Spontaneous intracranial hypotension – lumbar epidurals and cervical spine instability

Ross Hauser, MD

A patient came into our center. She had a very long history of low back pain. One day her low back pain turned into a new and chronic headache. As her low back pain was flaring and surgery for her was not an option at this time, the patient decided that she would visit her doctor and ask about some type of pain relief. She and her doctor decided on a lumbar epidural steroid injection.

Shortly after receiving the injection, the patient noticed the new onset of headache especially when she was standing or sitting. When she came to visit us for her low back pain, we suspected she had intracranial hypotension caused by a very slow cerebrospinal fluid leak in her spine from the epidural injection.

It is common to suspect cerebrospinal fluid leak from a epidural or a lumbar puncture (spinal tap). About 1 in 200 women will suffer from headaches following childbirth because they received an epidural and it caused a leak. Anyone receiving an epidural may be subject to headaches.

But for many of the people we see, they may already have headaches and other symptoms prior to an epidural or they may not have even had an epidural but they have the symptoms of cerebrospinal fluid leak.

Mystery conditions and symptoms and no relief

We see many patients who suffer from many conditions, among them, headaches, low back and neck pain. Some of these people also suffer from challenging neurological type problems that are often misdiagnosed. Some cases are so complicated people may think that these symptoms are all in their heads or that doctors don’t know what to think so they diagnosis everything and start sending the patient to cardiologists, neurologists, gastroenterologists, rheumatologist and in some cases the psychiatrist.

The difficulty of a patient, perhaps one with low back pain or other neurologic type symptoms is in getting their doctors to understand. Look at the following study published in the Journal of neurological surgery reports in October of 2020. (1)

Brief explanatory note: As may have been explained to you in your past doctor visits, an orthostatic headache is when your headache develops when you are standing or vertical, sitting upright in a chair for instance and the headache is relieved when horizontal, you lay down.

“Spontaneous intracranial hypotension describes the clinical syndrome brought on by a cerebrospinal fluid (CSF) leak. Orthostatic headache is the key symptom, but others include nausea, vomiting, and dizziness, as well as cognitive and mood disturbance. . . “

Finding and plugging the leak

“Initial treatment is by bed rest, but when conservative measures fail, attention is focused on finding and plugging the leak, although this can be very difficult and some patients remain bedbound for months or years.”

It’s not the plugging the leak that may work, it is finding the obstruction

“Recently, (the authors of this study) have proposed an alternative approach in which obstruction to cranial venous outflow would be regarded as the driving force behind a chronic elevation of cerebrospinal fluid pressure, which eventually causes dural rupture.”

I want to stop here and explain this extraordinary observation by these researchers because it is the same observation we have made in some patients we see at our center. First the definitions:

  • A dural rupture or tear occurs in the dura mater of the brain.
  • The dura matter is the cerebrospinal fluid filled membrane that protects the brain (the brain floats in it) and spinal cord. A tear or rupture in this membrane is usually caused as a result of injury, trauma or from surgical complication.
  • A leak of the cerebrospinal fluid can cause the symptoms described above. This can be caused by lumbar puncture.
  • As noted the brain floats in the dura mater and it buoyed by the cerebrospinal fluid. When the cerebrospinal fluid level drops, the brain sinks in the skull causing greater headache. It is worse when standing because gravity is draining the brain fluids out. When you are lying down the fluid remains in the brain.

What the researchers of this study are suggesting is that is the rupture or cerebrospinal fluid (CSF) leak is being caused by a pressure buildup in the cranial venous outflow. Something is compressing the veins of brain and the brain stem. Let’s get back to the study to finish up:

Here the authors write: “Instead of focusing on the site of rupture, therefore, investigation and treatment can be directed at locating and relieving the obstructing venous lesion, allowing intracranial pressure to fall, and the dural defect to heal.”

What are we seeing in this image?

The blue balloon represents the jugular vein. In the study above the researchers are suggesting relieving the obstructing venous lesion, allowing intracranial pressure to fall, and the dural defect to heal.

