What Is Idiopathic Intracranial Hypertension

What Are The Symptoms Of Iih

The most common symptom of IIH is headache. These headaches are different from most other headaches because they usually get worse with lying down. Many people also have vision problems including blurred vision, dim vision, loss of peripheral vision, flashing lights, vision that dims or blacks out with bending over or straining or double vision. Some people hear a whooshing sound in their head in time with their heart beat.

Causes Of Chronic Intracranial Hypertension

Possible causes of chronic intracranial hypertension include:

• a blood clot on the surface of your brain, known as a chronic subdural haematoma
• an infection in your brain, such as meningitis or encephalitis
• hydrocephalus, where fluid builds up around and inside your brain
• abnormal blood vessel, such as an arteriovenous fistula or arteriovenous malformation
• a blood clot in one of the veins of your brain, known as a venous sinus thrombosis

Rare causes include a blockage in the circulation of fluid at the bottom of the skull , inflammation of the blood vessels in the brain and abnormal skull growth in children .

How Is It Treated

Once IIH is confirmed, comprehensive eye exams and visual field tests are performed in order to monitor any changes in vision.

Recommendations for sustainable weight loss via healthy eating including salt restriction, along with exercise is critical. If lifestyle changes are not successful, weight loss surgery may be recommended for those with a BMI greater than 40.

For many people, weight loss can be difficult. Often times subtle changes to their weight will help relieve symptoms. A drug that decreases CSF production, Acetazolamide , is sometimes used as an alternate therapy and can also aid in weight loss.

In cases where rapid progression is present and the patient does not respond to treatments, surgery may be needed to help relieve the pressure on the optic nerve. Shunting, which involves surgically inserting a tube to drain the CSF, can be used to lower pressure. Another procedure called optic nerve sheath fenestration, where pressure on the optic nerve is relieved, is another a technique not widely utilized.

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Outlook For Idiopathic Ih

Idiopathic IH is not usually life threatening, but can be a lifelong problem. While many people find their symptoms are relieved with treatment, but the symptoms can come back and can have a significant impact on your life.

There’s also a risk that you could lose your vision, even though treatment can help reduce this risk.

Permanent vision loss is estimated to happen to 1 in every 5 to 20 people with idiopathic IH.

Find out more about living with IH at the IIH UK website.

Page last reviewed: 21 October 2019 Next review due: 21 October 2022

How Is Idiopathic Intracranial Hypertension Treated

For most people, intracranial hypertension symptoms improve with treatment. Your provider may recommend:

• Weight loss: If you have a high BMI, weight loss can reduce IIH symptoms. Your healthcare provider may recommend losing 5% to 10% of your body weight.
• Medication: Some medicines manage IIH symptoms. Your provider may prescribe acetazolamide or topiramate to help your body produce less CSF. You may also take a diuretic to decrease fluid retention.
• Surgery: In severe cases, you may need surgery for IIH. Your provider may recommend a spinal fluid shunt. A shunt is a long, thin tube placed in your brain to drain excess CSF. Or you may have an eye surgery called optic nerve sheath fenestration. Your provider makes small incisions around your optic nerve to allow better CSF drainage.

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What’s The Treatment For Iih

For most people, IIH symptoms get better with treatment. Treatments include:

Weight loss. For people who are overweight or obese and have IIH, weight loss is usually the first treatment. Losing about 5 to 10 percent of your body weight can help lessen your symptoms for example, if you weigh 200 pounds, that means losing about 10 to 20 pounds. Talk with your doctor about safe, sustainable ways to lose weight.

Medicine. Your doctor may recommend a medicine called acetazolamide in addition to weight loss. This medicine helps your body make less CSF.

Surgery. If other treatments dont work, your doctor might suggest surgery to help relieve the pressure. In shunt surgery, doctors make a small hole and add a thin tube, called a shunt, to help extra fluid drain from around your brain into the rest of your body. There is also an eye surgery where doctors make a small hole in the covering around the optic nerve.

