Headache & Pain
Headache associated with intracranial neoplasms
Sep. 30, 2024
Worddefinition
At vero eos et accusamus et iusto odio dignissimos ducimus qui blanditiis praesentium voluptatum deleniti atque corrupti quos dolores et quas.
Primary cough headache …
- Updated 08.12.2023
- Released 02.14.2002
- Expires For CME 08.12.2026
Primary cough headache
Introduction
Overview
The authors discuss the clinical manifestations, etiology, differential diagnosis, diagnostic evaluation, and management of primary cough headache. In this article, studies giving insight into the cause of primary cough headache in patients with or without Chiari I malformations are reviewed. Morphometric MRI studies that have shed new light on the etiology of primary cough headache are summarized, and evidence that CSF leaks may present as cough headache without an orthostatic component is presented. Although indomethacin is typically used to treat primary cough headache, the authors discuss other treatment options, including topiramate.
Key points
|
• Primary cough headache is typically bilateral, of sudden onset, lasts less than 1 minute, and is precipitated rather than aggravated by coughing. | |
|
• Primary cough headache can be triggered by other sudden Valsalva maneuvers, but it is not typically triggered by sustained physical exercise. | |
|
• Every patient presenting with cough headache should have an MRI of the brain to rule out a posterior fossa lesion. The MRI should be done with gadolinium looking for pachymeningeal enhancement because headache secondary to spontaneous leak can present as cough headache alone, with no orthostatic component. | |
|
• Indomethacin is the de facto preventive drug of choice for primary cough headache. |
Historical note and terminology
Headache aggravated or brought on by cough has long been known to be a symptom of intracranial disease (12). This chapter will focus on primary cough headache, which by definition is unassociated with an intracranial disorder (19).
Table 1. International Headache Society Diagnostic Criteria for Primary Cough Headache
|
(A) At least two headache episodes fulfilling criteria B-D |
(1) Headache arises moments after the cough or other stimulus.
(2) Headache reaches its peak almost immediately, and then subsides over several seconds to a few minutes (although some patients experience mild to moderate headache for 2 hours).
(3) The syndrome of cough headache is symptomatic in about 40% of cases and the large majority of these represent Chiari type I malformations. Other reported causes of symptomatic cough headache include CSF hypotension, carotid or vertebrobasilar disease, cerebral aneurysms, middle cranial fossa or posterior fossa tumors, midbrain cyst, basilar impression, platybasia, subdural hematoma, and reversible cerebral vasoconstriction syndrome. Diagnostic neuroimaging plays an important role in the search for possible intracranial lesions or abnormalities. Because subtentorial tumors account for more than 50% of intracranial space-occupying lesions in children, cough headache in pediatric patients should be considered symptomatic until proved otherwise.
Table 2. International Headache Society Diagnostic Criteria for Probable Primary Cough Headache
|
(A) Either of the following: (1) a single headache episode fulfilling criteria B-D; (2) at least two headache episodes fulfilling criterion B and either of criteria C and D |
Primary cough headache can be triggered by other sudden Valsalva maneuvers but is typically not triggered by sustained physical exercise (37). This is the opposite of the typical circumstance in primary exercise headache.
In 1932, Tinel described four patients with headache brought on by coughing, nose-blowing, breath-holding, and bending the head forward (25). Before Symonds and Rooke’s reports, cough headaches and exertional headaches were always considered ominous symptoms, and there was no clear recognition that benign or primary types of these headaches existed. In 1968, Rooke noted that “in every patient with this complaint, an intracranial lesion of potentially serious nature, such as brain tumor, aneurysm, or vascular anomaly, has been suspected; and even when no such lesion could be identified, an uneasy uncertainty usually has remained" (47).
The landmark paper entitled “Cough Headache” by Sir Charles Symonds brought attention to this disorder (53). Symonds clearly described cases of both secondary and primary cough headache. He presented patients with headache provoked by coughing, and noted that sneezing, straining at stool, laughing, or stooping could also provoke the headache. He did not describe headache precipitated by physical exercise. He outlined the clinical course of primary cough headache and suggested a pathophysiological mechanism for the disorder.