Another means of obstruction

The jugular vein can be obstructed by a narrowing of the vein causing less blood to get out of the brain. When you have a balloon that is relaxed, such as the one on the left, the internal volume is constant. When you stretch the balloon as is done in the right image, look what happens. The vein is being compressed. Less blood and fluid is getting out of the brain. A sludgy back up is occurring. How is this balloon / vein being stretched? Cervical neck and spine instability. This will be explained below along with treatment methods.

Before I discuss obstructing venous lesion, I want to bring in one more study to help us understand what these doctors are saying. Problems of headaches and spontaneous intracranial hypotension can be a drainage problem and the way to resolve it is to unclog the pipe NOT patch the hole.

Pressure on the cranial nerves and blood flow

Here is a study that was published in the Emergency medicine journal : EMJ.(2) Many people, maybe yourself included, who had these sudden headaches, especially when you rose to your feet, may have sought emergency medical care at an emergency room or urgent care setting. The authors of this study then sought to explain to their fellow emergency room doctors what these headaches were all about. Here are some of the learning points they presented:

  • Orthostatic headache relieved on lying down is the most common presentation of spontaneous intracranial hypotension: a cause of severe acute headache.
  • However, cranial nerve palsies (impacting the cranial nerve VI or abducens nerve which conducts the movements of the eyes in tandem, cranial nerve VII or facial nerve – among its functions are to help sense taste and perform facial expressions, and the cranial nerve VIII or vestibulocochlear nerve which helps with understanding and transmitting the sense of sounds and balance to and from the inner ear to the brain), nausea, vomiting, coma, tinnitus, vertigo, cervical myelopathy and parkinsonism can be presenting symptoms.
  • It is three times more likely that the patient is a woman.

An explanatory note: What is explained above are the conditions that may be present when a person arrives at an emergency room.  This article is focusing on cervical spine instability as one possible causes for spontaneous intracranial hypotension even in some who had lumbar epidural. These symptoms can also be present in parkinsonism or Parkinson’s disease which we do not treat and they may be suffering from stroke.

What we are looking at in this paper are the common signs that cervical spine instability and venous obstruction may be at play. These are the symptoms of nausea, vomiting, tinnitus, vertigo, and cervical myelopathy. Symptoms that can be attributed to elevated pressure inside the brain. Also note another component of cervical spine instability, the possibility that the cranial nerves are being impinged upon. Above a connection can be suggested with visual impairment, hearing impairment, balance, vertigo dizziness, etc.

Back to the learning points of the paper: 

  • Spontaneous intracranial hypotension may involve hypovolaemia of cerebrospinal fluid rather than its reduced pressure within the central nervous system.
  • Cerebrospinal fluid and intracranial blood are constant at any time, but are in a state of constant flux. The volume of each normally goes up and down and the fluid and blood act upon each other to keep everything in balance. Especially in drainage of the brain fluids. Think of flushing a toilet. The water goes down and as soon as the dirty water is flushed, fresh water now drains in from the toilet tank to the toilet bowl. I make this analogy in my article: Brain Toilet Obstruction.
  • NOTE: The Monro-Kellie doctrine is a medical term you may have become familiar with in your medical history. A doctrine is a theory and this theory states that when the liquid sum of volumes of brain, cerebrospinal fluid (CSF) and intracerebral blood is constant. An increase in one should cause a reciprocal decrease in either one or both of the remaining two. When you flush the toilet the volume of the water going out, should be replaced by the volume of the water coming in. This may not be happening and can suspect doctors to the problem of hypovolaemia.

Now we need some more explanatory notes and we will briefly jump to another paper to help explain.

  • Hypovolaemia of cerebrospinal fluid is a decrease in the amount of fluid. Something you would expect in a leak – OR – when you have a clogged pipe. Such as when you turn the faucet on and nothing comes out.

