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Journal of Neurology, Neurosurgery, and... Mar 2024Idiopathic intracranial hypertension (IIH) is a condition of significant morbidity and rising prevalence. It typically affects young people living with obesity, mostly... (Review)
Review
Idiopathic intracranial hypertension (IIH) is a condition of significant morbidity and rising prevalence. It typically affects young people living with obesity, mostly women of reproductive age, and can present with headaches, visual abnormalities, tinnitus and cognitive dysfunction. Raised intracranial pressure without a secondary identified cause remains a key diagnostic feature of this condition, however, the underlying pathophysiological mechanisms that drive this increase are poorly understood. Previous theories have focused on cerebrospinal fluid (CSF) hypersecretion or impaired reabsorption, however, the recent characterisation of the glymphatic system in many other neurological conditions necessitates a re-evaluation of these hypotheses. Further, the impact of metabolic dysfunction and hormonal dysregulation in this population group must also be considered. Given the emerging evidence, it is likely that IIH is triggered by the interaction of multiple aetiological factors that ultimately results in the disruption of CSF dynamics. This review aims to provide a comprehensive update on the current theories regarding the pathogenesis of IIH.
Topics: Humans; Female; Adolescent; Male; Pseudotumor Cerebri; Intracranial Hypertension; Headache; Obesity
PubMed: 37798095
DOI: 10.1136/jnnp-2023-332222 -
Acta Neurochirurgica Nov 2023Elective use of intraparenchymal intracranial pressure (ICP) monitoring is a valuable resource in the investigation of hydrocephalus and other cerebrospinal fluid...
BACKGROUND
Elective use of intraparenchymal intracranial pressure (ICP) monitoring is a valuable resource in the investigation of hydrocephalus and other cerebrospinal fluid disorders. Our preliminary study aims to investigate ICP changes in the immediate period following dural breach, which has not yet been reported on.
METHOD
This is a prospective cohort study of patients undergoing elective ICP monitoring, recruited between March and May 2022. ICP readings were obtained at opening and then at 5-min intervals for a 30-min duration.
RESULTS
Ten patients were recruited, mean age 45 years, with indications of a Chiari malformation (n = 5), idiopathic intracranial hypertension (n = 3) or other ICP-related pathology (n = 2). Patients received intermittent bolus sedation (80%) vs general anaesthesia (20%). Mean opening pressure was 22.9 mmHg [± 6.0], with statistically significant decreases present every 5 min, to a total reduction of 15.2 mmHg at 20 min (p = < 0.0001), whereafter the ICP plateaued with no further statistical change.
DISCUSSION
Our results highlight an intracranial opening pressure 'spike' phenomenon. This spike was 15.2 mmHg higher than the plateau, which is reached at 20 min after insertion. Several possible causes exist which require further research in larger cohorts, including sedation and pain response. Regardless of causation, this study provides key information on the use of ICP monitoring devices, guiding interpretation and when to obtain measurements.
Topics: Humans; Middle Aged; Intracranial Pressure; Prospective Studies; Hydrocephalus; Pseudotumor Cerebri; Arnold-Chiari Malformation; Monitoring, Physiologic; Intracranial Hypertension
PubMed: 37695437
DOI: 10.1007/s00701-023-05780-7 -
Eye and Brain 2023Cerebrospinal fluid disorders have a wide-ranging impact on vision, headache, cognition and a person's quality of life. Due to advances in technology and accessibility,... (Review)
Review
Cerebrospinal fluid disorders have a wide-ranging impact on vision, headache, cognition and a person's quality of life. Due to advances in technology and accessibility, intracranial pressure measurement and monitoring, usually managed by neurosurgeons, are being employed more widely in clinical practice. These developments are of direct importance for Ophthalmologists and Neurologists because the ability to readily measure intracranial pressure can aide management decisions. The aim of this review is to present the emerging evidence for intracranial pressure measurement methods and interpretation that is relevant to Neuro-ophthalmologists.
PubMed: 37790122
DOI: 10.2147/EB.S404642 -
Expert Opinion on Investigational Drugs 2023Idiopathic intracranial hypertension is a neurological condition characterized by a raised intracranial pressure and papilledema that causes debilitating headaches.... (Review)
Review
INTRODUCTION
Idiopathic intracranial hypertension is a neurological condition characterized by a raised intracranial pressure and papilledema that causes debilitating headaches. While the extent of the pathophysiology is being discovered, the condition is emerging as a systemic metabolic disease distinct to people living with obesity alone. Idiopathic intracranial hypertension is becoming more common and therefore establishing licensed therapeutics is a key priority.