In 1968, Rooke reviewed 93 patients with primary exertional headache (47). He did not separate cough headache from headaches caused by running. However, his data underscored Symonds’ concept that cough headache could be benign.
Clinical manifestations
Presentation and course
Primary cough headache is typically bilateral, has a sudden onset, and, after peaking, almost immediately dissipates over seconds to a few minutes, with the ICHD-3 permitting residual pain for up to 2 hours (19). It is precipitated rather than aggravated by coughing and can be prevented by avoiding coughing. The International Headache Society criteria specify that structural lesions must be ruled out by neuroimaging before the diagnosis of primary cough headache can be made. It is not associated with nausea, vomiting, conjunctival injection, lacrimation, nasal congestion, or rhinorrhea (12).
Because of the historical importance of Symonds’ cases, they will be reviewed in detail (53). Eighteen of the 21 cases of primary cough headache reported were men. The age range was 37 to 77 years, with the average age 55 years. Symonds’ patients generally complained of severe bilateral head pain that lasted 2 minutes to 10 minutes. Two patients had severe pain for a few minutes followed by a dull ache that lasted 1 hour to 2 hours. The quality of the pain was usually described as bursting. Most patients had pain at the vertex spreading to both frontal regions. Other maneuvers besides cough could trigger the pain, but physical exercise was not mentioned as a precipitant. Two patients could trigger headache by quickly rotating the head. Two patients could cough without pain while lying down. Two patients noted that when they awoke in the morning they could cough without pain, but that the liability appeared after they assumed the vertical posture. One patient noted a decrease in pain severity if he coughed with his neck extended. Two patients had relief of cough headaches after lumbar puncture; one had relief for a few weeks and then had complete relief after air encephalography, and the second had complete relief 6 months after lumbar puncture. Two patients recovered after an infected tooth was extracted. Nine patients had eventual recovery, six had significant spontaneous improvement over a period of 18 months to 12 years, and the remaining six either had no change or died of an unrelated disease. Five patients had a past history of migraine.
Rooke considered cough headache to be a form of exertional headache and reviewed the clinical features of 93 patients with primary exertional headache (47). The male to female ratio was 4:1, and exertional headache was twice as common in those over 40 years as compared with those aged 10 to 40 years. Rooke outlined the generally favorable course of primary exertional headache. Eleven patients had complete headache relief by 1 year, 30 had complete relief of headache within 5 years, and 73 either improved or were headache-free after 10 years. Seven of 31 patients noted a respiratory infection before their exertional headaches began, and four patients had unequivocal relief after dental extractions. One patient had complete headache relief after pneumoencephalography.
Raskin has reviewed the clinical features of primary cough headache (41; 43). Many patients note headache onset during lower respiratory tract infections accompanied by cough or during vigorous weightlifting programs. The headache arises moments after the cough, reaches its peak almost immediately, and then subsides over several seconds to a few minutes. Sometimes the pain remains at the peak for several seconds before decreasing. Most patients are pain-free between attacks, but some have a dull headache following the triggered attack that may persist for hours. Raskin warns that these patients often complain of continuous headaches, thus, emphasizing the need to directly ask about cough as a trigger. Nausea and other migrainous features are uncommon, and although the headache is usually bilateral, it is occasionally unilateral.
Pascual prospectively studied 28 patients with primary cough headache (37). All patients presenting with cough headache were evaluated with MRI. The duration of pain in primary cough headache was usually seconds, with a mean duration of the symptomatic phase being 11 months. Primary cough headache was located in the occipital region only 11% of the time.
In a 2009 study of 74 patients with primary cough headache, 66 patients had headaches for less than 30 minutes, but eight patients had headaches that lasted longer than 30 minutes (08). Sixty-eight percent of patients with primary cough headache had bilateral headache, and 73% had more than one headache trigger (straining at stool, bending down, sneezing, Valsalva maneuver, heavy lifting, “exertion,” sexual activity, or laughing).
