The secondary cough headache and Chiari type I malformation

Ross Hauser, MD

This article is for people who suffer from a complicated set of symptoms and conditions related to cervical spine instability that include a secondary diagnosis of cough headaches. A primary cough headache means that the headache is brought on by cough, nose-blowing, sneezing, uncontrollably laughing, crying, or trying to have a bowel movement during times of constipation. These same triggers can also bring on secondary cough headaches, but as may have been explained to you by your healthcare provider, something else is the primary concern as to the cause of these headaches. In this article, we will focus on one possible cause, cervical spine instability causing pinched nerves, and compressed arteries and veins that provide blood flow and circulation to the brain.

At our center, we see many patients with symptoms and conditions secondary or caused by a primary problem of Craniocervical Instability, Upper Cervical Spine Instability, and Lower Cervical Spine instability. This will include the various secondary headaches among many other challenges they face.

Also see our article: The function of cerebral blood vessels and their connection to thunderclap headaches in cervical spine instability

Almost half of cough headaches are secondary, usually to a Chiari type I malformation.

An October 2014 study in the medical journal Headache (1) shared with its medical professional audience, insights into these secondary headaches. Much of what is here is likely problems that have already been explained to you by your current and previous healthcare providers.

“Activity-related headaches can be provoked by Valsalva maneuvers (“cough headache”), prolonged exercise (“exertional headache”), and sexual excitation (“sexual headache”). These entities are a challenging diagnostic problem as (they) can be primary or secondary and the etiologies for secondary cases differ depending on the headache type. In this paper, we review the clinical clues that help us in the differential diagnosis of patients consulting due to activity-related headaches.

Cough headache is the most common in terms of consultation. Primary cough headache should be suspected in patients older than 50 years if the pain does not predominate in the occipital area if the pain lasts seconds, when there are no other symptoms/signs, and if indomethacin relieves the headache attacks.

Almost half of cough headaches are secondary, usually to a Chiari type I malformation. Secondary cough headache should be suspected in young people when pain is occipital (back of the skull) and lasts longer than one minute, especially if there are other symptoms/signs and if there is no response to indomethacin. Every patient with cough headache needs a craniocervical MRI.”

In a May 2021 study (2) of computational fluid dynamics (computer-predicted fluid flow) to predict which patients would suffer from cough-associated headaches, researchers wrote: “Cough-associated headaches are thought to be (characteristic) for Chiari malformation type I patients and have been shown to be related to the motion of cerebrospinal fluid (CSF) near the foramen magnum.” (Our note: it should be more said, “disruption of the motion” of cerebrospinal fluid is related to cough-associated headaches.)

In this study fifty-one symptomatic Chiari malformation type I patients with cerebellar tonsillar position greater than 5 mm were followed.

  • The patients were divided into two groups based on their symptoms (those WITH cough-associated headaches and those WITHOUT cough-associated headaches).
  • Using computer assistance, the researchers found integrated longitudinal impedance (a measure to demonstrate restricted fluid flow) was significantly higher in Chiari malformation type I patients with cough-associated headaches compared to non-cough-associated headaches.
  • In simple terms, where there was fluid flow disruption, the more likely cough-associated headaches would be a symptom. With this knowledge, doctors could more properly target proper treatment for these headaches as a component of Chiari malformation type I.

Now let’s take this explanation and put it in perspective of the daily challenges some of the people who have contacted us face.

In the explanation above, the secondary cough headache may be suspected in people who have craniocervical, upper cervical spine, and lower cervical spine instability. In other words, to demonstrate a complicated subject simply, people who have neck pain and cracking neck and neck movement problems, when they sneeze, cough, laugh, yell, get terrible intense headaches. They have problems that follow this type of path:

  • The patient has an MRI with herniated discs from Herniated disks C3-C7.
  • Whenever they turn their head to the left or right or move their head up and down, there is neck pain, sometimes, cracking, popping, and clicking
  • They have headaches, sometimes described as migraine-like every day for weeks and then no headaches, then the headaches return in a cycle-like fashion.
  • They will describe an intense pain at the back of the skull or across their foreheads and at the crown of the skull caused by a cough or sneeze.
  • They will talk about the worsening condition of their other symptoms during this time including difficulty swallowing, and ear fullness.

The  Chiari type I malformation

Above you read that “Almost half of the cough headaches are secondary, usually to a Chiari type I malformation.” It is likely that if you are reading this article you have had a cervical spine MRI looking for cervical spine abnormalities including Chiari type I malformation. We have a more extensive article Chiari malformation: Non-surgical alternatives to Chiari decompression surgery, that will help explain our observations and treatment recommendations. In addition, is our more recently added piece: Cerebellar tonsillar ectopia herniation and Chiari 1 malformation – The key may be the syrinx causing the symptoms.

