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Increased intracranial pressure is an under-diagnosed cause of difficult to treat headaches. Persistent chronic headaches that do not respond to treatment may be due to increased pressure inside the head. These headaches may resemble chronic migraine headaches and many doctors will try treating these patients with preventive medications, such as Neurontin (gabapentin), Topamax (topiramate), amitriptyline (Elavil), or Botox injections. If these approaches do not provide relief, measurement of intracranial pressure should be considered. Most patients who suffer from increased intracranial pressure have swelling of the optic nerves (papilledema), which can be detected by examining the back of the eye, a standard part of a neurological and ophthalmological examination. However, some people with increased pressure do not have papilledema and they are the ones who present a diagnostic challenge. This condition is also called pseudotumor cerebri because tumors also raise intracranial pressure. To measure the pressure a spinal tap (lumbar puncture) is performed. The cerebrospinal fluid circulates around the brain, within its ventricles and around the spinal cord. Putting a needle into the spinal fluid at the lumbar spine level is much safer than anywhere else and gives the reading of the pressure everywhere within this enclosed space, including the brain.

Factors that predispose to increased intracranial pressure include delayed effects of a head trauma, certain medications, excessive amounts of vitamin A, obesity, and other. One of the more recent theories suggests that narrowing of the veins that drain blood from the brain is responsible for this condition. This diagnosis is made by performing an angiogram or a magnetic resonance venogram (MRV, a test done by an MRI machine), tests that show blood vessels.

In addition to headaches, increased pressure can cause nausea, dizziness, pulsating noise in the ears, and blurred vision. If left untreated, the increased pressure can lead to loss of vision.

If no obvious causes are found the condition is called idiopathic intracranial hypertension. Its treatment begins with the attempts to lose weight if the person is overweight. Pregnant women who are more prone to develop this condition often obtain relief after the delivery. Medications that can help include acetazolamide (Diamox) and topiramate (Topamax). If medications are ineffective a neurosurgeon can place a shunt that drains cerebrospinal fluid into the abdomen. This is a relatively simple procedure, but it does carry a risk of infections and other complications. Shunting is reserved for patients who have uncontrollable headaches or are threatened with loss of vision.

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Spinal tap, or lumbar puncture headache occurs in one out of four people undergoing this procedure. Spinal tap is usually done to examine spinal fluid for infections, bleeding, multiple sclerosis, and other conditions. A small percentage of people undergoing epidural anesthesia, which involves placement of the same kind of needle into the same space between vertebrae, also develop a spinal tap headache. This happens because the needle is accidentally placed too far and it causes a leak of spinal fluid. Spinal tap headache is very easy to diagnose – it stops as soon as the person lies down and begins within minutes of sitting up. Normally, the brain floats in cerebrospinal fluid, but if this fluid is drained away by a spinal tap, the brain sags, pulls on the brain coverings, called meninges, and causes a severe headache. The majority of people do not develop this headache after a spinal tap because as soon as the needle is withdrawn, the hole in the dural sac that covers the spinal cord and the brain closes. In some people, especially if it takes a few sticks to get the fluid flowing and with a larger needle, the hole may not close right away and the fluid keeps leaking inside the spine. In most people the headache stops on its own within a day or two. If it doesn’t, the problem can be fixed by a “blood patch” procedure. It involves taking the patient’s own blood from the vein and injecting it into the same space between vertebrae where the spinal tap was done. Patient’s blood clots and seals the persistent leak of the cerebrospinal fluid, which stops the headache, often within minutes.
A similar headache can rarely occur without a spinal tap or even a trauma to the spine. It is called spontaneous low cerebrospinal fluid headache and it is also very positional, meaning that it gets better when the person is lying down. This headache is more difficult to diagnose, but an MRI scan of the brain sometimes shows inflamed meninges around the brain, which suggests this diagnosis. Finding a leak is more difficult and requires looking at the flow of the spinal fluid and searching for a leak. When a single leak is found, a blood patch procedure can help, but with multiple leaks the treatment becomes more complicated. A single case of using Botox to helps this type of headaches was described here last year.

