Day 276 - CSF Leaks and Spontaneous Intracranial Hypotension

"Spontaneous Intracranial Hypotension (SIH) is a condition where a patient gets postural headaches due to a leak of the Cerebrospinal Fluid (CSF) in the spinal membrane. What happens is that the leak causes low CSF pressure within the nervous system, and thus causes a constant string of headaches to the patient. The problem with SIH is that it is very hard to diagnose and there is not a great deal of information on why it occurs.

Cerebrospinal Fluid is formed in ventricles of the brain. It moves through the ventricles and leaves the brain at the base, underneath the cerebellum. Then, the fluid moves into the spinal cord and the nerves, and finally returns to the brain. Throughout this time, the CSF is moving through a membrane, called the dura that surrounds the brain and spinal cord. The condition of Intracranial Hypotension (IH) develops when a rupture occurs in the membrane. Thus, the CSF leaks out of the dura, causing a dislocation of the brain downward and "pressure on pain-sensitive structures." IH can develop as a result of brain surgery, spinal surgery, or any major trauma to the head. However, as in the case of SIH, the rupture can sometimes occur spontaneously. In other words, there is no known cause for the rip in the dura. Some doctors speculate that the spontaneous tear is due to the initial weakness of the dura, or a traumatic event that went undetected; however there is not a great deal of information on this subject.

The principal symptom of Spontaneous CSF leaks is headaches. The headaches only occur when the patient is upright, and gradually disappear when the patient is lying down. In most cases, the headaches gradually increase from the moment the patient wakes up in the morning. However, in other cases the headaches are quick and severe. The acuteness of the headaches varies in each case, which affects how quickly the condition is diagnosed. Some of the related symptoms of SIH are a loss of hearing, tinnitus, vertigo, stiffness of the neck, nausea, and even vomiting.

Since the primary symptom of SIH is constant and severe headaches, it is often misdiagnosed. In some cases of SIH, the condition disappears as spontaneously as it appeared. Mild cases can be cured through a general increase in fluids, especially caffeine, and lots of rest. However, more serious cases will require a procedure called the epidural blood patch. This is a procedure where autologous blood is injected into the patient's lumbar spine. The blood travels through the spinal cord, finds the rupture in the dura, and clots the rupture.

Spontaneous Intracranial Hypotension is a very rare type of a Cerebrospinal Fluid leak, but unlike other types, it does not occur due to any specific traumatic event or surgical procedure in the nervous system. Although there is not much information on the possible causes of SIH, it is suspected that an intrinsic weakness of the spinal membrane or an abnormality of the brain structure causes the rare condition."*

*http://serendip.brynmawr.edu/exchange/node/1688#1

For some reason or another, the headache has been behaving itself again as of late (except this morning when I had a bit of a relapse). I am still taking the supplements the kinesiologist advised using; they seem to be working. Along with that, I have been doing acupuncture on a regular basis for the past month or so, and I feel it’s really made a difference.

During one of the acupuncture sessions I felt my hands heat up until they felt as if they were on fire (my circulation is not great, and my hands are always freezing cold - it was such a nice feeling to suddenly have boiling hot hands). During the following session, the same thing happened but to the right half of my back, where one of my kidneys is. Very interesting considering that the Chinese acupuncturist suggested my headache may be related to my kidney. The needles were definitely doing something.

In the meantime I have been trying to figure out why my headache worsens the second I lie down, whether this be during the day or at night. I can lie down for just two minutes at any time and the headache will hurt more than when I am standing. In the mornings, I am often forced out of bed as I know that once I walk around for a few minutes the pain will slightly subside, albeit by no means in its entirety.

A number of people I have come across on the MD Junction forum have suggested looking into CSF leaks. Like many conditions, Spontaneous Intracranial Hypotension has been found to be more common in women than in men. The condition usually develops while the patient is between 40 and 60 years of age (not my case).

Like so many neurological disorders (and indeed disorders in general), little is known as to its primary causes. By what I have read, it would appear that I can rule out SIH as a possible cause for my headache - I don’t belong to the age category, I have not had any major brain injuries, and my headache actually worsens when lying down as opposed to improving, and most certainly never disappears in its entirety. Having said that, everybody is different, and you can just never say never, can you?

I just struggle to believe that after 276 days of constant headache (who would even believe it's possible to have a headache for that long?), I still don't have an answer as to what it is.

Day 260 - Intracranial Hypertension

“In the 1890s, a German physician named Heinrich Quincke coined the term “pseudotumor cerebri” to describe a neurological disorder which he believed had all the symptoms of a brain tumor, but without the presence of an actual tumor. The “false brain tumor” that Quincke identified more than a century ago is known today as idiopathic intracranial hypertension (IIH).

There are two forms of chronic intracranial hypertension: idiopathic intracranial hypertension (IIH), whereby there is no identifiable cause that triggers the raised intracranial pressure, and secondary intracranial hypertension, which, in contrast to IIH, always has an identifiable cause (such as a head trauma, an underlying disease, a reaction to a certain drug). Intracranial hypertension literally means that the pressure of cerebrospinal fluid (CSF) within the skull is too high. “Intracranial” means “within the skull.” “Hypertension” means “high fluid pressure.”