A study published in 2021 looked at 679 patients with cough in a respiratory clinic in China; the study showed a prevalence of cough headache of 18% (60). Risk factors for developing primary cough headache included increased severity of the patient’s cough and age of 31 to 50.
Over the years, some rare clinical characteristics have been reported. Bruyn described a patient who could trigger headache by coughing or yawning (06). A case of cough headache presenting as unilateral toothache completely responsive to indomethacin has been reported (34). This patient’s pain was in the right maxillary region and radiated to the ipsilateral temple, ear, and occiput. Unilateral cough headache with circannual periodicity has been reported (39). This patient’s cough headaches were often induced by exertion as well. Unilateral primary cough headache coexisting with chronic paroxysmal hemicrania has been reported (28; Pavão and Viana 2017).
Bougea and colleagues reported three cases from Greece with the coexistence of primary cough, sexual, and exercise headaches. All the patients responded significantly to indomethacin (04).
Ran and associates described two patients with laugh-induced headache (40). They reported that laugh-induced headache could be categorized as primary laugh-induced headache and secondary laugh-induced headache, which can be a subset of either primary cough headache or primary exercise headache.
Ferrante reported a 71-year-old female with primary Valsalva maneuver headache without primary cough headache. She had headache associated with bending but did not have primary cough headache (16).
As cough is a common symptom of COVID-19, a few studies have looked into the incidence of cough headache as it relates to COVID-19 infection. The studies have found that a cough headache-like pattern may occur in 2% to 16% of patients (48). Further studies must be conducted to determine the rate of cough headache after COVID-19 infection.
Prognosis and complications
Of the 21 patients reported by Symonds, nine had eventual recovery, six had significant spontaneous improvement over a period of 18 months to 12 years, and the remaining six either had the disorder continue without change or died of an unrelated disease (53). The disorder persisted for 1 to 2 months in the 28 patients reviewed by Pascual and colleagues (37). Chen and colleagues noted that at a mean follow-up of 51.4 months, 84% of patients with primary cough headache completely remitted (08).
In a retrospective report by Killeen and colleagues of 68 patients with nonoperative Chiari I malformations, 40% had cough headache (secondary cough headache) (22). According to the study, the presence of cough headaches was a significant negative predictor of concomitant symptom improvement. The authors implied that patients with cough headaches should be considered for surgical intervention rather than being managed conservatively (22).
Clinical vignette
A 60-year-old man presented with a 5-year history of head pain triggered by coughing and other Valsalva maneuvers. The pain would occur almost immediately after the cough, and would last approximately 15 seconds. He could ride a stationary bike for 30 minutes without the pain occurring. He had no other headache between cough-induced paroxysms of pain. The pain was severe, sharp, and bifrontal in location. He denied nausea, vomiting, photophobia, phonophobia, aura symptoms, ptosis, conjunctival injection, lacrimation, nasal congestion, rhinorrhea, or facial sweating with the attacks of pain. There was no history suggestive of transient ischemic attacks. He was on no medications and had no significant past medical history. He did not smoke or drink alcohol, and there was no family history of headache. Neurologic examination as well as MRI and MRA of the brain were normal. He was placed on indomethacin 25 mg three times a day, which stopped his cough-induced headaches. After 2 years he was able to stop indomethacin and was no longer predisposed to cough headache.
Biological basis
Etiology and pathogenesis
The etiology of cough headache is not completely understood. It seems intuitive that it is associated with increased intracranial pressure, as coughing increases intracranial pressure. What causes the pain is not clear, however, as elucidated by Sir Charles Symonds in 1956:
|
In the normal person, coughing, however violent, does not cause headache, and perhaps the first question to be answered is why it does not. That it causes an increase in intracranial pressure from venous dilatation we know from observing the response when measuring the cerebrospinal fluid pressure. The intracranial venous dilatation is presumably obtained at the expense of cerebral capillaries and expulsion from the cranial cavity of such a proportion of the cerebrospinal fluid as can be accommodated by the spinal subarachnoid space. Under these conditions it has been argued . . . that there should be a displacement of the brain towards the foramen magnum. This would involve traction upon the structures, vascular and dural, anchoring the brain to the skull, which are known to be sensitive to this form of stimulation. Yet no headache results, presumably because the degree and direction of traction are inadequate to cause it (53). |
Symonds later hypothesized that in primary cough headache, “the pain is due to stretching of a pain-sensitive structure within the posterior fossa, which may . . . be due to an adhesive arachnoiditis" (53).
