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Idiopathic intracranial hypertension is a disorder characterised by raised intracranial pressure that predominantly affects young, obese women. Pathogenesis has not been fully elucidated, but several causal factors have been proposed. Symptoms can include headaches, visual loss, pulsatile tinnitus, and back and neck pain, but the clinical presentation is highly variable. Although few studies have been done to support evidence-based management, several recent advances have the potential to enhance understanding of the causes of the disease and to guide treatment decisions. Investigators of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) reported beneficial effects of acetazolamide in patients with mild visual loss. Studies have also established weight loss as an effective disease-modifying treatment, and further clinical trials to investigate new treatments are underway. The incidence of idiopathic intracranial hypertension is expected to increase as rates of obesity increase; efforts to reduce diagnostic delays and identify new, effective approaches to treatment will be key to meeting the needs of a growing number of patients.

Idiopathic intracranial hypertension (IIH) is a neuro-ophthalmological condition characterised by a raised intracranial pressure and papilloedema that causes disabling headaches. The main risk factors of female sex and living with obesity have been known for some time, however the knowledge of the underlying pathophysiology is evolving. Papilloedema can impact the visual function, and the majority of people are offered acetazolamide. Those with sight threatening disease need urgent management, though there is little high quality evidence to recommend any particular surgical intervention. Headache treatment is an unmet clinical need and simple medication overuse advice has the potential to reduce the chronification of migraine-like headaches. IIH is emerging as a systemic metabolic disease distinct from people living with obesity alone. While weight loss is the main stay of disease modifying therapy this is challenging to access and many healthcare professionals that manage the condition have no formal training or accessible pathways for weight management. The aim of this “how to do it” article is to present the latest advances in knowledge of IIH that we pragmatically included in routine clinical care for people living with the condition.

Assessment and monitoring of intracranial pressure (ICP) are important in the management of traumatic brain injury and other cerebral pathologies. In the eye, ICP elevation and depression both correlate with optic neuropathies, the former because of papilledema and the latter related to glaucoma. While the relationship between ICP elevation and papilledema is well established, the relationship between low ICP and glaucoma is still poorly understood. So far, ICP monitoring is performed invasively, but this entails risks including infection, spurring the study of non-invasive alternatives. We review 11 methods of non-invasive estimation of ICP including correlation to optic nerve sheath diameter, intraocular pressure, ophthalmodynamometry and two-depth transcranial Doppler of the ophthalmic artery. While none of these methods can fully replace invasive techniques, certain measures show great potential for specific applications. Although only used in small studies to date, a MRI based method known as MR-ICP, appears to be the best non-invasive technique for estimating ICP, with two-depth transcranial ultrasound and ophthalmodynamometry showing potential as well.

BackgroundIdiopathic intracranial hypertension (IIH) is a potentially disabling condition. There is a lack of evidence and national guidance on how to diagnose and treat paediatric IIH, leading to variation in clinical practice. We conducted a national Delphi consensus via the Children’s Headache Network to propose a best-practice diagnostic and therapeutic pathway.MethodsThe Delphi process was selected as the most appropriate methodology for examining current opinion among experts in the UK. 104 questions were considered by 66 healthcare professionals, addressing important aspects of IIH care: assessment, diagnosis, treatment, follow-up and surveillance. General paediatricians, paediatric neurologists, ophthalmologists, opticians, neuroradiologists and neurosurgeons with a clinical interest or experience in IIH, were invited to take part.ResultsThe Delphi process consisted of three rounds comprising 104 questions (round 1, 67; round 2, 24; round 3 (ophthalmological), 13) and was completed between March 2019 and August 2021. There were 54 and 65 responders in the first and second rounds, respectively. The Delphi was endorsed by the Royal College of Ophthalmologists, which engaged 59 ophthalmologists for round 3.ConclusionsThis UK-based Delphi consensus process reached agreement for the management of paediatric IIH and has been endorsed by the Children’s Headache Network and more broadly, the British Paediatric Neurology Association. It provides a basis for a pragmatic clinical approach. The recommendations will help to improve clinical care while minimising under and over diagnosis.

We aim to evaluate the retinal nerve fiber layer (RNFL) thickness measured with optical coherence tomography (OCT) in comparison with papilledema grade, and to assess the relationship between RNFL thickness, papilledema grade, and intracranial pressure (ICP) in idiopathic intracranial hypertension (IIH). Sixty-five patients with active IIH (AIIH) with papilledema, 39 with chronic IIH (CIIH) without papilledema and 80 healthy controls (HC) were examined with OCT and fundus imaging. RNFL thickness, papilledema grade and ICP level were assessed in 55 with AIIH and 26 with CIIH. RNFL thickness was significantly higher in AIIH compared to CIIH or HC. RNFL thickness correlated strongly with papilledema grade (coefficient 0.78, p < 0.01) and moderately with ICP (coefficient 0.569, p < 0.01). RNFL thickness was associated with papilledema progression (R 2  = 0.656, p < 0.01): specifically, with increases of 9 µm from normal to mild grade (p > 0.05), 91 µm from normal to moderate (p < 0.01), and 214 µm from normal to severe (p < 0.01). ICP showed a weaker correlation with papilledema grades (R 2  = 0.339, p < 0.05), with significant increase (8 cm H 2 O, p < 0.01) only from normal to severe papilledema. RNFL correlated strongly with papilledema grade and moderately with ICP levels. RNFL thickness increased proportionally per papilledema grade.

