Emerging themes in idiopathic intracranial hypertension

Abstract

Purpose Idiopathic intracranial hypertension (IIH) is a rare disorder characterised by raised intracranial pressure. The underlying pathophysiology is mostly unknown and effective treatment is an unmet clinical need in this disease. This review evaluates key emerging themes regarding disease characteristics, mechanisms contributing to raised intracranial pressure and advances in potential therapeutic targets. Findings IIH is becoming more common, with the incidence rising in parallel with the global obesity epidemic. Current medical management remains centred around weight management, which is challenging. Metabolic investigations of patients have identified specific androgen profiles in cerebrospinal fluid (CSF), which suggest an endocrine dysfunction impacting CSF secretion in IIH. Glucagon-like peptide-1 (GLP-1) and 11β-hydroxysteroid dehydrogenase type 1 (11β-HSD1) have been found to play a role in CSF dynamics in IIH and have formed the basis of the first clinical trials looking at new treatments. Conclusions Identification of novel molecular targets thought to underlie IIH pathology is now being translated to clinical trials.

Idiopathic Intracranial Hypertension: Evaluation of Admissions and Emergency Readmissions through the Hospital Episode Statistic Dataset between 2002–2020

“…When compared to the primary care data in the UK, which have previously been published, the incidence over corresponding years is similar. [ 4 ] Likewise, within this cohort there are patients over the age of 65 years ( Figure 2 A), which is not typical of the known phenotype of IIH, described by the IIH literature as a disease that occurs with the major risk factor being weight gain in women of working age [ 1 , 2 , 7 ]. A recent retrospective case series analysis of IIH above the age of 65 years reported that the diagnosis was more commonly seen in males, with incidentally found papilloedema; they had fewer headaches, and on investigation had lower lumbar puncture opening pressure, as compared to IIH controls below the age of 50 years [ 48 ].…”

“…Changing awareness of the condition due to publication of the IIH guidelines [ 23 , 24 ] and emerging evidence [ 1 , 2 ] could have influenced the trends, in particular, the recommendation that CSF shunting should only be performed for sight threatening disease and not headache symptoms. Similarly, recommendations for medical headache management could have possibly reduced the number of those who had previously been admitted for repeat lumbar punctures or sought surgery for management.…”

“…Idiopathic Intracranial Hypertension (IIH) is characterized by an elevation of intracranial pressure (ICP) with no identifiable cause [ 1 , 2 ]. There is a rising incidence in this disease, and it appears that the incidence is related to country specific prevalence of obesity [ 3 , 4 , 5 ].…”

Styloidogenic-cervical spondylotic internal jugular venous compression, a vascular disease related to several clinical neurological manifestations: diagnosis and treatment—a comprehensive literature review

“…Impairment in venous drainage reflects on CSF dynamics that could be responsible of several symptoms associated to styloidogenic jugular vein compression (headache, tinnitus, visual disturbances) (29)(30)(31).…”

Awareness, Diagnosis and Management of Idiopathic Intracranial Hypertension

“…Current medical management remains centered around weight management, which is challenging. Identification of novel molecular targets thought to underlie IIH pathology is now being translated to clinical trials [ 37 , 38 ].…”