CSF Leaks and Spontaneous Intracranial Hypotension
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. (1) 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. In 1995, a study showed that only one in fifty-thousand people in Minnesota had SIH. It also showed that SIH was more common in women than in men, and that the condition usually developed while the patient was between 40 and 60 years old. (5) When first researching this condition, my two main questions were: Why does it develop and can it be cured? In order to answer these questions, one must first examine how CSF runs through the body and the specific symptoms of SIH.
Cerebrospinal Fluid is formed in ventricles of the brain. It moves through the ventricles and leaves the brain at the base, underneath the cerebellum.(2) 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."(5) IH can develop as a result of brain surgery, spinal surgery, or any major trauma to the head. (3) 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.(5)
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.(5)
Since the primary symptom of SIH is constant and severe headaches, it is often misdiagnosed. Unfortunately, misdiagnosis can increase the painful treatment for other conditions that imitate SIH (such as Chiari malformation) as well as the possibility for early treatment. In a study done by Dr. Wouter I. Schievink between 2001 and 2002, he found that 94% of patients who had SIH were initially misdiagnosed when they visited a doctor about their symptoms.(4) Some of the common treatments due to misdiagnosis included craniotomies (surgical incisions in the skull) and cerebral arteriographies (a procedure that uses an injection of dye and x-ray images to examine arteries in the brain). More than half the number of cases of SIH has been reported in the last decade.(5) Therefore, because SIH is so often misdiagnosed, it is not likely that there has been a drastic increase of the condition, but rather that more cases of it are being correctly diagnosed.
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. Success of the epidural blood patch in patients with IH can usually be determined immediately after the procedure. If it is not successful, the procedure can be repeated several times with a larger amount of blood injected (no more than 30 ml) and in many cases can lead to a permanent closing of the torn dura. (5)
However, in more severe cases of Spontaneous Intracranial Hypotension, the epidural blood patches that are injected into the lumbar spine are ineffective. At this point, an effort to locate the exact position of the rupture in the membrane can be made and then another epidural blood patch may be performed at that location.(5) This location-specific procedure is more effective than a regular epidural blood patch, and should cure the condition. However, there are a few cases where even this procedure is not successful. Further procedures to resolve the issue include an injection of fibrin glue into the specific location of the rupture and (in the most persistent cases) even surgery, both of which seem to cure the condition.(5)
In conclusion, 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. The symptoms of SIH are so small and so common, that it can often be misdiagnosed. Therefore, the condition of SIH, although thought to be very rare, is probably more likely than one might think. So how can someone prevent SIH? Is there anything a person cando to prevent the rupture of the dura? Unfortunately, there is no clear answer because very little information exists as to the primary cause of the tear. All one can really do is avoid brain surgery or any head trauma that could trigger the onset of regular IH. Hopefully, as more observations are gathered about Spontaneous Intracranial Hypotension, the causes of it will be identified and the ways to help prevent it will become more apparent.
