What are the recommended first-line treatment options for managing idiopathic intracranial hypertension?

First-line treatment options for managing idiopathic intracranial hypertension (IIH) primarily focus on reducing intracranial pressure and preserving vision. The initial management typically includes weight loss through lifestyle modifications, as obesity is a major risk factor for IIH. Pharmacological treatment with acetazolamide, a carbonic anhydrase inhibitor, is commonly recommended to reduce cerebrospinal fluid production and lower intracranial pressure. Acetazolamide is considered the first-line drug therapy and has been shown to improve symptoms and visual outcomes.

In cases where acetazolamide is not tolerated or contraindicated, alternative medications such as topiramate may be considered, which also has carbonic anhydrase inhibitory effects and can aid in weight loss. However, specialist advice is often sought for alternative or adjunctive pharmacological treatments.

For patients with severe or rapidly progressive visual loss, or those who do not respond adequately to medical therapy, surgical options such as cerebrospinal fluid shunting or optic nerve sheath fenestration may be necessary, but these are not first-line treatments and require specialist referral.

Regular monitoring of visual function and symptoms is essential to guide treatment adjustments. The management approach is multidisciplinary, involving neurologists, ophthalmologists, and sometimes neurosurgeons.

This treatment strategy aligns with the European Headache Federation guideline and other recent literature emphasizing acetazolamide as the cornerstone of medical management, alongside weight management and close monitoring Hoffmann et al. 2018Biousse 2012Batra & Sinclair 2014.