From the Guidelines
Idiopathic intracranial hypertension (IIH) workup and treatment should prioritize weight loss, particularly for patients with a BMI >30 kg/m2, as it is the only disease-modifying therapy in typical IIH, as recommended by the consensus guidelines on management 1. The management of IIH involves a multidisciplinary approach, with the primary principle being to modify the underlying disease through weight loss, as stated in the consensus guidelines on management 1.
Key Principles of Management
- Weight loss: recommended for overweight patients, aiming for a 5-10% reduction in body weight, as it can significantly improve symptoms 1.
- Medication therapy: first-line treatment is acetazolamide, starting at 500mg twice daily and titrating up to 1-2g daily as tolerated, which reduces CSF production.
- Protecting vision: serial visual field testing and optical coherence tomography should be performed to monitor for progression.
- Minimizing headache morbidity: treatment focuses on reducing intracranial pressure and preventing vision loss.
Diagnostic Approach
- Brain imaging (preferably MRI with venography) to rule out structural causes.
- Lumbar puncture showing elevated opening pressure (>25 cmH2O) with normal CSF composition.
- CT or MR venography is mandatory to exclude cerebral sinus thrombosis within 24 hours, as recommended by the guidelines on investigation of papilloedema 1.
Treatment Options
- Medical therapy: acetazolamide, topiramate, and high-dose IV steroids for rapidly progressive visual loss.
- Surgical interventions: optic nerve sheath fenestration, CSF shunting procedures (lumboperitoneal or ventriculoperitoneal shunts), and venous sinus stenting in selected cases. Regular follow-up with both neurology and ophthalmology is essential to monitor treatment response and adjust therapy accordingly, as stated in the consensus guidelines on management 1.
From the Research
IIH Workup
- The diagnosis of idiopathic intracranial hypertension (IIH) involves a combination of clinical evaluation, neuroimaging, and measurement of cerebrospinal fluid (CSF) pressure 2, 3.
- Key diagnostic and monitoring techniques for papilledema include neuroimaging, measurement of CSF pressure, perimetry, optical coherence tomography, and ocular sonography 2.
- Neuroradiological signs, such as those seen on MRI or CT scans, can suggest the diagnosis of IIH, especially in patients who do not present with papilloedema 3.
- Other assessments, such as visual evoked potentials, ocular ultrasonography, fundus fluorescein angiography, and autofluorescence, can be useful in the diagnostic process 3.
IIH Treatment
- The main goals of treatment for IIH are to preserve visual function and alleviate symptoms, which can usually be achieved with a combination of weight loss, medical therapies, and surgical interventions depending on the severity of symptoms and vision loss, response to treatment, and subsequent clinical course 4.
- Acetazolamide is considered the mainstay of medical therapy for IIH, as supported by the findings of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) 2.
- Surgical options, such as CSF diversion procedures, endovascular venous sinus stenting, and optic nerve sheath fenestration, may be considered when IIH is refractory to medical treatment or when it presents fulminantly 2.
- A single lumbar puncture may have a moderate effect on ophthalmological outcomes, but its therapeutic effect on headache outcomes is unclear and its usefulness as a therapeutic measure in IIH remains controversial 5.