1)Abstract of two cases by T.A. Rando and R.A. Fishman, general information on SIH
2)Discovery Health: CSF leak, general information on Cerebrospinal Fluid leaks
3)University of Maryland Medical Center, general information on Cerebrospinal Fluid leaks
4)Archives of Neurology, Abstract of a study on the Misdiagnosis of SIH
5)Medscape: Spontaneous CSF leaks, A review by Dr. Wouter I. Schievink
I have had this exact problem. It started in February 2004 and I have had it and the really bad headaches since then. Just recently, my headaches has subsided. A little background: I woke up one day in February 2004 with a excruciating headache. I went to numerous doctors and they all said it was migraine headaches. They gave me many different pain killers and nothing worked. Finally I went to a neurologist. After giving him my simptons, he said "let's check your spinal fluid." He then proceded to put a big needle in my back to check and have my spinal fluid analyzed. He got very little out of my back. He had a lab run some test, but everything came up negative. He gave me some pain killers, that did not work and then that was it. So I went to this other neurologist and he said that he had a patient two months prior that had the same condition and we should try a blood patch. Did that and that did not work, he had me try it again and that did not work. He then suggest that I check into the hospital and run a boat load of tests. He then consulted with another doctor and with all the different tests (MRI, Cat scans, etc) noticed that I had holes in the back of my neck. Both doctors decided that I would stay in the hospital and have my spinal fluid drained every hour and lay flat in the hospital bed to see if the dura would close. After a week and a half in the hospital, they did another test and some of the tear closed but I still had some still torn. (Mind you, I had many tears in the back of my neck and slightly down my spine). I was released from the hospital with the same headaches and continued to live with them until this day, though less severe. When I read your piece on spontaneous leaks, I could not believe that I am not the only one out there that has this problem! THANK YOU!!! I don't know what caused my leakes, I have had no trauma, nothing so I am at a loss. I would love to be able to talk to someone about my case and maybe sent my doctor's and hospital's records to you to put in your database to help other people. Lastly, I just read an short article about the actor George Clooney that had the same thing. I would be great to here more on this subject. I a wait your response. Sandra
I had a Sponaneous Spinal Fluid Leak in 2003 for which I finally received a blood patch procedure after suffering for 3 weeks through countless tests. I have tinnitus as a result of this condition which began 2 weeks after the initial spinal headache. I was wondering if any one else has this same problem. Its very hard to find information on this since Spontaneous Leaks are so rare (1 in 50,000). I would appreciate learning more about this.
Comment on the article on CSF leaks and hoping the person who posted a comment will read this one, or perhaps someone at Serendip could forward it to him/her. No, you are not alone with suffering a CSF leak and yes, they can be very hard to find and fix. Here's the link to a discussion community of fellow sufferers and those who suspect they have a leak and don't know. There are a lot of great resources here to help you go back to your Dr's and get more help. Two blood patches is often just the beginning of treatment, not the end, but many neurologists are not up on their reading on this rare problem and don't know what else can be done, or perhaps have never done it themselves and don't want to refer you..... http://brain.hastypastry.net/forums/forumdisplay.php?f=118
Additional comments made prior to 2007
also have intracranial hypotension from two epidural shots for a lumbar
disc problem. The back problem is not so bad anymore but I still
suffer from moderate to severe head pressure 24/7. For two years
I was bedridden with this terrible ailment, unable to stand along with
many of the cranial nerve side effects (double vision, nausea,
radicular neuropathy). Three blood patches failed to
help....slowly over time I was able to do more. Myelograms and
cisternographies have not been able to see my leak, though my spinal
pressure was measured twice to be low, as well as my brain mris showing
enhancement. Though I am functional again I still have many
restrictions including exercise (frustraing as an ex-athlete). I'm
also a licensed nutritonist and your paper touches on some interesting
points....why doesn't the dura heal? The biggest obstacle with
this conditon is the difficulty with diagnosis because most doctors
believe the dura must heal in x amount of time and. So to accept that this doesn't occur with everyone is one problem and
then why it isn't healing is another. I wonder if certain
fundamental factors are missing relating to connective tissue.
Not necessarily one of the conventional connective tissue disorders but
something missing or interfering with attaining adequate amino acids to
fully optimize connective tissue sythesis/regeneration. At any
rate, this can be an unbelievably debilitating condition....check out
Mass General Hospital's neurology department's...brain forum and look
under csf leak diagnosis and you will see it is more common than
perhaps once thought. Plus these headaches are so not like migraine,
although it is not uncommon for migraines to develop on top of
them. These headaches/pressure are all about physics...an intense
pulling sensation..like G-forces pulling your head down. I'm praying
for more research....maybe better imaging to find smaller leaks.