Williams measured CSF pressures from the lumbar region and the cisterna magna in 16 patients while coughing in the sitting position (57). All patients had “disease in the cervical region” requiring myelography. None of the cases had a complete blockage of the spinal subarachnoid space. During a cough, there was a phase during which the lumbar pressure exceeded the cisternal, followed by a phase in which the cisternal pressure exceeded the lumbar. Thus, on coughing, the intrathoracic and intra-abdominal pressure was felt to be transmitted through the valveless veins around the vertebrae to the epidural veins, which then distended with blood (58). Once distended, these veins compressed the spinal dura, causing a pressure wave that passed into the head and then rapidly downwards again (58). Williams commented that the upward passage of fluid was relatively easy but that the downward rebound from the head towards the spine might cause tissue to jam in the foramen magnum. This would create a pressure difference between the head and spine, which he termed the “craniospinal pressure dissociation" (57). He postulated that in Chiari I malformations, the ebb and flow of fluid through the foramen magnum could progressively impact the tonsils, leading to pain. Williams subsequently investigated the etiology of cough headache in two patients with Chiari I malformations (58). He verified that these patients had a craniospinal pressure dissociation preoperatively. Decompression of the cerebellar tonsils relieved the cough headache and eliminated the craniospinal pressure dissociation. Nightingale and Williams later described four other patients who were successfully treated with surgical decompression (36). Sansur and colleagues have commented that Williams also detected craniospinal dissociation exacerbated by cough in patients harboring Chiari I malformations without headache, and noted that he did not compare patients with and without headache associated with coughing to see if they differed in degree of craniospinal pressure dissociation (49). Thus, they feel that Williams' theory does not explain why some patients with Chiari I malformations have cough headache whereas others do not, and they argue that craniospinal pressure dissociation is an unlikely explanation for the cause of headache associated with cough in these patients (49). In an attempt to clarify the etiology of cough headache in patients with Chiari I malformations, Sansur and colleagues studied 30 patients with Chiari I malformations and 15 healthy adults. Of the 30 patients with Chiari I malformations, 11 had headache aggravated by cough. Lumbar subarachnoid pressure was measured at baseline and during cough in all patients. Those with Chiari malformations had measurements performed before and after suboccipital decompression. Peak intrathecal pressures during cough and at baseline were elevated in patients with headache associated with cough (and Chiari I malformations) compared with either patients without headache (and Chiari I malformations) or healthy volunteers. After surgery, intrathecal pressures during cough were significantly lower than preoperative values, and headache aggravated by cough was resolved partially or completely. Thus, the authors hypothesized that headache linked to coughing in patients with Chiari I malformations was associated with a sudden increase in intrathecal pressure caused by obstruction to the free flow of CSF in the subarachnoid space (49). Bhadelia and colleagues analyzed CSF flow with cine phase-contrasted MRI in patients with Chiari I malformations (02). This imaging modality showed differences in CSF flow patterns between patients with Chiari I malformations with and without cough headache. In the region of interest just below the foramen magnum, those with cough headache had much shorter CSF systole and longer CSF diastole compared to those without it. In addition, CSF diastolic duration of 0.75 or more of the cardiac cycle was moderately sensitive and specific in predicting the presence of cough headache in patients with Chiari I malformations.