AREA COVERED
The translation of preclinical work in idiopathic intracranial hypertension is evident by the two early phase trials evaluating 11-β-hydroxysteroid dehydrogenase inhibitor, AZD4017, and a glucagon like peptide-1 receptor agonist, Exenatide. This review summarizes these two early phase trials evaluating targeted medicines for the treatment of intracranial pressure. The modulation of these two distinct mechanisms have potential for therapeutic intervention in people living with idiopathic intracranial hypertension.
EXPERT OPINION
The clinical trial landscape in idiopathic intracranial hypertension is a challenge due to the rarity of the disease and the lack of agreed meaningful trial outcomes. Further preclinical work to fully understand the pathogenesis is required to enable personalized targeted drug treatment.
Topics: Humans; Pseudotumor Cerebri; Intracranial Hypertension; Papilledema; Obesity; Headache; Drugs, Investigational
PubMed: 38006580
DOI: 10.1080/13543784.2023.2288073 -
Medicine Oct 2023Idiopathic intracranial hypertension (IIH) is a condition of unknown etiology that primarily affects obese women of childbearing age. Symptoms include disabling... (Review)
Review
Idiopathic intracranial hypertension (IIH) is a condition of unknown etiology that primarily affects obese women of childbearing age. Symptoms include disabling headaches, visual disturbances, and intracranial noises (pulsatile tinnitus). Currently, no standardized treatment guidelines are available and the current management focuses on weight loss and acetazolamide use. There is an increasing body of evidence suggesting that the initial use of topiramate may be considered in IIH treatment. Acetazolamide is the recommended initial treatment for IIH, with topiramate often used as a second-line agent. Topiramate has multiple benefits to indicate it would pose effective in IIH management. Through varying mechanisms, it leads to weight loss and improves migraine headache control, the most common headache phenotype in IIH. Topiramate also inhibits the carbonic anhydrase enzyme like acetazolamide to reduce intracranial pressure and treat papilledema. The safety profile of topiramate is comparable or superior to acetazolamide. To date, there are limited studies comparing topiramate to acetazolamide or other treatment modalities in IIH. Based on its varying mechanisms of action, topiramate is a strong potential treatment agent for IIH, yet acetazolamide is often chosen first-line. However, the data supporting use of acetazolamide or topiramate is inefficient to designate one agent preferred over the other. There is a need for further studies assessing topiramate use in the treatment of IIH, and comparing topiramate use to other treatment modalities.
Topics: Humans; Female; Pseudotumor Cerebri; Acetazolamide; Topiramate; Intracranial Pressure; Weight Loss; Headache; Intracranial Hypertension
PubMed: 37861536
DOI: 10.1097/MD.0000000000035545 -
World Neurosurgery Apr 2024This review explores the future role of venous sinus stenting (VSS) in the management of idiopathic intracranial hypertension and pulsatile tinnitus. Despite its... (Review)
Review
This review explores the future role of venous sinus stenting (VSS) in the management of idiopathic intracranial hypertension and pulsatile tinnitus. Despite its favorable safety profile and clinical outcomes compared with traditional treatments, VSS is not yet the standard of care for these conditions, lacking high-level evidence data and guidelines for patient selection and indications. Current and recently completed clinical trials are expected to provide data to support the adoption of VSS as a primary treatment option. Additionally, VSS shows potential in treating other conditions, such as dural arteriovenous fistula and cerebral venous sinus thrombosis, and it is likely that the procedure will continue to see an expansion of its approved indications. The current lack of dedicated venous stenting technology is being addressed with promising advancements, which may improve procedural ease and patient outcomes. VSS also offers potential for expansion into modulation of brain electrophysiology via endovascular routes, offering exciting possibilities for neurodiagnostics and treatment of neurodegenerative disorders.
Topics: Humans; Treatment Outcome; Pseudotumor Cerebri; Stents; Cranial Sinuses; Endovascular Procedures; Intracranial Hypertension; Retrospective Studies
PubMed: 38590072
DOI: 10.1016/j.wneu.2023.12.128 -
Indian Journal of Ophthalmology Jul 2023To study the safety and efficacy of optic nerve sheath fenestration surgery in patients with optic disc edema due to different etiologies.