Cerebrospinal fluid is one of three major components inside the skull; the other two are the blood supply that the brain requires to function and the brain itself. Under normal circumstances, these components work together in a delicate balance. Since the skull is made of bone and cannot expand, an increase in the volume of any one component is at the expense of the other two components. For example, if the brain swells and becomes enlarged, it simultaneously compresses blood vessels, causing the sub-arachnoid space to fill with more spinal fluid. This results in an increase in intracranial pressure (i.e. cerebrospinal fluid pressure), as well as a decrease in blood flow.

CSF has several important functions. It cushions the brain within the skull, transports nutrients to brain tissue and carries waste away. CSF is produced at a site within the brain called the choroid plexus, which generates about 400-500 ml. (one pint) of the fluid each day.

Chronic IH can cause both rapid and progressive changes in vision. Vision loss and blindness due to chronic IH are usually related to optic nerve swelling (papilledema), which is caused by high CSF pressure on the nerve and its blood supply. In addition, individuals with this disorder often suffer severe pain. The most common form is a chronic headache, which is generally unresponsive to the most potent pain medication.”*

*http://www.ihrfoundation.org/intracranial/hypertension/info/C16

As I type this post, my headache is surprisingly ‘good’. About 2/10 - excellent news! Hopefully by the end of the post it won’t have increased, given its moody temper.

Lately I have been trying to find out why my headache hurts much more the second I lie down (whether during the day or at night) - so far I have only come across one other person (on a forum) whose headache also worsens when lying down. He doesn’t know why this happens either, but he strongly suggested placing corn bags on my head at night. He explained that he has found some relief from wrapping very cold corn bags around his head, having left the bag in the fridge all day long.

A number of people have told me to look at Intracranial Hypertension, as apparently those who are afflicted with this illness also have a stronger headache when lying down.

Curiously, Intracranial Hypertension was first documented in the sixteenth century by a Dutch explorer, Gerrit de Veer, who identified the toxic effects of polar bear liver on early Artic explorers. Several men in his expedition developed secondary intracranial hypertension (SIH) and nearly died after consuming polar bear liver, which contains lethal levels of Vitamin A. Excessive ingestion of vitamin A is now a recognized SIH cause (no doubt children will be delighted to hear this).

When looking at the possibility of having Idiopathic Intracranial Hypertension, one must:

a) have signs and symptoms of increased intracranial pressure, such as papilledema and headache;
b) have no localizing findings on neurological examination (Localizing findings are findings that point to injury of specific brain areas. For instance, a localizing finding could be the inability to move a certain muscle.);
c) have a normal MRI/CT scan with no evidence of venous obstructive disease;

d) have high intracranial pressure of 250mm/H2O or above on a spinal tap, with no abnormalities of cerebrospinal fluid;

e) be awake and alert;

f) have no other cause of increased intracranial pressure found.

I can safely tick all of these but two (namely d and f, as I have not had either of these examined as yet).

Given that my unmitigated headache still remains a bit of a mystery to all, it could be an idea to also look into this condition. Just the thought of returning to my GP grates me - the fact that I have to re-explain everything from scratch pretty much every time is tedious enough, but more than anything what I find frustrating is that I know that deep down many of these doctors just think that I am a hypochondriac. If only they knew.

Day 254 - Lyme Disease

“Lyme disease is an infection that derives from a tick bite. The disease has a variety of symptoms, including changes affecting the skin, heart, joints and nervous system. It is also known as borrelia or borreliosis.

Lyme disease is caused by an infection from a micro-organism (Borrelia burghdor feri), itself transmitted by a bite from the wood tick, a blood-sucking parasite which normally lives on deer.

The wood tick is found in many areas, particularly in forests where deer are common. A tick will settle anywhere on a human body, but prefers warm, moist and dark places like the crotch or armpits. When the tick has found a suitable place on the body, it sticks in its probe to draw up blood, exposing the host to the risk of infection.

Some patients with Lyme disease feel like they have caught influenza - the symptoms may be:

• drowsiness
• headaches
• mild fever
• joint and muscle pains
• swollen lymph glands
  

Interestingly, the disease only became apparent in 1975 when mothers of a group of children who lived near each other in Lyme, Connecticut, made researchers aware that their children had all been diagnosed with rheumatoid arthritis. This unusual grouping of illness that appeared "rheumatoid" eventually led researchers to the identification of the bacterial cause of the children's condition, what was then called "Lyme disease" in 1982.”*

*http://www.netdoctor.co.uk/diseases/facts/lymedisease.htm *http://www.medicinenet.com/lyme_disease/article.htm

I have managed to find a great online support community, and have across a number of people who have been suffering from New Daily Persistent Headache (NDPH). Although I haven’t officially been diagnosed with the condition, it’s refreshing to see others who understand what it is like to constantly have a headache.

One person with NDPH has been suffering for 18 years. Imagine 18 years with a headache! And me, who thought that over eight months was bad enough. Just the thought is barely conceivable.

I have been in touch with a lady who woke up with a severe headache in July last year which never went away. In March this year she was diagnosed with Lyme Disease and is now trying to spread the word as she is, finally, headache free.

She suggested I look into the condition. Having done some online research, it does not immediately appear that I have the illness as I have no other symptoms, although she told me that a couple of her friends with Lyme only have a constant headache and nothing more; another acquaintance of hers has the typical Lyme tired and constant headache but no other symptoms either.

She sent me a link to this interesting video documentary on Lyme Disease.

Whether I am afflicted with Lyme or not, I am helping her to spread the word as she only managed to find out what her headache was caused by through her constant persistence and desire to get a diagnosis. Once again, another thing to look into.