Patients with primary cough headache do not have tonsillar herniation, so Raskin has hypothesized that in these circumstances, the pain is due to heightened sensitivity of yet unidentified receptors (41). Raskin successfully treated four cough headache patients with repetitive intravenous dihydroergotamine and offered that unstable serotonergic neurotransmission might be important in the etiology of cough headache (41). One case of cough headache occurred after periaqueductal gray electrode implantation (44). The phenotypic cough headache developed immediately after periaqueductal gray implantation and was relieved by indomethacin. The patient used indomethacin for 4 months and then remained headache-free after stopping the medication. She remained headache-free at 5-year follow-up. After noting that primary cough headache could be provoked by a sudden increase in intracranial pressure and could remit after the sudden decrement in intracranial pressure that attended lumbar puncture, Raskin later hypothesized that the nature of the receptors sensitive to intracranial pressure alterations was probably the key to understanding primary cough headache (43).
Wolff thought that cough headache often occurred around the time of a systemic infection. He believed that the systemic illness altered the vascular tone in the head and found that most patients with cough headache had a prior history of migraine (59).
A case of a man with a large goiter who could trigger headache by raising his arms has been reported (24). This maneuver would decrease venous return from the head, increasing intracranial pressure. Presumably the sudden increase in venous pressure was enough to cause headache.
Wang and colleagues postulated that primary cough headache was due to CSF hypervolemia, leading to an increased craniospinal pressure dissociation during coughing, thus, explaining the response to acetazolamide, indomethacin, and lumbar puncture (56).
The same group reported the association of primary cough headache with morphometric MRI evidence of posterior cranial fossa overcrowding (09). Based on the midline sagittal MRI images, several parameters indicating posterior fossa crowdedness were measured in 18 patients with primary cough headache as well as in 18 age- and sex-matched controls. No patients had MRI evidence of a Chiari I malformation. The study showed that patients with primary cough headache had a comparable hindbrain tissue area but a smaller posterior cranial fossa area as compared with control subjects. They also observed shorter clivus lengths, lower tonsillar tips, and narrower prepontine and premedullary cisterns in patients with primary cough headache. The authors suggested that posterior fossa overcrowding might cause a relative obstructive CSF pathway around the craniocervical region, contributing to a higher intracranial pressure during cough (09).
Donnet and colleagues found stenosis in the transverse sinuses, jugular veins, or both in five of seven patients with primary cough headache, but the significance of these findings is unclear (10).
Liu and colleagues reported a case of recurrent cough headaches precipitated by Valsalva maneuver with a transient increase of intracranial pressure, which was diagnosed by measuring left sigmoid sinus pressure. Along with increased sigmoid sinus pressure, the patient had bilateral internal jugular vein valve incompetence, which was also diagnosed by Doppler ultrasonography during Valsalva maneuver. The authors proposed that cough headache might be associated with a transient increase of intracranial pressure induced by internal jugular vein valve incompetence (27).
As clinical features may not distinguish primary from secondary cough headache, Lane and Davies hypothesized that the modified Valsalva test may differentiate the two groups of etiologies (26). They took 16 consecutive patients with cough headache and used the modified Valsalva test in which a 10-second forced exhalation was applied to a connecting tube of a sphygmomanometer set to a pressure of 60 mm HG. Ten of the 11 patients with a positive modified Valsalva test had posterior fossa pathologies on MRI (eight Chiari I malformations, two meningiomas). They proposed that primary cough headache may involve transient orbital venous plexus congestion in the presence of jugular venous incompetence as well as a reduced threshold for trigeminal sensory activation. Tinel and colleagues and Gupta and colleagues initially postulated this mechanism of venous involvement.
The etiology of benign cough headache was best summed up by Symonds himself, who in 1970 wrote “as far as I am aware its origin remains a mystery" (54). Perhaps a patient with primary cough headache has a lowered threshold for the development of pain associated with the increased intracranial pressure normally caused by coughing.
Epidemiology
In a population-based study, primary cough headache had a lifetime prevalence of around 1% (45). Studies showed about 0.4% to 1% of patients with headache consulted a medical clinic because of cough headache, and primary cough headache was more prevalent in men (37; 08).
Prevention
Any chest disease that may be causing the cough should be identified and treated (11). If an angiotensin-converting enzyme inhibitor is causing cough, a switch should be made to a different medication, if possible (37).