So here there is a discussion that this may be a backed up brain “toilet” problem and not a pressure problem. Now we will jump to another study:

Why some patients diagnosed with spontaneous intracranial hypotension do not have intracranial hypotension but have sludgy cerebrospinal fluid. It is not a leak, it is a clog.

A paper published in the medical journal Neurology (3) offers this explanation in trying to figure out why some patients diagnosed with spontaneous intracranial hypotension do not have intracranial hypotension

  • Ten women (aged from 28 to 49 years) with characteristic orthostatic headache without a previous history of dural tear were examined.
  • All the patients had diffuse pachymeningeal enhancement (thickening of the dura matter – the cerebrospinal fluid had become SLUDGY and thick).
  • Since some patients with spontaneous intracranial hypotension syndrome have normal cerebrospinal fluid pressure and since a downward displacement of the brain due to a reduction of the buoyant action of cerebrospinal fluid may induce symptoms, cerebrospinal fluid hypovolemia, not intracranial hypotension, may be the cause. IN OTHER WORDS, there is not enough fluid in the brain. You flushed the toilet, but there was no water in the tank to replace it.
  • Based on the Monro-Kellie doctrine, detecting leaked cerebrospinal fluid and venous engorgement (epidural vein dilatation and pachymeningeal enhancement) is an important clue to diagnose so-called spontaneous intracranial hypotension syndrome. Dilatation of epidural veins suggests cerebrospinal fluid hypovolemia in appropriate conditions.

What are we seeing in this image?

The brain needs to drain out or flush out toxins and refill itself with fresh fluids. In the image to the left I point out that obstruction of the veins and arteries will cause a “clogged toilet.” What happens when the toilet clogs?

  • The arteries that bring fresh oxygen, nutrients, and clean fluids to the brain will be impeded.
  • The veins that help flush out toxic buildup will clog.

Ultimately this will result in an accumulation of cerebrospinal fluid in various parts of the brain including the frontal lobe. This will destroy neurons and brain tissue. This can be one explanation for the problems of memory, cognitive function and brain fog described by some patients with cervical spine instability.

To the right we see a brain without obstruction and the flow of blood in and blood out is not impeded.

Explaining: Dilatation of epidural veins suggesting cerebrospinal fluid hypovolemia in appropriate conditions such as Giant Cervical Epidural Veins

A study published in the American Journal of Neuroradiology (4) described the problem of giant cervical epidural veins after lumbar puncture in a case of Intracranial Hypotension. In fact that is the title of the paper. Here is what happened in this case study:

Introduction:

  • “A 29-year-old woman presented with dilated epidural veins and incapacitating headache after undergoing a lumbar puncture. Two months later, the results of follow-up MR imaging were normal. These findings suggest that temporary dilation of the epidural vein may occur in association with post-lumbar puncture intracranial hypotension syndrome. In these cases, it seems useful to confirm whether the patient has recently undergone a lumbar puncture. “Giant cervical epidural veins are rarely seen on imaging studies. In most cases, major enlargement has been caused by an unknown arteriovenous (artery or vein) malformation or a jugular vein thrombosis. We present a rare case of cervical epidural vein dilation.

The symptoms:

  • “A previously healthy 29-year-old woman presented with vertigo and neck pain. A neurologic examination revealed a positive right Romberg’s sign.” (Many of you may have had this test. You stand up. You close your eyes. If you lose your balance you test Romberg’s sign positive.)
  • The remaining results of the neurologic examination were normal. The results of CT of the head were normal.
  • A few hours later, a very incapacitating postural headache occurred. The results of a neurologic examination were unchanged, and MR imaging of the head showed no parenchymal abnormalities (no thickening of the dura mater).
  • MR images of the cervical spine and contrast-enhanced CT of the neck revealed dilated cervical epidural veins. No intracranial or spinal arteriovenous fistula or compressive lesion of the spine was identified. (No leaks).