PURPOSE
To study the safety and efficacy of optic nerve sheath fenestration surgery in patients with optic disc edema due to different etiologies.
METHODS
Records of 18 eyes of 15 patients who underwent optic nerve sheath fenestration for vision threatening optic disc edema were reviewed retrospectively, and results were analyzed. Improvement of visual acuity was the main measure of outcome. Improved visual fields, resolution of optic disc edema, diplopia, and headache were other benefits that were observed.
RESULTS
Fifteen patients between 13 and 54 years of age were included in the study. Three patients underwent successive bilateral surgery. Idiopathic intracranial hypertension was the most common cause for optic disc edema and was found in 80% of the patients. Mean preoperative logMAR acuity was -1.9789 ± 1.46270, which improved to -0.9022 ± 1.23181 (p < 0.005) in the operated eye, and mean logMAR acuity of contralateral eye improved from -1.3378 ± 1.50107 to -1.0667 ± 1.33813 (p < 0.05).
CONCLUSION
Early optic nerve sheath fenestration is an effective modality for treating optic disc edema due to a wide myriad of causes and helps resolve the associated symptoms.
Topics: Humans; Papilledema; Optic Nerve; Retrospective Studies; Pseudotumor Cerebri; Decompression, Surgical
PubMed: 37417132
DOI: 10.4103/IJO.IJO_3027_22 -
Operative Neurosurgery (Hagerstown, Md.) Oct 2023Idiopathic intracranial hypertension (IIH) is a syndrome characterized with increased intracranial pressure and normal cerebrospinal fluid (CSF) composition. Treatment...
BACKGROUND AND OBJECTIVE
Idiopathic intracranial hypertension (IIH) is a syndrome characterized with increased intracranial pressure and normal cerebrospinal fluid (CSF) composition. Treatment is mainly conservative, whereas CSF diversion surgery is the most frequently used surgical intervention. Endoscopic endonasal optic nerve decompression (EOND) is a newer surgical treatment of this patient group. This study presents a single clinic's case series with comparative results of unilateral an bilateral EOND with or without optic nerve fenestration.
METHODS
Sixteen patients with IIH syndrome who underwent 18 EOND procedures by a single neurosurgeon were evaluated with MRI and digitally subtracted angiography preoperatively. Both preoperative and postoperative visual acuity and perimetry and fundoscopy examinations were routinely performed. All patients underwent sphenoidotomy using the endoscopic binostril approach; however, unilateral or bilateral optic nerve decompression and accompanying optic sheath fenestration was determined on a case-by-case basis, after which all patients were also evaluated for the outcome of headaches.
RESULTS
Most of the patients were female, and the mean age was 30.28 ± 9.78 years. CSF pressure was increased in all patients (406.43 ± 112.91 mm of H 2 O), and the follow-up period was 61.72 ± 21.67 months. In patients with unilateral EOND, visual fields improved in 83%, visual acuity in 70%, headache in 75%, and papilledema in 27% of cases. In patients with bilateral EOND, perimetry improved in 86%, visual acuity in 43%, headache in 50%, and papilledema in 57% of cases.
CONCLUSION
EOND is an effective surgical option in the treatment of IIH. Bilateral decompression is preferable in patients with bilateral visual involvement, and optic nerve fenestration may prove to be helpful in patients with IIH whose primary complaint is headache.
Topics: Humans; Female; Young Adult; Adult; Male; Pseudotumor Cerebri; Papilledema; Optic Nerve; Headache; Decompression
PubMed: 37345928
DOI: 10.1227/ons.0000000000000806 -
The Journal of Headache and Pain Jul 2023In idiopathic intracranial hypertension (IIH), sustained weight loss is the main pillar in modifying disease course, whereby glucagon-like peptide-1 receptor agonists...
BACKGROUND
In idiopathic intracranial hypertension (IIH), sustained weight loss is the main pillar in modifying disease course, whereby glucagon-like peptide-1 receptor agonists (GLP-1-RAs) could present an attractive treatment option.