Differential diagnosis
Before a diagnosis of primary cough headache can be made, intracranial masses and, specifically, posterior fossa lesions must be ruled out.
Brain and cervical spine in a patient presenting with cough headache and bilateral upper extremity sensory symptoms (MRI)
(A) Sagittal T1 image showing descent of the cerebellar tonsils into the foramen magnum and associated cervical syrinx. (B) Axial T2 image showing cross-section of the cervical syrinx. (C) Sagittal T1 image showing extent of th...
The percentage of patients with secondary cough headache differs markedly between the 2009 Chen and 2008 Pascual studies (11% vs. 59%). Chen and colleagues hypothesize that this difference may be related to a lower prevalence of Chiari I malformation in the Asian population, realizing that data on said prevalence are absent (37; 08). Pascual and colleagues found clinical differences between patients with primary and secondary cough headache (37). Symptomatic or secondary cough headache started earlier in life (mean 44 vs. 60 years old), had a longer attack duration, showed an occipital location in seven out of 10 cases, and did not respond to indomethacin. In the Pascual study, 82.5% of patients with secondary cough headache had posterior fossa semiology-like dizziness, unsteadiness, face/upper limb numbness, vertigo, syncope, or “other symptoms,” but it is not clear how many had abnormal neurologic examinations. Seven patients with secondary cough headache had no other accompanying symptoms or signs, and six patients with Chiari I malformations had cough headache without other subjective complaints or abnormal findings on examination. A large study of 83 patients with primary and secondary cough headache revealed that clinical features, neurologic examinations, and drug response could not safely differentiate primary from secondary cough headache (08). In the secondary cough headache group, three of nine patients had gait ataxia with or without dysmetria, whereas all of the primary cough headache sufferers had normal neurologic examinations (08). Indomethacin response should not be used to differentiate primary from secondary cough headache, as a patient with a Chiari I malformation-associated cough headache that responded completely to indomethacin has been reported (13). Chen and colleagues also reported a patient with secondary cough headache who responded completely to indomethacin (08).
Table 3. Some Secondary Causes of Cough Headache
|
• Chiari I malformation |
Unilateral cough headache has been reported with carotid stenosis (05; 46). In the case reported by Britton and Guiloff, the patient initially had right-sided cough-induced headaches without focal neurologic deficits. Two years later, he developed episodic sensory symptoms in his left hand. Later that same year he developed left hand weakness and numbness of the left face. He was admitted to a hospital and found to have pyramidal weakness in the left hand. CT of the head was normal, and a myelogram showed no abnormality of the cord or foramen magnum. Twenty-four hours later, he developed a continuous right-sided headache, “similar to the cough headache," and the next day, he developed a severe left hemiparesis. CT showed a large right anterior cerebral artery and middle cerebral artery infarct. Cerebral angiogram showed stenosis of the right internal carotid artery close to its origin, and the possibility of dissection was raised. Echocardiography was normal. Angiogram 5 weeks later revealed an occluded right internal carotid artery. The patient was left with hemiparesis, but the cough headache had not recurred at the time of the case report. The authors admitted that the relation of the cough headache to the abnormality of the right internal carotid artery was uncertain. They recommended studying the carotid arteries in patients with cough headache when the headache was associated with focal neurologic symptoms or signs and no intracranial or foramen magnum lesion could be found (05). The patient described by Rivera and colleagues initially had right-sided cough headache alone (46). Four months later, the cough headache was associated with focal neurologic symptoms (right “blunted vision," weakness of the left arm, facial paresis, dysarthria, abnormal spontaneous movements of the left arm). The focal symptoms, which lasted 10 to 15 minutes, occurred after coughing and also with hypotension episodes during dialysis. He was admitted to the hospital and found to have bilateral carotid bruits with an otherwise normal neurologic examination. CT of the head was normal, and carotid ultrasound showed significant left common carotid stenosis. The patient was treated with antiplatelet agents and codeine with “transient improvement," and died 1.5 years after his cough headache started (46). Whether the carotid stenosis actually caused the cough headache is unclear.