The doctors discuss this case:

  • The cervical epidural venous system provides independent accessory venous drainage from the intracranial compartment.
  • The cervical epidural vein may enlarge in pathologic situations such as when compressive lesions of the spinal cord or disk disease is present. (Note: Cervical Spine Instability).
  • Enlarged epidural veins are often seen in impaired drainage of the internal jugular circulation
  • In the patient of this case study, the only abnormal MRI findings were giant lateral epidural veins.
  • The principal differential diagnosis of dilated epidural veins were:
    • disk disease, lesion of the spinal cord, and vascular malformations; however, these abnormalities are associated with other findings, including signal abnormalities, direct evidence of pial lesions, focal lesions, or internal jugular vein thrombosis.

Jugular drainage and cervical spine instability

I have a much larger article Cervical spine compression causes internal jugular vein stenosis which goes into more detail about venous compression and venous obstruction. Here is a summarized version:

The compression of the jugular vein

A January 2020 paper published in the journal CNS Neuroscience and Therapeutics (5) offered a series of findings related to compression of the jugular vein(s). These findings led to a designation of “cervical spondylotic internal jugular venous compression syndrome,” in the following patients:

Nonfocal neurological symptoms like headache, head noise, tinnitus, and visual impairment are tightly correlated to unilateral or bilateral internal jugular vein syndrome.

  • This study saw many of the things we see: That some nonfocal neurological symptoms like headache, head noise, tinnitus, and visual impairment are tightly correlated to unilateral or bilateral internal jugular vein syndrome.

How does venous obstruction (jugular vein stenosis) occur in a neck? An explanation of cervical spine and neck instability

Upper cervical instability is a primary focus of the Hauser Neck Center at Caring Medical Florida. Every day we are making discoveries in patients who have bizarre and disabling neurological symptoms that have gone undiagnosed or unresolved by their local primary care doctors or even other well-known specialty clinics.

What are we seeing in this image?

A cervical venous system that makes its way out of the brain.

The brain drains primarily via the internal jugular and vertebral venous plexus. Most venous compression syndromes that lead to such things as brain fog, memory problems, intracranial hypertension, pseudotumor cerebri, dizziness, head pressure, eye pain, and decreased or blurry vision occur at the J3 segment (upper cervical area) of the internal jugular vein. The J3 segment can get impinge by anterior subluxation of the atlas, occipital-atlanto (C0-C1), and atlantoaxial instability (C1-C2) along with altered musculoskeletal biomechanics as occurs with forward head posture. Realigning and stabilizing the atlas while destroying the cervical lordotic curve resolves most venous compression syndromes, including venous hypertension, venous ischemia, and internal jugular venous obstruction and the symptoms with them.

Over the many years of helping people with cervical spine problems, we have come across a myriad of symptoms that seemingly go beyond the orthopedic, musculoskeletal, and neuropathic pain problems commonly associated with cervical spine disorders, “herniated disc,’ and cervical radiculopathy. While many patients can understand that cervical neck instability can cause problems with pinched nerves and pain and numbness that can extend down into the hands or even into the feet, they can have a lesser understanding that their cervical spine instability also pinches on arteries and the veins in the neck and disrupts, impedes and retards blood flow into the brain and the drainage of this blood and other fluids that can cause intracranial pressure and the symptoms we described above and those we will describe below.

The main danger of brain venous congestion is that it increases intracranial pressure, this pressure is then transmitted to the brain’s arteries, which then increase blood flow to ensure adequate oxygenation of the brain. If the blood vessels cannot respond because of their obstruction in the neck, then brain ischemia can ensue.

The brain’s blood vessels may initially be able to respond via autoregulation (increases in blood vessel diameter in the brain) for a time, but if the cervical/brain venous congestion continues because of cervical dysstructure and cervical instability, the increased intracranial pressure will eventually damage the brain neurons, and ultimately, the brain tissue itself.

While the most common cause of arterial or venous obstruction in patients seen at Caring Medical is narrowing caused by cervical instability, it can also be from autonomic nervous dysfunction. Autonomic nervous dysfunction or dysautonomia can cause detrimental changes in the arterial blood flow to the brain or venous blood flow out.

