From the Guidelines
The management of idiopathic intracranial hypertension (IIH) should focus on weight loss, reducing intracranial pressure, preserving vision, and alleviating symptoms, with the primary principle being to treat the underlying disease through weight loss, as stated in the consensus guidelines on management 1. The cornerstone of treatment is weight loss, with a goal of 5-10% total body weight reduction, as this has been shown to reduce intracranial pressure and improve symptoms 1.
- Key aspects of IIH management include:
- Weight loss for patients with a BMI >30 kg/m2, which should be done with sensitivity 1
- Medications such as acetazolamide, typically started at 500 mg twice daily and titrated up to 1000-2000 mg daily in divided doses as tolerated, with side effects including tingling in extremities, altered taste, and fatigue
- Topiramate (25-50 mg twice daily) as an alternative, offering the dual benefit of carbonic anhydrase inhibition and appetite suppression
- Urgent surgical intervention for severe or rapidly progressive visual loss, with options including optic nerve sheath fenestration to protect vision or cerebrospinal fluid diversion procedures (lumboperitoneal or ventriculoperitoneal shunting) to reduce intracranial pressure
- Regular ophthalmologic monitoring, including visual field testing and optical coherence tomography, to assess for papilledema resolution and visual function improvement
- The pathophysiology of IIH involves impaired cerebrospinal fluid absorption leading to increased intracranial pressure, which is why treatments target either CSF production reduction or weight loss to address the underlying mechanisms 1.
- For patients with IIH without papilledema (IIHWOP), management should focus on headache control, with the same approach as typical IIH, and surgical management should not be routinely considered unless advised by experienced clinicians within a multidisciplinary team setting 1.
From the Research
Management Approach for Idiopathic Intracranial Hypertension (IIH)
The management of IIH involves a combination of conservative measures, pharmacological therapy, and surgical intervention.
- Conservative measures focus on weight loss, as IIH is commonly associated with obesity, particularly in young women 2.
- Pharmacological therapy includes the use of diuretics such as acetazolamide, which has been shown to reduce intracranial pressure by inhibiting carbonic anhydrase in the choroid plexus 3.
- Surgical intervention is considered for refractory and sight-threatening cases, with options including cerebrospinal fluid (CSF) diversion, optic nerve sheath fenestration, venous sinus stenting, and bariatric surgery 2, 4, 5.
Surgical Interventions
Surgical treatments are recommended for medically refractory IIH and aim to reduce intracranial pressure, relieve headache, and salvage vision.
- Venous sinus stenting (VSS) has been shown to improve papilledema, visual fields, and headaches in a significant proportion of patients, with a low severe complication rate and failure rate 5.
- CSF diversion techniques, such as shunting procedures, can diminish papilledema, visual field deterioration, and headaches, but are associated with a higher severe complication rate and failure rate compared to VSS 5.
- Optic nerve sheath fenestration (ONSF) can ameliorate papilledema, visual field defects, and headaches, with a low severe complication rate and failure rate 5.
Pharmacological Therapy
Acetazolamide is a commonly used medication for the treatment of IIH, with studies showing its efficacy in reducing intracranial pressure and improving visual outcomes 2, 3.
- However, the evidence for its use is of low certainty due to the limited number of high-quality studies and the risk of bias in existing trials 2.
- Other pharmacological agents, such as methazolamide, zonisamide, and topiramate, may also be effective in the management of IIH, but further research is needed to establish their efficacy and safety 3.