Cough headache secondary to an unruptured cerebral aneurysm has been reported (52). The patient was a 42-year-old woman who for 24 days complained of severe right temporal pain induced by coughing or bending forward. The pain lasted 1 to 5 minutes and was followed by a dull ache that lasted 1 hour. Neurologic examination was normal 2 weeks after the onset of symptoms, and indomethacin failed to give her relief. Twenty-four days after headache onset, she complained of a continuous right-sided head pain that was worsened by coughing, straining, or bending forward. On examination, she was found to have right-sided ptosis, and the next day, she developed a more complete third nerve palsy. CSF showed 16 RBC/mm3 and 11 WBC/mm3, and cerebral angiogram revealed an 8 mm right-sided aneurysm at the junction of the posterior communicating artery and the internal carotid artery. She was free of cough headache postoperatively at 1-year follow-up (52). Rooke, however, reported that none of the 14 patients with unruptured aneurysms that he studied complained of exertional headache (47). Senegor reported a patient with a large venous angioma of the posterior fossa presenting with cough headache (50). However, close inspection of this case reveals that the headache was exacerbated, not elicited, by coughing and, thus, would not readily be confused with benign cough headache.
Patients with headache secondary to spontaneous CSF leak (CSF volume depletion or low-pressure headache) can present with intermittent, transient, severe headaches provoked by Valsalva-type maneuvers in the absence of orthostatic headache (33; 03; 14; 15). A patient with sinusitis and pneumocephalus on CT of the head and sinuses presented with symptoms of sinusitis and cough headache (20). It was felt that the patient’s coughing caused a fistula between the sinuses and the brain through a ball valve mechanism, and this led to pneumococcal meningitis. The cough headache and pneumocephalus seen on CT resolved with antibiotic therapy (20).
Kato and colleagues reported a case of a 52-year-old woman who was initially diagnosed as primary cough headache but was later found to have reversible cerebral vasoconstriction syndrome (21). They postulated that some cases of reversible cerebral vasoconstriction syndrome are caused by an aberrant sympathetic response to activities that cause an intracranial pressure surge. Primary cough headache should be recognized as a possible presentation of reversible cerebral vasoconstriction syndrome, even without thunderclap headache or neurologic deficits (21).
Pascual and colleagues found differences between patients with primary cough headache and primary exertional headache, lending credence to their separate classification (37). Cough headache was triggered by Valsalva maneuvers, whereas exertional headache was triggered by sustained physical exercise. Primary cough headache affected a much older population than those affected by primary exertional headache. The average age at onset for primary cough headache was 60 years, whereas the average age at onset for primary exertional headache was 40 years (37).
Migraine, cluster headache, and the headache associated with idiopathic intracranial hypertension can be aggravated, but not typically elicited, by cough (12; 55). A small number of patients have been reported, however, with cluster headache attacks triggered only by Valsalva maneuver, including coughing (23; 30).
Diagnostic workup
Given the differential diagnosis outlined above, every patient with cough headache should have an MRI of the brain to rule out a posterior fossa lesion. The MRI should be performed with gadolinium looking for pachymeningeal enhancement because headache secondary to spontaneous CSF leak can present as cough headache alone with no orthostatic component (33).
If imaging reveals tonsillar descent, one should consider dynamic CSF testing, which can help in situations when high or low CSF pressure is part of the differential diagnosis (32).
Whether a patient with an unruptured aneurysm can present with cough headache is not clear, but it seems reasonable to obtain an MRA of the intracranial circulation in most cases. We do not typically request carotid ultrasounds or MRAs of the extracranial circulation in the evaluation of cough headache unless the patient gives a history consistent with transient ischemic attacks.
