The secondary cough headache, thunderclap headache, and cervical spine instability and neck pain

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 by 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 health care 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 health care 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 which 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, and especially if there are other symptoms/signs and if there is no response to indomethacin.

Every patient with cough headache needs a craniocervical MRI.”

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 and 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) that alleviated the pressure and creates 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 that 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, described 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, nausea, blood pressure swings, and vision problems, they will often say, “yes, those too.” In many, they will report the 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.

Ross Hauser MD and Brian Hutcheson, DC discuss Chiari malformation and syrinx

Ross Hauser MD and Brian Hutcheson, DC discuss Chiari malformation or in its less invasive or milder form, cerebellar tonsil ectopia. Cerebellar tonsil ectopia is usually described as a patient with a slight tonsillar protrusion thru the foramen magnum without the symptoms recognized coming from Chiari malformation.

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

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.

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

At 9:15 a the video above a discussion of loss of 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 is 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 and 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, the 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 a priority when the symptoms of the headache leap to the forefront of problems because of a recent surge in the headaches or in their severity.

The laughing headache

In many patients, laughing can trigger a secondary cough headache. Some in the medical community also designate this situation as a “laughing headache.”

We are going to look at a 2017 study in the journal Headache (2) to look for other causes of the secondary cough headache and show similarities with and possible triggers caused by laughter. Here are the learning points of this paper:

“Laughing is a rare precipitating factor for headaches, and the pathogenesis underlying laugh-induced headache remains unclear.”

Next, the researchers presented two case histories:

Two cases of headache triggered predominantly by laughing are presented.
In the first patient, magnetic resonance imaging of the brain revealed cerebellar tonsillar herniation through the foramen magnum.
In the second patient, we did not find any evidence of intracranial disease.

Now here is the challenge:

Changes in the spatial structure in the posterior cranial fossa and cerebrospinal fluid circulation may contribute to the development of secondary laugher headaches with shared similarities with the cough headache

Here again, we have doctors speculating on possible connections because the connections in many cases are not clear and are wrapped in complexity. Let’s examine the problem of cerebrospinal fluid circulation. I will present here a summary of my more extensive article: Cervical Spine Instability, Vein blockage, fluid build up, and intracranial hypertension.

We see many patients who have a serious health challenge in having intracranial hypertension. In many of these people, intracranial hypertension was not initially thought of as a primary problem as their doctors instead tackled the symptoms that these people were facing. Symptoms included dizziness, headache, vision problems such as sensitivity to light where exaggerated pupillary hippus dilating and constricting which can cause problems with light sensitivity and the pupil fails to respond correctly to light sources. These people also faced symptoms and diagnosis of Tinnitus or ringing in the ears, neck pain, and tremors.

Once a problem of intracranial hypertension or a build-up of pressure around the brain was discovered, a myriad of tests and treatments were tried. Once obvious causes such as head injury or stroke were ruled out, initial testing may have looked for causes in blood clots, infection, and tumors. Once tests ruled those out as causes your diagnosis of intracranial hypertension, you then got an updated diagnosis of idiopathic intracranial hypertension, which means no one knows why you have intracranial hypertension.

The thunderclap headache

Many people will report to us a diagnosis or a description of a “thunderclap headache.” They will tell us how extremely painful these headaches are. How these headaches come about them suddenly and without warning (like the loud clap of thunder on a sunny day). Just like thunder, these headaches reach the highest pain intensity within the first minute and can pass very quickly. It is important that this type of headache be explored as a symptom of a more serious neurological or brain issue such as hemorrhage or aneurism. In this section we will talk about the “benign” thunderclap headache, where the headache seemingly, let’s stress the word seemingly has no obvious cause.

We are going to briefly cite a paper from Dr. Anish Bahra of The National Hospital for Neurology and Neurosurgery, University College London published in the Journal of Neurology in March 2020 (3) that explains the various pathologies of the thunderclap headache.

“Thunderclap onset headache has also been associated with a multitude of secondary pathologies. A systematic review of the literature from 2014 identified 119 causes in a total of 2345 cases reported in isolation, case series or cohorts. . . By far the largest contribution was primary (a separate) headache in 459 cases, 213 primary thunderclap headaches, followed by primary sexual headache in 182, bath-related headache in 37 and exertional headache and combinations of the aforementioned.

Three-hundred and ninety-eight cases were precipitated by cerebrovascular disorders (blood flow in the brain was disrupted by varying causes, this could included stenosis, clot, etc.) 206 from subarachnoid haemorrhage, 46 from other sources of intracranial haemorrhage, venous and arterial thromboembolism, intracranial dissection, stroke, hypertensive encephalopathy and vasculitis.

