intracranial hypertension (IIH) is a heterogenous disease. The knowledge on disease trajectory and long-term outcomes and its predictors is limited. WHAT IS NEW: • A higher opening pressure and factors suggestive of the metabolic syndrome predict an unfavorable disease course whereas female sex and disease recurrence are significantly associated with poor visual outcomes • A third of the patients diagnosed with IIH experience ongoing headaches despite achieving favorable visual outcomes. This subset, characterized by lower disease-severity indicators at onset may represent a distinct subgroup warranting a different therapeutic approach.

Clinical and prognostic importance of craniospinal elastance and pressure volume index in pediatric pseudotumor cerebri syndrome.

OBJECTIVE: The aim of the study was to determine the clinical and prognostic significance of craniospinal elastance (Ecs), pressure volume index (PVI), and cerebrospinal fluid (CSF) total protein data in terms of resolution of papilledema and improvement of headache in children with pseudotumor cerebri syndrome (PTCS). METHODS: This is a retrospective observational study of 33 children with definitive PTCS. The relationships between lumbar puncture (LP) measurements, especially Ecs and PVI, and papilledema and headache resolution time were investigated. RESULTS: In children with definite primary and secondary PTCS, higher opening pressure and decreased PVI were found to be associated with faster resolution of papilledema and faster improvement of headache (r = 0.904, P < 0.01; r = 0.894, P < 0.01 respectively). This effect was higher in children with secondary PTCS (P = 0.022). While papilledema resolution time and treatment time were statistically significantly higher in the secondary group (P = 0.035, P = 0.040), there was no significant difference between the two groups in terms of headache relief time (P = 0.051). Based on the primary and secondary groups, from the cut-off points determined, it was found that ≤ 41.60 ((AUC = 0.706, P = 0.0420), specificity 56%, sensitivity 86.7%) for opening pressure and ≤ 69.37 ((AUC = 0.702, P = 0.0448), specificity 48.2%, sensitivity 83.3%) for PVI were statistically significant. A very strong negative linear correlation was found between CSF total protein and lumbar puncture measurements and clinical outcomes (P < 0.001). CONCLUSION: Ecs and PVI may be markers that can provide potentially important data on pediatric PTCS prognosis. CSF total protein may also provide clinical benefit.

Asymptomatic idiopathic intracranial hypertension: Prevalence and prognosis.

BACKGROUND: Little is known about the presentation and prognosis of asymptomatic idiopathic intracranial hypertension (IIH). Papilloedema can be found incidentally on routine fundus examination, with many of these patients actually having symptoms on direct questioning. The aim was to evaluate visual and headache outcomes in people with IIH who present with or without symptoms. METHODS: Prospective observational cohort study, between 2012 and 2021, 343 people with confirmed IIH diagnosis were enrolled in the IIH:Life database. Outcomes such as vision (LogMAR); Humphrey visual field perimetric mean deviation (PMD) and optical coherence tomography (OCT) and headache were evaluated using LOESS (locally weighted scatterplot smoothing) graphs and regression analysis. RESULTS: One hundred and twenty-one people had incidentally found papilloedema, with 36 people with completely asymptomatic presentations. Those with asymptomatic IIH at diagnosis had similar visual prognosis compared to those with symptomatic disease. Sixty-six percent of the asymptomatic cohort became symptomatic during follow-up, and of these the predominant symptom was headache (96%). Headache frequency during follow-up was lower in the asymptomatic cohort. CONCLUSIONS: The prognosis of those with IIH who present with or without symptoms is similar.

Cranial decompression and expansion surgery for the treatment of refractory idiopathic intracranial hypertension: case report and systematic review.

OBJECTIVE: The purpose of this study is to systematically review the literature on the clinical outcomes following different surgical techniques in patients with refractory idiopathic intracranial hypertension (IIH). BACKGROUND: IIH is a condition characterised by increased cranial pressure (ICP) in the absence of an intracranial lesion that does not adequately respond to different medical and surgical therapies. Cranial decompression or expansion surgeries are a last resort therapy for patients with refractory IIH. METHODS: A systematic literature search of the databases of PubMed, Embase and Medline from inception to 2019 was performed. Searches were limited to the English language and to clinical studies. Studies were included if clinical outcomes following different cranial decompression or expansion techniques were reported. We also add one case of our own experience with performing a bilateral frontoparietal expansion craniotomy and subtemporal craniectomy. RESULTS: Five manuscripts, describing 38 procedures, met the inclusion criteria. Thirty-one patients were female (82%). The mean age was 26.2 years. The techniques studied included subtemporal craniectomy (27/38, 71%), internal cranial expansion (10/38, 26%), and cranial morcellation decompression (1/38, 3%). Thirty-five patients presented with headaches of which 17 noted postoperative improvement or resolution (49%). Visual deficits were documented in 30 patients and 25 reported postoperative improvement (83%). Papilledema disappeared in 23 of 32 patients with this sign at presentation (72%). In our patient, symptoms completely resolved postoperatively and a 6% increase in intracranial volume was measured. CONCLUSIONS: Cranial vault decompression or expansion surgeries may be an effective last resort therapy for patients with refractory IIH. These surgeries expand the intracranial volume, and thus may normalise ICP, leading to clinical improvement.

Visual recovery after unilateral optic nerve sheath fenestration for pseudotumor cerebri syndrome.

PURPOSE: To evaluate the effect of optic nerve sheath fenestration (ONSF) on the rate of visual function improvement in patients with pseudotumor cerebri syndrome (PTCS). METHODS: Retrospective chart review of patients with PTCS who underwent ONSF between 1998 and 2017. Visual function was evaluated by evaluating visual field (VF), mean deviation (MD), retinal nerve fiber layer (RNFL) thickness, papilledema grade, and visual acuity (VA) prior to and after ONSF. RESULTS: Seventeen female patients aged 17 to 36 years underwent unilateral ONSF. Follow-up averaged 40.1 months. VF MD improved steadily in both eyes up to 12 months. Average RNFL thickness improved in the operated eye from 347 ± 166 mm to 92 ± 27 mm (p < .001) and the non-operated eyes from 306 ± 165 mm to 109 ± 46 mm (p < .001). The grade of papilledema improved in the operated eye from 3.3 ± 1.3 to 0.3 ± 0.7 and the non-operated eye from 3.0 ± 1.6 to 0.18 ± 0.4. There was an exponential rate of improvement in papilledema and RNFL thickness, with the greatest improvement occurring within the first 30 days. Average visual acuity remained intact in both eyes before and after surgery. CONCLUSIONS: ONSF in appropriately selected patients leads to rapid improvement in papilledema and a steady recovery in VF.

A Case Report of Pseudotumor Cerebri Syndrome with a Huge Retroperitoneal Cyst.

Background: 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. Case Presentation: 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/m2. 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 cmH2O) 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. Conclusions: 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.