One reason is that the vein is getting stretched out in the neck. How? One way is that the patient’s head is moving forward on their shoulders. When the head is in this position, the veins get pulled on and stretched out. This narrows the veins. A narrowed vein has less room for blood and fluid to flow in, this narrowing caused by cervical spine instability, which leads the head forward is characteristic of the problems faced with stenosis.

Posture-induced changes

At our center, a major factor in deciding a course of treatment is understanding the dynamics of symptom alleviation and severity by the position of the patient’s head in real-time. During the examination we have the patient rotate their heads to all the positions they can manage and monitor symptom changes. Sometimes head rotations in our examination rooms can bring upon very dramatic and immediate symptoms. We are replicating the patient’s real-life experience so we can make the best assessment of treatments.

You probably understand the importance of this more than your doctors. You know that if you turn for head to the left and look down, your ears may fill up. You know that if you look to the right and look up you may become instantly dizzy. You know that if you look down a lot your hands may tingle, you may get bloated. These are examples of how the different positions of your head can make symptoms better or worse. So shouldn’t your tests be taken while you move your head through these various positions?

Unfortunately, many doctors can only rely on static images. The patient lays on a table and has an MRI. Hold still, don’t move. Of course, the image that is taken reflects only the head and neck in that position, not the other positions the patient may be able to achieve and the positions that make their symptoms worse.

Atlantoaxial instability: C1 and C2 hypermobility causes cervical spine instability and artery, vein, and nerve compression

Atlantoaxial instability is the abnormal, excessive movement of the joint between the atlas (C1) and axis (C2). This junction is a unique junction in the cervical spine as the C1 and C2 are not shaped like cervical vertebrae. They are more flattened so as to serve as a platform to hold the head up. The bundle of ligaments that support this joint are strong bands that provide strength and stability while allowing the flexibility of head movement and to allow unimpeded access (prevention of herniation or “pinch”) of blood vessels that travel through them to the brain.

In a 2015 paper appearing in the Journal of Prolotherapy(6) our research team wrote that cervical ligament injury should be more widely viewed as the underlying pathophysiology (the cause of) atlantoaxial instability and the primary cause of cervical myelopathy (disease) including the problems I have written about in this article.

The problems of Atlantoaxial instability are not problems that sit in isolation. A patient that suffers from Atlantoaxial instability will likely be seen to suffer from many problems as they all relate to upper cervical neck ligament damage and cervical instability. As demonstrated below this includes cervical subluxation, (misalignment of the cervical vertebrae). One of the causes of Internal jugular vein stenosis is this cervical misalignment and its “pinching,” or “herniation,” not of a disc, but of the arteries and veins. This creates the situation of ischemia (damage to the blood vessels) or in the case of this article internal jugular vein ischemia.

The case for identifying loss of cervical lordosis as the cause of your symptoms

The cervical ligaments are strong bands of tissues that attach one cervical vertebra to another. In this role, the cervical ligaments become the primary stabilizers of the neck. When the cervical ligaments are healthy, your head movement is healthy, pain-free, and non-damaging. The curve of your cervical spine is in correct anatomical alignment.

When the cervical spine ligaments are weakened, they cannot hold the cervical spine in proper alignment or in its proper anatomical curve. Your head begins to move in a destructive, degenerative manner on top of your neck. This is when cervical artery and jugular vein compression can occur.

Through extensive research and patient data analysis, it became clear that in order for patients to obtain long-term cures (approximately 90% relief of symptoms) the re-establishment of some lordosis, (the natural cervical spinal curve) in their cervical spine is necessary. Once spinal stabilization is achieved and the normalization of cervical forces by restoring some lordosis, lasting relief of symptoms was highly probable.

The Horrific Progression of Neck Degeneration with Unresolved Cervical Instability

The Horrific Progression of Neck Degeneration with Unresolved Cervical Instability. Cervical instability is a progressive disorder causing a normal lordotic curve to end up as an “S” or “Snake” curve with crippling degeneration.