Purpose of ReviewIdiopathic intracranial hypertension (IIH), pseudotumor cerebri syndrome (PTCS), and benign intracranial hypertension are all terms that have been used for a neurologic syndrome consisting of elevated intracranial pressure (ICP), headache and vision loss without mass lesion or underlying infection or malignancy. In this review article, categorization, diagnostic criteria, symptom management strategies, and disease treatment options for pseudotumor cerebri syndrome will be discussed.Recent FindingsThe Idiopathic Intracranial Hypertension Treatment Trial has now proven that acetazolamide should be the first line therapy in primary PTCS, but other treatment options exist in patients who cannot tolerate acetazolamide or in selected cases, which requires surgical intervention for PTCS which acutely threatens vision. Headache has also been shown to require focused treatment beyond therapies that lower ICP, specifically targeting coexistent primary headache disorders and medication overuse.SummaryAdvances in treatment and diagnostic modalities have improved understanding of PTCS types and their treatment. The pathophysiology of primary PTCS, however, remains incompletely understood, but continued evaluation of cerebrospinal fluid flow dynamics, aquaporins, hormones, natriuretic peptides, and the link with female gender and obesity may lead to future answers.

Purpose of Review Rebound intracranial hypertension (RIH) is a post-procedural treatment complication in patients with spontaneous intracranial hypotension (SIH) characterized by transient high-pressure headache symptoms. This article reviews the epidemiology, clinical features, risk factors, and treatment options for RIH. Recent Findings This article discusses how changes in underlying venous pressure and craniospinal elastance can explain symptoms of RIH, idiopathic intracranial hypertension (IIH), and SIH. Summary The pathophysiology of RIH provides a clue for how high and low intracranial pressure disorders, such as IIH and SIH, are connected on a shared spectrum.

Background Idiopathic intracranial hypertension (IIH) mainly affects obese young women, causing elevated intracranial pressure, headaches, and papilledema, risking vision loss and severe headaches. Despite weight loss as the primary treatment, the underlying mechanisms remain unclear. Recent research explores novel therapeutic targets. Aims This review aimed to provide a comprehensive understanding of IIH's pathophysiology and clinical features to inform pathogenesis and improve treatment strategies. Methods Recent publications on IIH were searched and summarized using PubMed, Web of Science, and MEDLINE. Results The review highlights potential pathomechanisms and therapeutic advances in IIH. Conclusion IIH incidence is rising, with growing evidence linking it to metabolic and hormonal disturbances. Early diagnosis and treatment remain challenging. We integrate recent studies and findings to provide a thorough and detailed exploration of various facets of idiopathic intracranial hypertension, including its epidemiology, pathophysiological mechanisms, clinical features, and treatment strategies. This contributes to a better understanding of the disease and potential therapeutic targets.

The application of Friedman’s criteria to diagnose suggested IIH WOP will prevent many chronic patients with migraine (CM) from proper diagnosis and treatment. Our prospective study aimed to compare the prevalence of suggested IIH WOP in case of following Friedman’s criteria and in case of novel proposed criteria (OP > 200 mmH2O and radiological finding ≥ two), also reporting the predictive radiological signs for IIH WOP. Refractory chronic patients with migraine underwent ophthalmologic, neurological evaluation, MRI, and a lumbar puncture (LP) with opening pressure (OP) measurement. CSF withdrawal was performed in patients with CSF OP > 200 mmH20. Suggested IIHWOP was defined according to Friedman’s criteria and our novel criteria. Regression analysis was performed to detect the strongest predictor of suggested IIHWOP. The effect of CSF withdrawal was evaluated clinically after two months. One hundred and two consecutive CM were enrolled (95 F, age 32.34 ± 9.45, and BMI 29.04 ± 5.89) without papilledema. Eighteen patients (17.65%) had OP greater than 250 mmH2O, and 20 patients (19.61%) with OP ≥ 200 mmH2O and ≤ 250 mH2O. Prevalence of suggested IIH WOP, according to Friedman’s diagnostic criteria, was three patients (2.9%) only. In case of our novel diagnostic criteria (Absent 6th nerve palsy, ICP > 200 mmH2O, and ≥ two radiological signs), eight patients (7.8%) were discovered. After CSF withdrawal, 85% of patients with migraine with OP > 200 mm H2O improved, specially CM with bilateral Transverse sinus stenosis (TSS). The prevalence of suggested IIH WOP in CM was 7.8%. Bilateral TSS was the only predictor of IIHWOP.