Many thanks for your paper ... Cindy, 4 February 2006
My husband started w/ severe fatigue approx 4. months ago. The fatigue lessened,but he began having chronic headaches.He has had a headache now for about 3 months. Spinal tap results came back as low pressure and high protein. He seems to think it is a low pressure headache. This is all new to us. We have never heard of such a thing. Apparently there are more of you out there who suffer from this. We would love to hear from anyone experiencing the same thing. He did have a blood patch today. Waiting to see if it helps. Thanks for that great article. Very good and informitive ... Lisa, 15 August 2006
REGARDING SPONTANEOUS INTRACRANIAL HYPOTENSION I am a 36yo female I have had this condition also on july 19 2006 I went to bed with a bad headache and woke up the next morning with sore shoulders and neck when I tried to get up I suffered the most excruciating pain in my neck and head it was so bad I wanted to vomit . When I visited the local hospital the doctor gave me panadol and sent me home without doing any tests. After 3 days of getting worse I went back to my doctor by this time I could barely walk because the pain was so severe the doctor sent me over for a cat scan which they picked up immediatley as some kind of fluid on my brain . After that I was flown by Royal Flying Doctor to Perth . While there I had 2 MRI one on my brain and the other on my spine after the spinal one they picked up that I had a leak in my spine. I just want to tell any one out there who has had or does have this condition that I really feel for you because it's such a traumatic illness and no one has any idea how painful it is also anyone who is suffering pain in the head so bad please demand you have a cat scan ... Deanne, 26 September 2006
I am just three weeks out of having a disctectomy done. The week prior to that I received an epideral to try amd fix the blown disc that I had. Is there anyway to minimize the pan of the headaches? I am being admitted into the hospital again on Monday to now correct the spinal fluid leakage problem. Does anyone have any suggestios concerning this matter or that can supply me with any kind of information? ... Heather, 22 November 2006
I would like to talk to others who are suffering from spinal fluid leaks. Can you help put me in touch? Or, can I talk to you? ... Brad Hennenfent, 22 January 2007
I read the article on Spontaneous Intracranial Hypotension and felt compelled to share my experience with this condition, which is still unresolved but hopefully soon to be treated. I should preface this by saying that I have never had injury or trauma to the head or spine. I developed a headache a little over two weeks ago that was very migraine-like except that none of the pain relievers I tried got rid of it entirely, it originates at the base of the skull and spreads forward, and it's relieved within five minutes of lying down. Also, after five days of the headache, I began to have a feeling of congestion in my ears accompanied by a soft rushing sound, followed by mild hearing loss. The first doctor I saw in Urgent Care gave me some migraine medicine; the second ordered a CT scan; the third ordered a brain MRI and had me see a neurologist; the neurologist saw a report of the MRI that indicated enlarged ventricles, thought I might have hydrocephalus and referred me to a neurosurgeon for another opinion (the neurologist also prescribed Diamox, a diuretic - yikes!); the neurosurgeon looked at my MRI and thought I had a Chiari malformation, but wanted a second opinion so he had me see another neurosurgeon; the second neurosurgeon made the diagnosis of intracranial hypotension and ordered a lumbar MRI to see if they could detect a CSF leak. My dilated ventricles caused a lot of confusion because that's not usually consistent with SIH, but it was eventually concluded that I've probably had hydrocephalus all my life but have never had symptoms, and that the hydrocephalus is not what's causing my current problems. The next step was supposed to be a tracer study to try to identify a leak and its location. Well, my concerned husband got tired of all the shuffling and waiting around among our local doctors, so he took me up to Stanford (about an hour's drive) and checked me in to the ER; as you can imagine, we got much farther much faster by doing that. Stanford neurologists agreed with the diagnosis and have arranged for a blood patch next week -- no messing around with a tracer study. The blood patch will be injected and then my head will be lowered so that the blood coats the entire inside of the dura along the spine, with the assumption that if there's a leak somewhere along there, it will be plugged up. Let's hope it works!!! ... Larisa, 25 March 2007
