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Treating IIH

Prompt treatment of idiopathic intracranial hypertension (IIH) is crucial in the prevention of permanent vision loss and brain injury.

Prompt treatment of idiopathic intracranial hypertension (IIH) is crucial in the prevention of permanent vision loss and brain injury. Treating this complex disorder requires correcting the anatomical and physiological disturbances that are causing elevated pressures in the brain. Below are approved treatment options that your medical team will consider based on how safe and effective they may be for you.

Weight loss

A conventional treatment option to improve symptoms of IIH is weight loss. A majority of obese -a body mass index (BMI) of 30 or greater– women with IIH had an improvement in their headaches and visual disturbances when they decreased their total body weight by 5-10%.₁ Physical evaluation of these women also found an improvement in their optic and intracranial pressures.₂

Weight loss is not straightforward. Scientific research has discovered significant differences at the cellular level in people who are obese. These findings show alterations in the pathways of food metabolism and permanent chemical responses in the brain in those who are overweight.₃ This means that eating less by calorie restriction may not be completely effective for those who have been battling weight loss for a long time.

Achieving weight loss can depend on many factors that include well-balanced food choices, behavioral changes, realistic forms of physical activity, prescription medications, and in rare cases, consideration of bariatric surgery.₄ Weight loss is a lifelong commitment that involves the support of family, friends, and your medical team.

Medications

Medications used to treat IIH are by prescription only and require close monitoring by a medical professional. There are no over-the-counter medications or herbal supplements that can be used as a replacement for the treatment of IIH.

Carbonic anhydrase inhibitors (CAI) are the standard prescription drug used to treat some symptoms of IIH. Acetazolamide (Diamox) works by decreasing the amount of cerebrospinal fluid (CSF) made, allowing for a decrease in the pressure in the brain. It is a great option because it is non-invasive and can provide symptom relief. The downside is once this medication is stopped, symptoms may return.

If one has a sulfonamide allergy or cannot tolerate taking acetazolamide (Diamox), then a diuretic can be considered. Medications such as furosemide (Lasix) and bumetanide (Bumex) work by increasing urination to remove excess fluid in the body. This in turn can help decrease the amount of CSF surrounding the brain and spinal cord to improve the pressure in the brain. In general, this class of medication comes with a long list of side effects not limited to electrolyte abnormalities, headache, nausea, vomiting, and dizziness.₅

Unfortunately, these medications are not curative and can only maintain patients in remission of this syndrome. In most cases, once a patient is taken off this therapy, symptoms will likely return.

Surgical Options

Optic Nerve Sheath Fenestration (ONSF)

The optic nerve is what sends visual messages from the eye to the brain giving you the ability to see. Around the optic nerve is a thick fibrous layer called the sheath. When pressure builds up between the optic nerve and sheath, it can cause visual changes that can progress to blindness. This finding can be seen in one with IIH.

To preserve vision, a procedure called optic nerve sheath fenestration can be performed by a neuro-ophthalmologist to relieve pressure within the brain. The goal of this treatment is to reverse visual disturbances and prevent permanent vision loss. Known risks of this surgical technique are double vision, pupillary changes, permanent vision loss, and damage to the vessels around the eye.₆

Lumbar Puncture

Also known as a spinal tap, a lumbar puncture is when a needle is inserted into the lower part of your back to enter your spinal canal and to remove cerebrospinal fluid (CSF). This procedure is both diagnostic and therapeutic. This means that CSF can be sent for testing and with removal of this fluid it can be a form of treatment. Certain signs and symptoms of IIH are attributed to the buildup of CSF. With a lumbar puncture, these issues can be resolved with removal of extra fluid. This intervention may need to be performed several times before the consideration of a ventriculoperitoneal or lumboperitoneal shunt.

Ventriculoperitoneal Shunt (VPS) and  Lumboperitoneal Shunt (LPS)

A shunt can provide permanent diversion of CSF in an effort to relieve pressure in the brain. This surgery is performed by a neurosurgeon who will place a long tube in one of the ventricles of the brain (or the lower back) and the other end in the abdominal cavity, with a valve in between that rests under the scalp. This procedure is considered in a person with IIH who does not have any visual involvement with mild to moderate headache symptoms in which medications did not help.₆ Complications of this procedure can be infection of the shunt, clogging of the tube not allowing it to drain CSF and overdrainage of CSF causing a headache. In some cases IIH-related symptoms were not relieved after placement of the ventriculoperitoneal shunt.₆ 

Venous Sinus Stenting (VSS)

Venous sinus stenting has been used in the treatment of IIH for over 20 years. It is an invasive procedure performed by a neurosurgeon. Specific criteria need to be met for one to be considered for placement of a venous sinus stent. The River trial is an ongoing study that is following patients who have had a venous sinus stent placed in the setting of IIH. Up to now, the results are showing the safety and efficacy of this treatment option. Significant improvement in headache, tinnitus, and visual disturbances are observed in patients with IIH after stent placement.₇ Complications of this invasive procedure include blockage of the stent and injury to associated blood vessels.₈

Treating IIH is multifaceted and necessitates a team of neurosurgeons, neurologists, ophthalmologists, and primary care physicians. Each treatment option carries its own risks and benefits and your treating physicians will consider each of them on an individualized basis. The collective goal is to relieve symptoms and prevent permanent brain or visual damage.

  1. Batra N, Prashant P, Wall M. Weight Management in Idiopathic Intracranial Hypertension. EyeRounds.org. Accessed June 2, 2022. https://webeye.ophth.uiowa.edu/eyeforum/tutorials/Weight-management-IIH.htm#:~:text=Establishing%20the%20role%20of%20weight,with%20this%20intervention%20%5B12%5D
  2. Kupersmith M, Gamell, L, Turbin R, et. al. Effects of weight loss on the course of idiopathic intracranial hypertension in women. Neurology. 1998;50(4):1094-1098.
  3. Ochner C, Tsaqi A, Kushner R, et al. Treating obesity seriously: when recommendations for lifestyle change confront biological adaptations. The Lancet Diabetes & Endocrinology. February 11, 2015;3(4): 232-234. https://www.thelancet.com/journals/landia/article/PIIS2213-8587(15)00009-1/fulltext 
  4. Subramanian S, Fletcher W Obesity and Weight Loss in Idiopathic Intracranial Hypertension: A Narrative Review. Journal of Neuro-Ophthalmology. June 2017;37(2):197-205. doi:10.1097/wno.0000000000000
  5. Jacob D, Khan S. How Do Loop Diuretics Work? Rx List. Reviewed October 14, 2021. Accessed June 1, 2022. https://www.rxlist.com/diuretics_loop/drug-class.htm
  6. Spitze A, Lam P, Al-Zubidi N, et al. Controversies: Optic nerve sheath fenestration versus shunt placement for the treatment of idiopathic intracranial hypertension. Indian journal of ophthalmology. October 2014;62(10):1015-1-21. 
  7. Patsalides A. AE-054 The river trial: A prospective single arm trial of stenting the transverse sigmoid sinuses with the river stent in patients with idiopathic intracranial hypertension resistant to medical therapy. Journal of NeuroInterventional Surgery. July 26, 2021;13:A92.
  8. Daggubati K, Liu K. Intracranial Venous Sinus Stenting: A Review of Idiopathic Intracranial Hypertension and Expanding Indications. Cureus. February 4, 2019;11(2):e4008. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6450594/#:~:text=Venous%20sinus%20stenting%20is%20an,pressure%20without%20an%20identifiable%20source.

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