Progress and recognition of idiopathic intracranial hypertension: A narrative review

Abstract

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.

Keywords: 11β‐hydroxysteroid dehydrogenase type 1 (11β‐HSD1); glucagon‐like peptide‐1 (GLP‐1); idiopathic intracranial hypertension (IIH); intracranial pressure (ICP); venous sinus stenting (VSS).

FIGURE 1

Proposed pathophysiological mechanisms underlying IIH and the target sites of major therapeutic agents. The pathogenesis of IIH is multifaceted, with no single explanation; key mechanisms include heightened CSF secretion from the CP, decreased CSF reabsorption through AG, venous sinus stenosis leading to venous hypertension, and dysfunction within the lymphatic and glymphatic systems. Additionally, adipose tissue dysfunction and hormonal disruption in IIH play an important role in promoting CSF secretion. Both acetazolamide and topiramate work by inhibiting carbonic anhydrase activity. AZD4017 acts as an inhibitor of 11β‐HSD1, thereby diminishing the local concentration of cortisol. GLP‐1R agonists attach to and stimulate the GLP‐1R, leading to the suppression of NKA activity and a decrease in sodium excretion. 11β‐HSD1, 11β‐hydroxysteroid dehydrogenase type 1; AG, arachnoid granulation; AQP‐1, aquaporin‐1; CA, carbonic anhydrase; CP, choroid plexus; CSF, cerebrospinal fluid; GLP‐1R, glucagon‐like peptide‐1 receptor; IAP, intra‐abdominal pressure; ICP, intracranial pressure; IIH, idiopathic intracranial hypertension; ITP, intrathoracic pressure; NKA, Na⁺/K⁺‐ATPase; NKCC1, Na⁺‐K⁺‐2Cl ⁻ cotransporter.

FIGURE 2

Management of IIH. The treatment of IIH focuses on vision preservation and headache relief. Effective weight loss and long‐term weight control are the core of IIH management. Overweight or obese patients should adopt a scientific and healthy lifestyle intervention for weight loss, including diet, exercise and behavioral intervention. 11β‐HSD1, 11β‐hydroxysteroid dehydrogenase type 1; CSF, cerebrospinal fluid; GLP‐1, glucagon‐like peptide‐1; IIH, idiopathic intracranial hypertension; ONSF, optic nerve sheath fenestration; VSS, venous sinus stenting.