Some Anatomy Around The Brain

The meninges form the protective lining that surrounds the brain within the skull, and the spinal cord within the backbone . There are three layers of meninges:

• The outermost layer that lies next to the skull or the vertebral column is called the dura mater.
• The middle layer is called the arachnoid mater.
• The inner layer that is closest to the brain or the spinal cord is called the pia mater.

There are also three spaces between the layers of meninges:

• The epidural space – the space between the vertebral column and the dura mater.
• The subdural space – the space between the dura mater and the arachnoid mater.
• The subarachnoid space – the space between the arachnoid mater and pia mater.

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Idiopathic Intracranial Hypertension Without Papilloedema

This is a rare form of IIH where there is no evidence of papilloedema in the setting of raised ICP. Headache is the principle morbidity in these patients.

The diagnostic criteria for definite IIHWOP are the same as for IIH, except there is no papilloedema, but unilateral or bilateral sixth nerve palsies may be present. A diagnosis of possible IIHWOP can be made without the presence of unilateral or bilateral sixth nerve palsies, however in this case at least 3 out of the following 4 features on brain imaging need to be present: an empty sella, flattening of the eye globe, widening of the space around the optic nerve and/or transverse venous sinus stenosis . In patients with IIHWOP risk of vision loss has not been identified and does not seem to develop over the disease course. The commonest symptoms of IIHWOP include headache, pulsatile tinnitus, visual phenomena such as visual obscurations, blurred vision, photopsia and diplopia .

Once a diagnosis of IIHWOP is established, all patients should be counselled about weight management. As there is no threat to vision, long term visual monitoring is not required. Management of headache should be the same as in IIH. Escalation of management to surgery to control elevated ICP in IIHWOP should not be routinely considered.

Diagnosis Of Pseudotumor Cerebri

Diagnosis involves ruling out other health problems including an actual brain tumor. A physical exam and a few tests can help identify pseudotumor cerebri and rule out other causes for pressure inside the skull. The tests include:

• Brain imaging such as MRI or CT scans

• A lumbar puncture to confirm the elevated pressure and withdraw a sample of fluid from around the spine for testing to exclude infectious and inflammatory causes of raised pressure.

• Exam to test eye function

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How Do You Feel

Headaches from IIH can mimic migraine or tension-type headaches, making diagnosis tricky. Additionally, IIH patients do not have a specific type of headache. That being said, a typical IIH headache is severe and throbbing, like a migraine. Pain can be intermittent or constant and may be associated with nausea and/or vomiting. Sometimes, people with IIH headaches notice pain behind the eye and/or eye movement pain.

Modifying The Underlying Disease

Weight loss is disease modifying in IIH and can induce disease remission. Weight loss of 15% in IIH significantly reduces papilloedema and headache. For many patients, weight loss is challenging to achieve and maintain. Patients in the UK NHS with body mass index â¥35 kg/m2 are currently eligible to be referred for weight management, with the aim of reducing weight by 10â15%. Trials are underway to determine whether patients with IIH benefit from weight loss surgery.

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What Can I Do To Manage Iih

• Maintain a healthy weight. Ask your healthcare provider what a healthy weight is for you. Ask your provider to help you create a weight loss plan if you are overweight.
• Eat a variety of healthy foods. You may need to limit the amount of fats and salt you eat. You may also need to limit foods rich in vitamin A and tyramine. Foods rich in vitamin A include beef liver, sweet potatoes, carrots, tomatoes, and leafy greens. Food and drinks that are high in tyramine include cheese, pepperoni, salami, beer, and wine. Ask if you need to be on a special diet.
• Drink liquids as directed. Ask your healthcare provider how much liquid to drink each day and which liquids are best for you.

Indication For Csfpressure Monitoring

Preoperative evaluation of the very young child with persistent symptoms should be undertaken when visual fields cannot be tested.