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Botox has been shown to relieve headaches of low spinal fluid pressure in a case reported at the last annual scientific meeting of the American Headache Society in Washington DC. Low spinal fluid headache usually occurs after a spinal tap (lumbar puncture) or rarely without an obvious cause. The diagnosis is made by doing a spinal tap which normally shows low pressure and by a characteristic appearance of the MRI scan of the brain. The woman who was treated by doctors from the Mayo Clinic had a spontaneous leak of the spinal fluid and did not respond to blood patches which is the first-line treatment for this condition and consists of injections of person’s own blood into the area around the leak. She also did not respond to a variety of medications. Botox injections provided her with relief for the first time in 20 years. She has continued to receive Botox injections for three years now with sustained results. It somewhat surprising that Botox would help because the cause of low pressure headaches is thought to be tugging on the nerves due to sagging of the brain, which normally is floating in the spinal fluid. It is possible that Botox just stops pain sensations regardless of the cause, whether it is due to migraine, shingles or other nerve disturbance.

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Ehlers-Danlos syndrome is a group of inherited disorders that are notable for excessive joint mobility with some people also having lax or stretchy skin, at times heart problems, and other symptoms. Headaches appear to be also very common.

We see Ehlers-Danlos syndrome in many of our migraine patients and most of our headache specialist colleagues also notice this association. However, there are very few studies that confirm this observation. One such study was recently presented at the annual scientific meeting of the American Headache Society in Washington, DC. The research was performed at a cardiology clinic in Texas. They looked at the records of 139 patients who were referred to this clinic in a period of one year. Of these 139 patients with Ehlers-Danlos syndrome, 90% were women and the average age was 32. Out of 139 patients, 70% suffered from headaches – 32% had tension-type, 26% had migraines, 9% had chronic migraines and 2% had sinus headaches. These numbers are much higher than what is seen in the general population, confirming clinical observations by headache specialists.

One form of Ehlers-Danlos syndrome affects not only joints and ligaments, but also the heart. So, when see a migraine patients who also appears to have Ehlers-Danlos syndrome, we also ask about symptoms related to the heart and if they are present refer such patients to a cardiologist.

Another presentation at the same meeting described a 23-year-old woman with Ehlers-Danlos syndrome who suddenly developed headaches that would worsen on standing up and improve on lying down. This is typical of headaches due to low cerebrospinal fluid (CSF) pressure, which was confirmed by a spinal tap. The most common causes of low CSF pressure are a leak caused by a spinal tap done to diagnose a neurological disease or caused by a complication of epidural anesthesia. Spontaneous unprovoked leaks have also been reported. In this patient with Ehlers-Danlos syndrome the leak probably occurred because of the lax ligaments that surround the spinal canal and contain the CSF. The report describes the most accurate test to document such leaks, which is an MRI myelogram.

The treatment of CSF leaks begins with a blood patch procedure, but if it is ineffective, surgery is sometimes done to repair the leak. A recent report suggested that Botox could be effective for low spinal fluid pressure headaches.

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While being overweight doese not cause migraines, in those who do suffer with migraines there is an inverse relationship between person’s weight and the frequency and severity of migraine headaches. Weight loss, including that due to weight loss (bariatric) surgery, has been reported to reduce the frequency of migraine headaches and migraine-related disability. Obesity is also associated with headaches due to increased intracranial pressure (also called pseudotumor cerebri) and losing weight improves such headaches as well.

However, while bariatric surgery may improve migraines, in a small number of people it can cause a different type of headaches. This rare type of headache is caused by a spontaneous leak of cerebro-spinal fluid (CSF), the fluid which surrounds the brain and the spinal cord. Such leaks are common after a spinal tap or can be a complication of epidural anesthesia. Loss of CSF can cause severe headaches, which are strictly positional. They are severe in the upright position, sitting or standing, but quickly improve upon lying down.