Treating cervical ligaments with Prolotherapy  – published research from Caring Medical

Prolotherapy is an injection technique that stimulates the repair of unstable, torn or damaged ligaments. When the cervical ligaments are unstable, they allow for excessive movement of the vertebrae, which can stress tendons, atrophy muscles, pinch on nerves, such as the vagus nerve,  and cause other symptoms associated with cervical instability including problems of digestion among others.

Treating and stabilizing the cervical ligaments can alleviate these problems by preventing excessive abnormal vertebrae movement, the development or advancing of cervical osteoarthritis, and the myriad of problematic symptoms they cause including nerve, vein, and arterial compression.

Research on cervical instability and Prolotherapy

In 2019, published in the medical journal Brain Circulation,(8) Evan Katz, a private practitioner published the findings of his office in treating the Cervical lordosis of seven patients (five females and two males, 28–58 years). “The aim of this study is to evaluate cerebral blood flow changes on brain magnetic resonance angiogram (MRA) in patients with loss of cervical lordosis before and following correction of cervical lordosis.”

These are some of the study’s learning points:

  • Loss of lordosis of the cervical spine is associated with decreased vertebral artery hemodynamics. “Vertebral arteries proceed superiorly, in the transverse foramen of each cervical vertebra and merge to form the single midline basilar artery” which continues to the circle of Willis and cerebral arteries. Based on this close anatomical relationship between the cervical spine, the vertebral arteries, and cerebral vasculature, we hypothesized that improvement in cervical hyperlordosis increases collateral cerebral artery hemodynamics and circulation. This retrospective consecutive case series evaluates brain magnetic resonance angiogram (MRA) in patients with cervical hyperlordosis before and following correction of cervical lordosis.

Note: The study cites a paper from Yuzuncu Yil University, Medical Faculty in Turkey published in the journal Medical Science Monitor. (9) In this study the research team suggests:

Because the loss of cervical lordosis leads to disrupted biomechanics, the natural lordotic curvature is considered to be an ideal posture for the cervical spine. The vertebral arteries proceed in the transverse foramen of each cervical vertebra. Considering that the vertebral arteries travel in close anatomical relationship to the cervical spine, we speculated that the loss of cervical lordosis may affect vertebral artery hemodynamics. . . the possible effects of loss of cervical lordosis on vertebral artery hemodynamics and their clinical outcomes are completely unknown. Because the vertebral arteries are the major source of blood supply to the cervical spinal cord and brain stem, the possible factors affecting these vessels warrant investigation.”

The study from Dr. Katz is one of the studies of further investigation. Following chiropractic adjustments he noted:

“This retrospective consecutive case series was performed to test the hypothesis that loss of cervical lordosis may be associated with the circle of Willis (the junction of several arteries at the base of the brain) and cerebral artery hemodynamics (More simply blood flow). The results of this case series revealed that the circle of Willis and cerebral artery parameters were significantly different between pre- and postcervical adjustments with preadjustment values showing lower values in comparison to postadjustment values. .  .Our findings demonstrate preliminary evidence that loss of cervical lordosis may play a role in the development of changes related to the circle of Willis and cerebral artery hemodynamics and decreased blood flow in the brain.”

 

References:

1 Higgins JN, Axon PR, Macfarlane R. Spontaneous Intracranial Hypotension Complicated by Subdural Effusions Treated by Surgical Relief of Cranial Venous Outflow Obstruction. Journal of Neurological Surgery Reports. 2020 Oct;81(4):e59. [Google Scholar]
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9 Bulut MD, Alpayci M, Şenköy E, Bora A, Yazmalar L, Yavuz A, Gülşen İ. Decreased vertebral artery hemodynamics in patients with loss of cervical lordosis. Medical science monitor: international medical journal of experimental and clinical research. 2016;22:495. [Google Scholar]

 

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