Preoperative evaluation of children with unremitting symptoms in theabsence of papilloedema who are unresponsive to medication should also be undertaken and they should be taken off the medication for a trial period. This process is essential in order to exclude low pressure headaches.

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Intracranial Hypertension Research Foundation : The First 5013 Nonprofit In The World To Advocate For Collaborate And Support New Medical Research For People Affected By Chronic Intracranial Hypertension Formerly Known As Pseudotumor Cerebri And Benign Intracranial Hypertension

The Intracranial Hypertension Research focuses on the creation, collaboration and support of new medical for chronic intracranial hypertension , formerly known as pseudotumor cerebri and benign intracranial hypertension. We work to expand knowledge about chronic intracranial hypertension, including idiopathic intracranial hypertension and secondary while helping to foster medical research that will lead to better, new treatments and a cure. The IH Research Foundation also offers education and support for people with chronic IH, families, physicians, medical professionals and researchers. We answer questions about chronic intracranial hypertension!

Chronic IH can be a lifelong disorder and is considered a rare disease. Idiopathic intracranial hypertension occurs without an identifiable, underlying cause. Secondary IH means that there is an underlying cause, such as another disease or drug reaction. Intracranial hypertension means high fluid pressure within the skull the pressure pushes on the brain and is transmitted along the optic nerves. Symptoms of chronic intracranial hypertension include vision loss, blindness, extreme pain , and general life-altering disability. Many people with chronic IH cannot work or go to school. Chronic IH occurs in both men, women and children of all races and ethnicities. Being overweight and a woman of childbearing age are associated with idiopathic intracranial hypertension, but not with secondary IH.

What Are The Symptoms Of Intracranial Hypertension

The most common sign of intracranial hypertension is a sudden, severe headache. Sometimes the headache is so painful that it wakes you from sleep. People with IIH may also have a change in vision. You might see double or have sudden blind spots. Several conditions can cause these symptoms, so check with your provider to find out if symptoms are related to IIH or another condition.

Intracranial hypertension symptoms can also include:

• Spinal tap to evaluate your CSF.
• Tests to check your reflexes, balance or muscle strength.
• Visual field test to check for blind spots in your vision.

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Competitive Landscape And Idiopathic Intracranial Hypertension Treatment Market Share Analysis

The idiopathic intracranial hypertension treatment market competitive landscape provides details by competitor. Details included are company overview, company financials, revenue generated, market potential, investment in research and development, new market initiatives, global presence, production sites and facilities, production capacities, company strengths and weaknesses, product launch, product width and breadth, application dominance. The above data points provided are only related to the companies focus related idiopathic intracranial hypertension treatment market.

Some of the major players operating in the idiopathic intracranial hypertension treatment market report are AvKARE, LLC., FDC., Avet Pharmaceuticals Inc., Ingenus, Janssen Global Services, LLC, Lannett, Medtronic, Mercury Pharma Group, Novast Holdings, Ltd., Nostrum Laboratories Inc., Sanofi, SGPharma Pvt. Ltd., Sophysa, Taro Pharmaceutical Industries Ltd., Teva Pharmaceutical Industries Ltd., Hikma Pharmaceuticals PLC, and Zydus Pharmaceuticals, Inc., among others.

Global Idiopathic Intracranial Hypertension Treatment Market Scope And Market Size

The idiopathic intracranial hypertension treatment market is segmented on the basis of drug classification, treatment, end user and distribution channel. The growth among segments helps you analyze niche pockets of growth and strategies to approach the market and determine your core application areas and the difference in your target markets.

On the basis of drug classification, the idiopathic intracranial hypertension treatment market is segmented into acetazolamide, methazolamide, furosemide, topiramate and others. Others are further segmented into tricyclic anti-depressants, beta-blockers and calcium-channel blockers.

On the basis of treatment, the idiopathic intracranial hypertension treatment market is segmented into drugs and surgery. Surgery is further segmented into optic nerve fenestration, neurosurgical shunts and cerebrospinal stenting.