Included were 18 cases likely to be incidental findings, primarily unruptured cerebral aneurysms.

One hundred and sixty cases of sudden and severe headache were reported in association with infection, 44% affecting the central nervous system and the remainder systemic with likely central nervous system involvement or, encompassed within the terminology of a ‘viral illness’ without further elaboration.

One hundred and seventy-three patients were diagnosed with reversible vasoconstrictive ‘syndrome’ (reversing a narrowing of the artery condition).

The largest contribution to the 119 non-vascular precipitants was from pituitary apoplexy in 43 cases and 32 related to alterations in cerebrospinal fluid. Less than 20 cases were related to the peripartum state and drugs.”

Of interest for our purpose and line of work in the alterations in cerebrospinal fluid.

Blurry vision, eye pain, eye pressure, light sensitivity and other vision problems, along with symptoms above among the more troubling and disabling symptoms that are often due to cervical spine instability. An summary and explanatory notes of this video can be found here at: Blurry vision, light sensitivity, brain fog, increased ocular pressure and cervical Instability.

How does venous obstruction occur in a neck?

We are finding in outcome research that a cause of cerebrospinal fluid accumulation and cause pressure inside the head is elevated venous obstruction (vein blockage).
This can be caused by a stretching of the veins. This can be caused by the patient’s head 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.

Treatment guidelines can be found in our articles:

Eating, Swallowing, TMJ, and Secondary Cough Headaches

As stated in this and the many articles on our website, the people we see suffer from many challenges, it is not only the headaches, the brain fog, the vision problems, the ear fullness, it can be many problems.

People will often contact us with MRI demonstrated cervical spine degenerative disease and osteoarthritis in the bones of the neck. They tell us about C1-C2 problems, C3-C7 problems. In the upper cervical area typically complaints are heard about C2-C3 causing pain in the back of the neck. This pain is triggered sometimes by eating in addition to coughing and sneezing. Problems with TMJ and clicking in the jaw are also noted.

A February 2021 paper in the journal Neurological Sciences (4) found that TMJ as a cause of secondary headache was seen in over 9% of patients treated for headache.

Postural change and structure change

We have seen many patients who report a worsening of their symptoms and conditions when they turn their head one way or the other or when they stand up. Many of these patients report alleviation of their symptoms when they lay down. This includes the onset, duration, and severity of headaches that these people suffer from.

In the journal, Practical Neurology, (5) doctors from the Mayo clinic offered guidelines in diagnosing secondary headaches. Here are some of the learning points of this paper in understanding a possible cervical spine primary cause.

Headaches precipitated and aggravated by postural change raise concern for abnormal intracranial pressure, either too high or too low.
Headaches that are worse when getting up from lying down flat on one’s back. “Supine raise” can be a concern for increased pressure.
Headache that occurs within seconds of being upright and resolves quickly after lying horizontally suggests low CSF pressure.
Individuals with spontaneous intracranial hypotension might report that the headache is usually worse the second half of the day or after they have been upright for a while or that they usually feel the best in the morning before they get out of bed.
Evaluation and management of low CSF pressure can be very complicated, especially if classic radiologic findings are not present.
Cervicogenic headache can also be aggravated by the position because of axial loading of the spine, or head-turning to a particular position. Orthostatic hypotension and postural orthostatic tachycardia syndrome (POTS) can also cause postural headaches, which are usually accompanied by other symptoms of orthostatic intolerance including dizziness and presyncope (feeling faint or you feel that you are going to faint.)

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 prior to 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 really get to know you and your case to ensure that it sounds like you are a good fit for the unique testing and treatments that 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 Ran Y, Liu H, Zhang M, Dong Z, Yu S. Laugh‐Induced Headache: Clinical Features and Literature Review. Headache: The Journal of Head and Face Pain. 2017 Nov;57(10):1498-506. [Google Scholar]
3 Bahra A. Other primary headaches—thunderclap-, cough-, exertional-, and sexual headache. Journal of neurology. 2020 May;267(5):1554-66. [Google Scholar]
4 Memmedova F, Emre U, Yalın OÖ, Doğan OC. Evaluation of temporomandibular joint disorder in headache patients. Neurological Sciences. 2021 Feb 18:1-7. [Google Scholar]
Diagnosing Secondary Headaches
5 Chiang CC, VanderPluym JH, Starling AJ. Teaching Images in Headache: Is This Just a Post-Dural Puncture Headache? A Case Report. Headache. 2020 Jun;60(6):1202-1203. doi: 10.1111/head.13820. Epub 2020 May 18. PMID: 32420618. [Google Scholar]

This article was updated January 14, 2022

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