Spinal cerebrospinal fluid (CSF) leak is an under-diagnosed cause of disabling daily headache with other neurological symptoms and complications that can happen to anyone. The underlying problem is a hole or tear of the dura mater along the spine, the tough layer that normally holds the CSF in around the brain and spinal cord. A spinal CSF leak is treatable, BUT ONLY if the correct diagnosis is made. The tagline – because your dura maters℠ – highlights the TREATABLE aspect of this often very disabling neurological disorder.
Because spinal CSF leak is unfamiliar to most physicians, it is often misdiagnosed. Patients can suffer for months, years or decades with the wrong diagnosis and the wrong treatments. Medications rarely help. The most common symptom of headache is often much worse after minutes to hours of being upright, so patients may have difficulty functioning for long while upright. The degree of DISABILITY can range from mild to profound.
Our primary goal of Leak Week is to raise awareness so that more patients might be correctly diagnosed and treated. We interviewed Dr. Stephen Silberstein of Jefferson Headache Center about spinal cerebrospinal fluid (CSF) leaks and intracranial hypotension.
Can you share with us some of your thoughts about why this diagnosis is often missed or delayed?
Health care providers are not often aware of it, and they don’t know what the fundamental symptoms are. So, for example, when patients say they have a headache, doctors don’t ask what makes it better, they don’t ask what makes it worse, they don’t ask when it came on. In general, spinal CSF leak headaches are postural, typically occur after standing, and are relieved by recumbency. Health care providers just think, well, you’re lying in bed due to the headache, not because lying down prevents you from getting a headache. That’s the greatest single barrier, something as simple as knowing as what makes a headache come and go. Now, migraine gets worse when you stand up. But a spinal fluid headache usually only occurs when you’re upright. People don’t even know the usual, let alone the atypical features of spinal fluid headache.
Even neurologists don’t always recognize it.
A patient who had a spinal tap showed up in the emergency room two days later with a postural headache and it wasn’t even recognized as a post-LP headache.
So basic awareness and education around it is necessary.
That is correct.
What are some of the incorrect diagnoses you see?
Migraine, non-specific headache, cervicogenic headache, occipital neuralgia, or chronic daily headache. After imaging it may be misdiagnosed as Chiari I.
We know from patients that we speak to that this can be very disabling due to the limited ability to function while sitting or standing upright. Can you explain the poor tolerance of upright posture to our audience?
When you rise up, in the presence of a spinal fluid leak, the pressure inside the head falls more than if you don’t have a leak, and it results in the distention of these pain-sensitive structures, which causes pain.
Is the head pain from this disorder different from other headache disorders?
Yes, because it’s the only headache that I know that’s due to a greater decrease of pressure inside the head when you stand up. [for all individuals the pressure inside the head decreases when you stand up, but for a spinal CSF leak patients, this drop in pressure is larger]
Do any other kinds of headaches have a postural component?
Most headaches get worse when you move. The other condition with a headache that has a postural component is something called POTS [Postural Orthostatic Tachycardia Syndrome], so that’s a thing to rule out.
Why do most available medications used for migraine headaches fail to help patients with this disorder?
Because the fundamental issue is low-pressure headache. Low pressure inside the head. Those medications might treat some of the symptoms, but you want to treat the cause.
What kind of complications do you see?
I’ve seen a couple of cases of people presenting in coma. We’ve seen subdural hematomas.
Is that a typical complication?
These can occur. Those are the two big ones that I’ve seen.
Any less common complications that can happen with this? Are there neurological or cognitive consequences?
Oh yes, people can have cognitive problems, they can look like they have dementia. You can have any neurological symptom related to the fact that the brain is being displaced.
Many patients are cured with treatment, but for some patients, a lasting cure seems more elusive. What are the reasons for this?
For three reasons. One: you may not know where the site of the leak is. Two: it may be difficult to repair. Three, there are some people who have the symptoms of low pressure without a leak. For instance, people with a very large or capacious dural sac, that acts very much like a reservoir: they have symptoms similar to a leak, but it’s not a leak, and when they get that area reduced, they get better.
As a headache neurologist, how do you help patients that continue to suffer despite available testing and treatments?
The first thing you want to do, if they don’t respond to blood patching, is you want to make sure they don’t have POTS; then you want to take a good look and make sure they don’t have a capacious sac; and then you may want to do studies — myelography — where you actually look to see if you can identify leaks. And then sometimes you might try blood patches in different areas of the spine.
What do you see as the top research priorities that may improve outcomes for patients?
Having a good diagnostic test for leaks. Something we could do to show that patient has a leak or doesn’t have a leak. The second thing we need is a better way of localizing leaks.
Do you have any additional thoughts that you would like to share?
There are some people who have multiple leaks that can’t be repaired, and it would be really nice if we could figure out a mechanism to control pain due to this distension in the intracranial component, so even though we may not be treating the cause, we could be treating the symptoms. Also, all doctors should be asking their headache patients if their headache goes away when lying down.
Stephen D. Silberstein, MD is a Professor of Neurology and the Director of the Jefferson Headache Center at Thomas Jefferson University. He has served the American Headache Society as President, Treasurer and Board of Directors member. He has over 300 publications to his credit and lectures extensively on headache disorders. He is also a member of the Medical Advisory Board of the Spinal CSF Leak Foundation.