METHODS
In this open-label, single-center, case-control pilot study, patients with IIH (pwIIH) and a body mass index (BMI) of ≥ 30 kg/m were offered to receive a GLP-1-RA (semaglutide, liraglutide) in addition to the usual care weight management (UCWM). Patients electing for UCWM only served as a control group matched for age-, sex- and BMI (1:2 ratio). The primary endpoint was the percentage weight loss at six months (M6) compared to baseline. Secondary endpoints included the rate of patients with a weight loss of ≥ 10%, monthly headache days (MHD), the rate of patients with a ≥ 30% and ≥ 50% reduction in MHD, visual outcome parameters, and adverse events (AEs).
RESULTS
We included 39 pwIIH (mean age 33.6 years [SD 8.0], 92.3% female, median BMI 36.3 kg/m [IQR 31.4-38.3]), with 13 patients being treated with GLP-1-RAs. At M6, mean weight loss was significantly higher in the GLP-1-RA group (-12.0% [3.3] vs. -2.8% [4.7]; p < 0.001). Accordingly, weight loss of ≥ 10% was more common in this group (69.2% vs. 4.0%; p < 0.001). Median reduction in MHD was significantly higher in the GLP-1-RA group (-4 [-10.5, 0.5] vs. 0 [-3, 1]; p = 0.02), and the 50% responder rate was 76.9% vs. 40.0% (p = 0.04). Visual outcome parameters did not change significantly from baseline to M6. Median reduction in acetazolamide dosage was significantly higher in the GLP-1-RA group (-16.5% [-50, 0] vs. 0% [-25, 50]; p = 0.04). AEs were mild or moderate and attributed to gastrointestinal symptoms in 9/13 patients. None of the AEs led to premature treatment discontinuation.
CONCLUSIONS
This open-label, single-center pilot study suggests that GLP-1-RAs are an effective and safe treatment option for achieving significant weight loss with a favorable effect on headache, leading to reduced acetazolamide dosage in pwIIH.
Topics: Humans; Female; Adult; Male; Pseudotumor Cerebri; Glucagon-Like Peptide-1 Receptor; Acetazolamide; Pilot Projects; Glucagon-Like Peptide 1; Headache; Weight Loss
PubMed: 37460968
DOI: 10.1186/s10194-023-01631-z -
Annals of the Royal College of Surgeons... Aug 2023Since the start of the pandemic, over 400 million COVID-19 swab tests have been conducted in the UK with a non-trivial number associated with skull base injury. Given...
Since the start of the pandemic, over 400 million COVID-19 swab tests have been conducted in the UK with a non-trivial number associated with skull base injury. Given the continuing use of nasopharyngeal swabs, further cases of swab-associated skull base injury are anticipated. We describe a 54-year-old woman presenting with persistent colourless nasal discharge for 2 weeks following a traumatic COVID-19 nasopharyngeal swab. A β2-transferrin test confirmed cerebrospinal fluid (CSF) rhinorrhoea and a high-resolution sinus computed tomography (CT) scan demonstrated a cribriform plate defect. Magnetic resonance imaging showed radiological features of idiopathic intracranial hypertension (IIH): a Yuh grade V empty sella and thinned anterior skull base. Twenty-four hour intracranial pressure (ICP) monitoring confirmed raised pressures, prompting insertion of a ventriculoperitoneal shunt. The patient underwent CT cisternography and endoscopic transnasal repair of the skull base defect using a fluorescein adjuvant, without complications. A systematic search was performed to identify cases of COVID-19 swab-related injury. Eight cases were obtained, of which three presented with a history of IIH. Two cases were complicated by meningitis and were managed conservatively, whereas six required endoscopic skull base repair and one had a ventriculoperitoneal shunt inserted. A low threshold for high-resolution CT scanning is suggested for patients presenting with rhinorrhoea following a nasopharyngeal swab. The literature review suggests an underlying association between IIH, CSF rhinorrhoea and swab-related skull base injury. We highlight a comprehensive management pathway for these patients, including high-resolution CT with cisternography, ICP monitoring, shunt and fluorescein-based endoscopic repair to achieve the best standard of care.
Topics: Female; Humans; Middle Aged; COVID-19; Cerebrospinal Fluid Rhinorrhea; Skull Base; Pseudotumor Cerebri; Fractures, Bone; Nasopharynx; Fluoresceins
PubMed: 36927165
DOI: 10.1308/rcsann.2022.0128