I AM 45 YEARS OLD AND I TO WIND UP WITH MIGRANES I REMEEMBER JUST THIS MONTH OF THIS YR. MY HEADACHES WERE SO BAD THAT I LAYED IN A DARK COOL ROOM AND JUST SLEPT I WAS FEELING NAUSEATED EVERTIME I HAD TO USE THE RESTROOM THE SMELL OF FOOD EVEN MADE ME SICK. RECENTLY I HAD A MRI AND THE DOCTOR TOLD ME THAT THE FLUIDS IN MY BRAIN WERE LOW. BUT I ALSO WENT THROUGH 3 BLOWS TO THE HEAD FROM MY EX-HUSBAND NOW I HAVE A NEW LIFE A WONDERFULL HUSBAND AND I ALSO TO WENT TO THE COMPUTER TO FIND NUMEROUS ANSWERS BUT NOTHING SEEM COMPLETED I WILL BE SEEING A NEUROLOGIST HERE THIS AUGUST BUT TILL THAN OUR HOUSE IS DARK AND COOL AND TAKE MEDICATION FOR HEADACHES HOW SEVERE CAN THIS BE AND WHAT HAVE THEY FOUND IN TODAY TECHNOLOGY? I HOPE SOMEONE OUT THERE CAN UNDERSTAND WHAT I AM GOING THROUGH AND WHAT RELIEVED DID THEY RECIEVE DURING ALL THIS PLEASE IF ANY ONE KNOWS PLEASE SEND WHAT INFORMATION YOU MAY HAVE CAUSE I WOULD LIKE TO HAVE MY LIFE BACK TO NORMAL ... Susie, 26 June 2007
One morning in Oct. 06 I woke up with severe headaches and after 6 weeks of working up the ladder at my HMO, a neurologist finally diagnosed me with CSF leak (SIH). Not content to being mollified by being put on Prozac, I did some cursory research on the net and found out that others had what I had and found that some had been referred to Dr. Schievink at Cedars Sinai in Los Angeles for treatment. These can include blood patches, glue patches and surgery. His staff is very familiar with the disease and he is "the" expert.
My HMO refused to send me to him (right down the street in my case) the cost you know, until I developed large subdural hematomas. At that point they had no choice but to refer me because it was totally out of their realm to effectively treat the underlying cause, the CSF leak, or the subdurals.
A long story short--after 2 blood patches, 1 glue patch and 2 spinal surgeries(laminectomies)at T9-11 to remove benign Tarlov cysts, to repair 2 leaks, I can now function quite nicely. I have a 20 lb. lifting limit, can't hike up steep hills, and can't push or pull on heavy objects. These modified "val salva" maneuvers, I have learned, will cause undectable (on a CT myleogram) leaks. They come and go because I will still get positional headaches at times. At my last reading my CSF pressure is ZERO.(0) cm.
I'm very fortunate at 60 years old to be retired and thank God every day for the wonderful treatment I have recieved. I think mine was a severe case, I use to do a lot of physical work and ran for 25 yrs and maybe those were contributing factors ... Harvey Geiss, 16 July 2007
I had a spontaneous CSF leak in Nov 2004. After weeks of being told I had migrains, I was sent to a neurologist. He immediatly knew it was a CSF leak and put me on bedrest. After 6 weeks on bed rest, I still had bad headaches everytime I stood up. I then had a test to show where the hole was and they could not find it. I went to a different hospital and asked if we could do the test standing up because that was when I had my pain, and they said their machines can't go up that far. So I asked if they could do it with me on my knees, and they said yes. With in minutes they saw a large tear at C7. After two blood patches my major headaches are gone. From day one, to the last blood patch it was 4 months of bed rest! Yuck! Now I am living my life wondering if this will happen again, and taking Neurontin for these annoying daily headaches. Scary stuff! ... Elizabeth Kilik, 11 October 2007
Reading article and reader comments on intracranial hypotension. I've suffered over past 15 years from initially intracranial hypotension and flipped at some point to intracranial hypertension (or pseudotumor cerebri)! Living in Cleveland, OH diagnosis ranged from CSF leak to Chiari Malformation to POTS to migraine headaches. I ended up at Mayo Clinic where intracranial hypertension was diagnosed JUly 2007. Mayo has some of the leading experts on this area of neurology in the country. Medication was tried for a short while and in August 2007 a VP Shunt was placed to releive the hypertension. Almost 9 weeks after surgery some headache symptoms arise mid-day when I'm upright- started irregularly a few weeks ago now more of a daily occurance, including one episode of nausea and vomiting. I'm following up back at Mayo Clinic in 1 week. Looking for anyone with similar experiences, resolution of symptoms, and return to normal family and work life ... Robert Schneider, 22 October 2007