A study of 338 patients who underwent bariatric surgery at the Cedars-Sinai Medical Center in Los Angeles detected 11 patients who developed a spontaneous CSF leak with severe headaches. Headaches started anywhere within three months and 20 years after surgery. Clearly, headaches starting 20 years later are not likely to be related to surgery, which suggests that this link between bariatric surgery and headaches is far from proven. Of these 11 patients, 9 improved with treatment. The typical treatment for a CSF leak is a “blood patch” procedure, which involves taking blood from the patient’s vein and injecting it into the area of the leak. When blood clots, it usually seals the leak and the headache improves within hours.

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Idiopathic intracranial hypertension is also called pseudotumor cerebri because just like with a brain tumor, the
pressure is increased inside the skull. This condition usually presents with a headache and sometimes with visual symptoms. Increased intracranial pressure is not only a very painful condition, but also, if left untreated, can cause loss of vision and strokes.

An observational study just published in JAMA Neurology reports on 165 patients with pseudotumor seen by a group of neurologists and ophthalmologists across the country. The mean age of these patients was 29 and only 4 were men. The vast majority of them were obese with an average body mass index of 40, while normal is below 25. Headache was present in 84% of patients and 68% reported transient loss of vision. Half of them had back pains and pulse-like noise in the ears (pulsatile tinnitus) was reported by 52%. Visual loss was found in 32% and it was usually loss of the peripheral vision with an enlarged blind spot in the middle.

The authors concluded that pseudotumor cerebri mostly occurs in young obese women. The importance of this report is in reminding physicians to consider this diagnosis in young obese women with headaches. The diagnosis is confirmed by performing a lumbar puncture (spinal tap), which is the only way to measure intracranial pressure. An MRI scan is also always done (before the spinal tap), to make sure that it is not a real tumor that is causing increased pressure and to visualize ventricles (fluid-filled spaces) inside the brain. These ventricles are usually small in patients with pseudotumor. Performing the lumbar puncture involves draining of the cerebrospinal fluid, which can immediately relieve the headache and also improve vision. Some patients require regular spinal taps or placement of a draining shunt (usually from one of the brain’s ventricle or spinal canal to the abdominal cavity).

However, many patients respond to medications, such as acetazolamide (Diamox) or topiramate (Topamax). Weight loss is the most effective, albeit difficult treatment. The same group of physicians reported that acetazolamide combined with weight loss was somewhat more effective than weight loss alone. Only rarely, when vision is acutely threatened, a surgical procedure to relieve pressure inside the optic nerve is performed by an ophthalmologist (the procedure is called optic nerve sheath fenestration).

In summary, increased intracranial pressure is often mistaken for chronic migraine and should be considered in every young female obese headache sufferer.

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A new treatment for pseudotumour cerebri was reported by a team of interventional neuroradiologists and neurosurgeons. Pseudotumour cerebri is a rare condition, which manifests itself by increased pressure in the head, leading to severe headaches, vision impairment and even complete loss of vision and brain damage. It affects more women than men and usually occurs without an obvious trigger, although pregnancy, obesity, and certain medications increase the risk of developing this condition. The diagnosis is made by performing a spinal tap (lumbar puncture) and measuring cerebrospinal fluid pressure. Typical MRI scan findings (narrowing of the ventricles – cerebrospinal fluid filled spaces in the brain) and finding of swollen optic nerves (papilledema)on eye exam confirm this diagnosis.

The new treatment is based on the theory that narrowing of a vein located at the back of the brain is the underlying cause, although this theory remains controversial. Narrowing of this vein is thought to reduce drainage of the cerebrospinal fluid from inside the brain, leading to build up of this fluid and increased pressure inside the skull. The usual treatments for pseudotumor include weight loss, medications that reduce pressure, such as acetazolamide (Diamox), and the surgical placement of a shunt to continuously drain spinal fluid from the brain, thus reducing the pressure.

The study, published in the online edition of the Journal of Neuro-Ophthalmology, shows that lowering pressure inside the vein alleviates the condition and improves vision. The doctors at Johns Hopkins used an advanced ultrasound scanner to thread an expandable metal stent through a vein in the groin, all the way to the transverse sinus, one of the main veins inside the skull draining fluid from the brain.

The study involved only 12 patients, but all of them had immediate relief of their headaches and 10 had lasting improvement. The researchers admitted that the efficacy of this treatment needs to be confirmed in a larger group of patients.

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