As Chiari malformation can be a significant cause of these headaches, I will briefly discuss this challenge here:

If you have been diagnosed with Chiari malformation, you may have found a great deal of relief in finally having someone figure out what was causing all the pain. Further many found relief in Chiari decompression surgery which removed bone from the back of the skull to widen the foramen magnum (where the spinal cord passes) which alleviated the pressure and created more space for the brain. In some patients, they reported that after their surgery and a period of improved symptoms, their brain fog, pain, vision problems, and other symptoms returned. Complicated brain surgery for them, in the end, was not as helpful as they would have hoped. Some of these patients do report that their doctors had advised them that the surgery may not reverse their problems, but instead slow down or pause their worsening symptoms. But the patients had hope. Now they are looking for other options besides a second brain surgery.

When we see patients with problems of cervical spine instability, Chiari malformation, and syrinx, these patients come in with more symptoms than they can even list. Typically they will tell us of their quality of life limitations, and describe a lot of symptoms, sometimes almost an impossible amount of symptoms. When we go through a checklist of symptoms with these patients and ask about other symptoms such as heartburn, vomiting, a sensation of being bloated, nauseablood pressure swings, and vision problems, they will often say, “Yes, those too.” In many, they will report a secondary cough headache.

I had a Chiari decompression surgery that included the removal of cerebellar tonsils. My neurosurgeon wants to do more surgery which I know I will not be able to tolerate. I developed Cerebrospinal fluid leaks from the surgery. It took me a long time to recover my health from these leaks. I had “lumbar drainage,” “more suturing,” and I ultimately developed brain herniation. 

What are we seeing in this image?

  • With Chiari malformation, you’ll have a descending of the cerebellum and the brain stem into the space of the foramen magnum. As the brain stem is thicker than the spinal cord the structures within the foramen magnum are under pressure and become condensed. Any motion of the head and neck can irritate and worsen symptoms.

Cerebellar tonsil ectopia is usually described as a patient with a slight tonsillar protrusion thru the foramen magnum without the symptoms recognized of coming from Chiari malformation.

The c2 vertebra is moving and causing basilar invagination, reducing the size of the opening in the skull (the foramen magnum)

At 9:15 the video above a discussion of the loss of the natural cervical curve (Cervical Dysfunction) leading to the Chiari malformation

  • Dr. Hauser explains that currently a patient is being treated for a 9 mm Chiari malformation. How did this develop? Initially, the patient explored surgery with a specialist but the specialist informed the patient that at best, it is a 50-50 outcome.
  • The patient also has basilar invagination. In our article: Atlantoaxial instability treatment and repair without surgery we discuss the problems of a c2 vertebra moving and causing basilar invagination and possibly the development of Chiari malformation.

For some people, surgery will be necessary.

Many people have very successful surgery to help them with their conditions related to Chiari malformation. These are the people we do not usually see in our office as they have had their problems successfully treated with surgery. We see the other people.

When Chiari malformation is not the cause of secondary cough headaches there is cervical spine degenerative disease. “A sudden increase in intracranial pressure.”

For those of you reading this article, looking for information for yourself or a loved one, the secondary cough headache is usually not what you or they are seeking treatment for, secondary cough headaches are usually one of a myriad of problems that they or you are facing. In many circumstances, the secondary cough headache is only given priority when the symptoms of the headache leap to the forefront of problems because of a recent surge in the headaches or their severity.

Summary and contact us. Can we help you? How do I know if I’m a good candidate?

Secondary cough headaches are called secondary because they are a symptom and condition of something else. To treat these cough headaches you must treat the primary cause. We hope you found this article informative and it helped answer many of the questions. Just like you, we want to make sure you are a good fit for our clinic before accepting your case. While our mission is to help as many people with chronic pain as we can, sadly, we cannot accept all cases. We have a multi-step process so our team can get to know you and your case to ensure that it sounds like you are a good fit for the unique testing and treatments we offer here.

Please visit the Hauser Neck Center Patient Candidate Form

References

1 Alvarez R, Ramón C, Pascual J. Clues in the differential diagnosis of primary vs secondary cough, exercise, and sexual headaches. Headache: The Journal of Head and Face Pain. 2014 Oct;54(9):1560-2. [Google Scholar]
2 Ibrahimy A, Huang CW, Bezuidenhout AF, Allen PA, Bhadelia RA, Loth F. Association between resistance to cerebrospinal fluid flow near the foramen magnum and cough-associated headache in adult Chiari malformation type I. Journal of Biomechanical Engineering. 2021 May 1;143(5):051003. [Google Scholar]

This article was updated October 31, 2022

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