Idiopathic intracranial hypertension (IIH), also called benign intracranial hypertension and Pseudotumor cerebri syndrome, is a disorder that may arise after there is an increase in the pressure level of the Cerebro-Spinal Fluid (abbreviated as CSF), which is the liquid that surrounds the brain and that lies below the skull in order to protect our brain and spine by working as a muffle or cushion in case we hit our heads with something. The CFS is a liquid that is constantly being produced and reabsorbed back to the bloodstream. This generally allows the pressure level to be constant, unless the disorder takes place.
What Symptoms Does Idiopathic Intracranial Hypertension Present?
Idiopathic intracranial hypertension or IIH presents a series of symptoms that affect various parts of the human organism. Most people suffering from IIH complain from headaches that do not appear in any specific area. Vomits and nausea have also been linked to the syndrome and the headache arising from it. Many also speak of a ringing or beep that goes on in one or both ears in a rhythmic fashion. Doctors call this “Pulsatile Tinnitus”. But not only that, IIH can also affect your vision quite badly actually, by making you see double figures, for example, by creating visual field defects or blurring in the central or peripheral areas of your vision field, or by momentarily darkening it or even completely blacking out your vision (called Transient Obscurations of Vision, or TOV). Patients have also complaint of color vision loss. Finally, a radiating radicular pain in the arms and legs may also be experienced by some patients, but this should not be too severe.
What Causes Idiopathic Intracranial Hypertension?
The precise factors that cause intracranial hypertension in most patients remain unknown. However, there seem to be a link to an excess amount of cerebrospinal fluid located between those organs and the skull.
This liquid acts like a muffle or a sort of airbag that should lessen the effect of a head blow and protect such vital organs from being damaged. The liquid is produced by the brain and latter reabsorbed into your bloodstream, carrying it all across your organism. The increase in the pressure level of this liquid may take place in the absorption process.
What tends to happen is that the pressure of the cerebrospinal fluid increases if the contents of the skull exceed its inner volume. For this to happen, the brain must expand or grown in size, and this happens generally when a brain tumor develops. This same happens, however, when your brain swells or if the amount of cerebrospinal liquid is larger than the average.
Certain scientists have shown that some people suffering from idiopathic intracranial hypertension show a skull structure different from the normal, with a narrowing in specific parts in the brain. It is yet unclear if this is linked to the condition, or if it is an effect or a cause.
Who Is at Risk?
Fortunately for most, IIH is very rare, only around 1 in every 100,000 or more. About 90% of people with IIH are obese middle-aged women. Leaving this two factors aside (women, overweight) there are others that also seem to have a weaker correlation with this condition. These are:
- The consumption of some drugs and medicines, such as steroids, certain antibiotics and contraceptive pills
- Being pregnant
- Other diseases that appear along with it, such as kidney malfunction or sarcoidosis
How Is Idiopathic Intracranial Hypertension Diagnosed?
No doctor will ever diagnose you with idiopathic intracranial hypertension without performing several thorough examinations, neurological exams and tests, most to evaluate a possible abnormal level in your intracranial pressure, vision problems, or to find papilledema.
Some of the tests that you may go through include:
- An examination of the lateral rectus muscles – These muscles help you in the process of moving your eyes around, and if they are weak a double vision may occur.
- A number of visual tests –You might have to take a kind of collyrium or eye drops that will dilate your pupils so the doctor can better examine the interior of your eye bulb and your retina, searching for plausible causes of possible vision problems. Several standard visual tests will also be performed to see how well the patient can see.
- Lumbar puncture – This one will most definitely be performed without certainly confirming you have idiopathic intracranial hypertension. It is also called spinal tap, and it is said to be a little painful. It is used to measure intracranial pressure levels by collecting a small sample of intracranial liquid and searching for possible causes for this.
- Brain imaging and MRI scans – These will search for possible brain tumors, seizures or injuries and other factors that may be causing the symptoms.
What Are the Treatments for Idiopathic Intracranial Hypertension?
In order for patients to feel alleviated from their symptoms or even to make them fully disappear, a series of effective treatments have been developed in laboratories by numerous scientists.
1. Weight Loss
If you are diagnosed with it and you are overweight, your doctor will advise you to lose weight. You may want to go to a dietitian to help you with this. Losing weight may not only improve your symptoms, it may also eradicate them fully. It will also be beneficial for your general wellbeing.
A series of prescriptive drugs are available for the general public at the pharmacies that may help you fight IIH. Some of these are:
- Diuretics –They are sometimes combined with Lasix, a diuretic that increases urine fluid and that way reduces fluid retention and pressure.
- Glaucoma drugs – These medicines can effectively reduce the amount of cerebrospinal fluid that is produced by the brain. Acetazolamide, found in Diamox, has proven to alleviate symptoms in at least half of the patients that have taken it. It can involve damaging side effects, however, such as kidney stones or chronic fatigue.
- Migraine meds – Eve though migraine does not appear to have a direct link with IIH, it has been observed that these medical drugs have often helped patients, not by eradicating the problem from the root, but rather alleviating symptoms, palliating the patient.
Certain surgical methods have also proven to be quite effective, for those patients who want to take their treatment to another level. Doctors speak of two major procedures:
- Optic nerve sheath fenestration – This procedure is performed by the surgical removal or part of the membrane surrounding the patient’s optic nerve. This will also allow an excessive amount of cerebrospinal fluid to leave the skull cavity. It has proven to be highly beneficial when it comes to improving vision problems, even in both eyes while the surgical operation only involves one eye in general. Despite this, in some cases the surgery has no effect and vision actually becomes worse.
- Spinal fluid shunt – This is performed by the insertion of a long thin tube into your skull cavity or spine, which will drain the excess amount of intracranial liquid the patient may have. This treatment is quite complicated however, and it can involve serious side-effects or additional surgeries. That is why it is rather reserved as a sort of Plan B option, in case all others have proven to be ineffective.
Note: About 10% patients experience a progressive vision-loss that may result in absolute blindness. And there exists the possibility of recurrence months or years later. That is why patients should really consider having regular medical check-ups.