Management strategies for idiopathic intracranial hypertension

Abstract

Idiopathic intracranial hypertension (IIH) is a debilitating medical condition with an unknown cause. Its a diagnosis of exclusion and alternative etiologies of raised increased intracranial pressure should be investigated. Unfortunately, to date, there have been no clinical trials to guide treatment of IIH. Current treatment consists of medical and surgical therapy. Medical therapy with weight loss and diuretics, for example, acetazolamide, is typically the mainstay of treatment for IIH. Patients who fail medical therapy due to intolerance, non-compliance, or patients with fulminant IIH may require further surgical treatment. The decision of which surgical procedure is optimal depends on the patient’s predominant symptoms (refractory headache vs risk of vision loss), the availability of local oculoplastic/neurosurgical expertise (optic nerve sheath fenestration or shunting) and patient preference. In this review the authors provide an overview of the different treatment options and outline the latest advances and strategies in the treatment of IIH.

Keywords::

• acetazolamide
• cerebrospinal fluid shunting
• idiopathic intracranial hypertension
• optic nerve sheath fenestration
• papilledema
• pseudotumor cerebri
• topiramate
• transverse venous stenting

Financial & competing interests

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending or royalties.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Idiopathic intracranial hypertension (IIH) is a debilitating illness with unknown cause.

• Typically, IIH is a disease of young, obese women.

• To date, there are no executed clinical trials to guide treatment of IIH.

• Weight loss is considered the best long-term treatment for IIH.

• Medical treatment can be achieved with use of acetazolamide, furosemide, topiramate and so on.

• Twenty-five percent of patients fail medical management indicated by acute onset of visual field (VF) defects or progression of VF defects; acute worsening of visual acuity secondary to papilledema; severe headache resistant to the standard medical therapy and psychosocial reasons, for example., inability to perform VF studies or non-compliance. Optic nerve sheath fenestration, cerebrospinal fluid shunting procedures and venous sinus stenting should be considered.

• The choice for surgical treatment of IIH depends in part on availability, surgeon’s experience and patient’s preference.

• The future is promising and should provide more answers and insight about the pathophysiology, and evidence-based guidelines in the treatment of IIH.