Medical treatment

The most commonly prescribed medication is Acetazolamide (Diamox), a carbonic anhydrase inhibitor used for glaucoma, some types of epilepsy and fluid retention. It reduces CSF production in most patients. Nearly all patients taking Diamox suffer side effects, tingling of the fingers and toes are the most common. Patients prescribed Diamox or any other diuretic should have their progress monitored closely and have regular blood tests. They may be advised to increase their intake of potassium as the body's natural stores can be depleted. As well as standard Diamox a sustained release form of the drug is also available, 'Diamox SR'. Many patients find that the side effects are reduced in comparison with the standard form. Furosemide, a diuretic, is sometimes prescribed but has been proven to have little effect on raised ICP.

On occasion Furosemide and Diamox are used in conjunction with each other. In extreme cases where sight is threatened patients may be treated with a short dose of steroids. Other medications prescribed include Topirimate, Amitriptyline and more. Analgesic’s (pain killers) are used to treat the pain associated with IIH, with varying degrees of success. As with all pain killers care must be taken as many can be addictive and some can have severe side-effects.

Some IIH patients are treated with therapeutic lumbar punctures (LPs) to remove excess CSF on a regular basis.

Non invasive pressure testing is just starting to be evaluated in the UK. Its disadvantage is that no CSF can be drained off to reduce pressure by this technique. Nevertheless, it is also being evaluated by NASA Space Agency for possible use as a CSF pressure measuring device on future space missions as some astronauts have developed IIH-like symptoms during prolonged weightlessness.

Weight Loss

There is now definitive evidence that weight and IIH are related. Weight loss has been shown to be the only treatment to modify the underlying condition. Over 90% of patients diagnosed with IIH are overweight women, suggesting that there is a link between obesity and IIH. Recent studies have shown that weight loss is an effective treatment to reduce papilloedema and headaches, putting IIH in to remission. Unfortunately, clinicians do not yet know exactly how much weight loss is needed. This can be very patient specific. 

Surgical treatment

Surgical intervention is usually only undertaken in severe cases: for example, to protect vision or when medications and other treatments are unsuccessful or not tolerated. There are a range of intervention options including:

Lumboperitoneal (LP) Shunt. This surgery involves diverting excess CSF by inserting a catheter into the subarachnoid space (spinal fluid filled space in the spine) around the patients waist and in to the peritoneum (abdominal cavity). Many LP shunts have a valve system and CSF reservoir.

Ventriculoperitoneal (VP) Shunt. This surgery involves diverting excess CSF by inserting a catheter in to a lateral ventricle (fluid filled space within the brain) down through the neck and into the peritoneum.

A Ventriculoatrial (VA) Shunt surgery is similar to VP shunt surgery diverting excess CSF to the 3rd Atrium of the heart. Some patients have shunt systems that drain the excess CSF to the pleura of their lung.

Special Subcutaneous Lumbar Shunt: This has been developed so that it can be placed in position using local anaesthetic only.

Shunt surgery has proven to be a successful long term relief from IIH symptoms and raised ICP. However for many patients, malfunctions, infection, kinking, blockages and over-draining of shunts are experienced, leading to frequent shunt revision surgery. All surgeries carry risks, can be stressful and have an emotional and physical effect on a patient. Discuss any surgical options with your Specialist to ensure you have all the facts.

Venous Sinus Stenting is also used as a surgical procedure for IIH sufferers who have Venous Sinus Stenosis. Stent surgery is performed after CT venography and manometery investigations. If the patient is found to be a good candidate, a stent is placed within the venous sinus and dilated which can result in the reduction of pressure and relieve symptoms. Stenting is undertaken in specialist hospitals, but to date, no long term evaluation has been on its success on treating IIH in the long term.

ONSF (optical nerve fenestration) may be considered (very rarely) if vision is severely affected or threatened. In this procedure, the sheath surrounding the optic nerve is slit, or a 'window' cut into the sheath to relieve the pressure on the nerve and allow the CSF to escape. ONSF is very effective at relieving the pressure on the optic nerves and thus helps to resolve papilledema. However the amount of CSF drained due to this procedure is negligible. ONSF has little effect on the overall ICP and can lead to complications including blindness.

Long Term Prognosis

Some patient’s symptoms may spontaneously disappear, for others a combination of medical and/or surgical treatments control their condition and they are able to lead relatively 'normal' lives. For others both medical and surgical treatments can be limited in their effectiveness and symptoms may remain. For these patients, treatments with combinations of painkillers and other medications are required to control the symptoms although their effectiveness varies.

Getting the Right Advice

IIH isn't an immediately life threatening condition, but for some people if symptoms cannot be brought under control, it can be a life changing condition. The dangers of unmonitored raised intracranial pressure are life threatening. However, the main risk to life with IIH stems from repeated surgical intervention to insert shunts particularly in the brain.

IIH UK is currently compiling a list of experts who can be contacted by hospitals, Dr’s and Consultants. The list will also be helpful for patients to know which Hospitals in their area have the services and expertise that they need.

IIH UK is also working with IIH experts to encourage widespread knowledge amongst all UK medical staff about IIH and encourage doctors, surgeons and ophthalmologists to contact each other when needing further help or advice when dealing with this deeply complex condition.

Read more about living with IIH

For more information on IIH, please take a look at our leaflets, links and resources page or join the International IIH support forum.

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