On the basis of end user, the idiopathic intracranial hypertension treatment market is segmented into hospitals, homecare, specialty clinics and others.

On the basis of distribution channel, the idiopathic intracranial hypertension treatment market is segmented into hospital pharmacy, retail pharmacy and online pharmacy.

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Tests For Chronic Intracranial Hypertension

A GP may suspect you have intracranial hypertension if you have symptoms of increased pressure on your brain, such as vision problems and headaches.

You may have several different tests to diagnose IH, such as:

• an examination to check functions such as your muscle strength, reflexes and balance. Any problems could be a sign of an issue with your brain or nerves
• an assessment of your eyes and vision
• a CT scan or MRI scan of your brain
• a lumbar puncture, where a needle is inserted into your spine to check for high pressure in the fluid that surrounds your brain and spinal cord

Idiopathic IH may be diagnosed if you have increased pressure on your brain and no other cause can be found.

Relationship Of Papilledema To Visual Loss

As stated earlier, the site of histologic damage in visual loss due to increased intracranial pressure is at the optic disc. Brain parenchyma tolerates generalized raised intracranial pressure well. Patients do not develop hemianopias. It is unlikely that the intracranial or intraorbital optic nerve is the location of the damage since the visual field defects are not typical and there is no histologic evidence to support this location. There is much evidence that the optic nerve head is an important site of damage in this disorder. This is based not only on histologic evidence, but also, the types of visual field defects seen in patients with IIH are similar to those found in disorders known to affect the optic nerve head — glaucoma and anterior ischemic optic neuropathy. The last site of damage is the retina. Here, there may be either a neurosensory detachment or visual loss from choroidal folds. However, the main location for visual loss in IIH is at the optic disc.

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Optic Nerve Sheath Fenestration

Optic nerve sheath fenestration consists of either creating a window or making a series of slits in the optic nerve sheath just behind the globe. This treatment is preferred for the patient with progressive visual loss with mild or easily controlled headaches, although over 50% of patients with the procedure gain adequate headache control . Since improvement in papilledema may occur in the unoperated eye and fistula formation has been demonstrated the mechanism of action may be local decompression of the subarachnoid space . Occasional failure of the fellow eye to improve and the asymmetry of papilledema may be explained by the resistance to CSF flow produced by the trabeculations of the subarachnoid space. The mechanism of action may also be closure of the subarachnoid space in the retrolaminar optic nerve by scarring. It is likely that both mechanisms contribute to protection of the optic nerve head.

Many large case series attest to the efficacy of this technique.- In these series, postoperative visual acuity or perimetry results were as good as or better than preoperative studies in about 90%. However, occasional patients lose vision in the perioperative period .

Mechanisms Of Visual Loss

The visual field defects found in IIH patients are optic disc-related. In other words, they are the type found when nerve fiber bundles are damaged at the level of the optic disk. These types of defects also occur with glaucoma and anterior ischemic optic neuropathy. This suggests a common mechanism for the visual loss in these disorders.

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What Is Idiopathic Intracranial Hypertension And What Causes It

In idiopathic intracranial hypertension there is raised pressure within the skull . Idiopathic means that the cause of this raised pressure is unknown. Various theories exist as to what may be the cause. Essentially, for some reason, there is too much CSF. The subarachnoid space that contains the CSF cannot expand and, because of this, the pressure around the brain rises.

This raised pressure leads to the symptoms of IIH. It can cause headaches and also swelling of the first part of the optic nerve – the optic disc – at the back of the eye . If papilloedema is not recognised and treated, it can lead to a condition called optic atrophy where there is deterioration and loss of function of the optic nerve. Severe sight impairment can occur as a result of this.

IIH is also known as benign intracranial hypertension. However, this name is not being used as much now. This is because the condition isn’t harmless . It can cause some quite disabling symptoms and can lead to loss of vision if it is not treated. Another old name is ‘pseudotumour cerebri’, as it can lead to some signs and symptoms of a brain tumour, without a brain tumour actually being present.