From the Guidelines
Idiopathic intracranial hypertension (IIH) should be managed with a focus on weight management, as all patients with IIH should be counselled about weight management, regardless of their weight 1.
Diagnosis and Treatment
The diagnosis of IIH is based on the presence of increased intracranial pressure without an identifiable cause, normal cerebrospinal fluid composition, and the absence of hydrocephalus or mass lesion 1.
Key Principles of Management
The management of IIH involves three main principles:
- Treating the underlying disease
- Protecting the vision
- Minimizing headache morbidity 1
Treatment Options
Treatment options for IIH include:
- Weight loss for overweight patients, as even modest weight reduction (5-10% of body weight) can significantly improve symptoms
- Acetazolamide as the primary medication, typically started at 500mg twice daily and increased as needed up to 2000-2500mg daily, divided into doses
- Topiramate (25-50mg twice daily) as an alternative for patients who cannot tolerate acetazolamide, with the added benefit of migraine prevention
- Surgical interventions, such as optic nerve sheath fenestration or CSF shunting procedures, in severe cases with progressive vision loss
Monitoring and Follow-up
Regular ophthalmologic monitoring is essential, typically every 4-6 weeks initially, then less frequently as the condition stabilizes 1.
Important Considerations
It is essential to note that the risk of vision loss in patients with IIH without papilloedema (IIHWOP) has not been identified, and these patients should be managed similarly to those with typical IIH, with a focus on headache management and weight counselling 1.
From the FDA Drug Label
For adjunctive treatment of: edema due to congestive heart failure; drug-induced edema; centrencephalic epilepsies (petit mal, unlocalized seizures); chronic simple (open-angle) glaucoma, secondary glaucoma, and preoperatively in acute angle-closure glaucoma where delay of surgery is desired in order to lower intraocular pressure. The FDA drug label does not answer the question.
From the Research
Diagnosis of Idiopathic Intracranial Hypertension
- Idiopathic intracranial hypertension (IIH) is a diagnosis of exclusion, meaning that other causes of increased intracranial pressure must be ruled out through history, imaging, and cerebrospinal fluid examination before the diagnosis can be made 2, 3.
- The condition is characterized by elevated intracranial pressure without radiological or laboratory evidence of intracranial pathology, except for empty sella turcica, optic nerve sheath with filled out cerebrospinal fluid spaces, and smooth-walled nonflow-related venous sinus stenosis or collapse 4.
- IIH typically affects obese women of childbearing age, and the incidence is increasing with the rising prevalence of obesity 4, 5.
Symptoms and Clinical Findings
- Persistent headache is the most common symptom of IIH, and visual impairment is a serious complication that may not be recognized by patients 4, 5.
- Papilledema is the major clinical finding, and if left untreated, it can cause progressive irreversible visual loss and optic atrophy 2, 3.
Treatment Options
- The main goals of treatment are alleviation of symptoms, including headache, and preservation of vision 2.
- For overweight or obese patients with IIH, weight reduction of 5% to 10% of total body weight at diagnosis is a long-term treatment strategy, along with a low-salt diet 2, 6.
- Medical treatment with acetazolamide is often initiated, and other medical treatments can be added or substituted when acetazolamide is insufficient as monotherapy or poorly tolerated 2, 6, 4.
- Surgical interventions, such as optic nerve sheath fenestration or cerebrospinal fluid shunting, may be required to prevent further irreversible visual loss in cases of severe or rapidly progressive visual deterioration 2, 6, 4, 3.
Management and Follow-up
- Visual surveillance is vital, as up to 25% of patients with IIH may develop visual impairment from optic atrophy 3.
- Long-term follow-up is recommended, as the disease may worsen after an initial period of stability 3.
- The choice of intervention depends on the relative severity of symptoms and visual loss, as well as local expertise 2, 6.