Management
Any chest disease that may be causing the cough should be identified and treated (11). If an angiotensin-converting enzyme inhibitor is causing cough, it should be changed to a different agent if medically feasible (37). Because the typical duration of primary cough headache is typically short, preventive rather than abortive treatment is used. Mathew established the efficacy of indomethacin at a dose of 150 mg per day in a double-blind study involving two patients with primary cough headache (29). Of 16 patients treated with indomethacin, Raskin reported that 10 responded completely, four had moderate improvement, and two had no response (42). The effective dosage ranged from 50 mg to 200 mg, with an average of 78 mg, and the duration of treatment was 6 months to 4 years (42). Raskin has noted that indomethacin rarely fails but that the dosage sometimes must approach 250 mg daily (41).
Chen and colleagues reported that 44% of patients with primary cough headache had a complete response and 29% a partial response to indomethacin at doses of 25 mg two or three times per day (08). Indomethacin decreases intracranial pressure (51), and that may be why it is effective in this condition as compared with other nonsteroidal antiinflammatory agents. Because some patients lose the liability to primary cough headache over time, treatment should be withdrawn periodically.
Acetazolamide (56) and methysergide (07; 01) have been reported to be effective in open-label trials. In the Wang study, four of five patients with indomethacin-responsive primary cough headache responded favorably to acetazolamide at maximum doses of 1125 mg to 2000 mg per day. Two of these patients responded completely to acetazolamide. The patients were allowed to adjust the maintenance dose to treatment effectiveness or side effects, and the mean maintenance dose was 656 mg. One patient withdrew from the study because of intolerable distal limb numbness (56). The methysergide-responsive patient described by Calandre and colleagues had unilateral, throbbing, cough-induced headaches lasting 30 minutes to “a few hours" (07). This patient only had headaches with coughing, straining, and stooping and responded completely to methysergide at an unknown dose after failing nicardipine and propranolol and partially responding to amitriptyline. The methysergide-responsive patient described by Bahra and Goadsby had unilateral cough-induced headaches lasting 15 to 30 minutes (01). She required 2 mg daily and then lower doses for 9 weeks total and was then headache-free after cessation.
Topiramate inhibits carbonic anhydrase; it has been reported to be effective at a dose of 50 to 100 mg/day in three patients with primary cough headache (31).
Several authors have written of the occasional efficacy of lumbar puncture (53; 47; 42). Raskin noted the effectiveness of 40 ml lumbar puncture in six of 14 patients (42). Three had immediate relief after the procedure, and the other three had relief over 2 days. One of the responders redeveloped cough headache 6 weeks after the initial lumbar puncture but responded completely to repeat spinal tap. Six of the eight who failed lumbar puncture responded to indomethacin. Chen and colleagues reported that eight of 10 patients with primary cough headache reported much improvement or complete remission after “CSF drainage” (08). Presumably, this was a lumbar puncture, as the CSF drainage was “for both diagnosis and treatment.” Chen and colleagues recommend CSF drainage as a second-line treatment. One of the four patients who did not have a complete response to CSF drainage subsequently responded to indomethacin, but the other three patients did not.
Raskin has written of the effectiveness of naproxen, ergonovine, intravenous dihydroergotamine, and phenelzine but found propranolol ineffective (41). Mateo and Pascual found naproxen partially effective in one case (28). Of the six patients with cough headaches and normal MRIs described by Calandre and colleagues, one found propranolol effective, whereas two found it ineffective (07). Aside from Raskin’s observations (41), there is one other report of the efficacy of intravenous dihydroergotamine in treating cough headache (18). One study reported the resolution of cough-induced headache as well as suppression of cough in six male patients following parenteral administration of metoclopramide (17).
A case report by Moreno-Ajona reported that a 53-year-old male with primary cough headache became headache-free by using noninvasive vagal nerve stimulation three times daily (35). This treatment was still effective 3 months after initiation.
Special considerations
Pregnancy
There is no known risk to the mother or child if benign cough headache occurs during pregnancy. It is not advisable to continue medications during pregnancy.
Media
References
- 01
- Bahra A, Goadsby PJ. Cough headache responsive to methysergide. Cephalalgia 1998;18:495-6. PMID 9793705
- 02
- Bhadelia RA, Frederick E, Patz S, Dubey P, Erbay SH, Do-Dai D, Heilman C. Cough-associated headache in patients with Chiari I malformation: CSF flow analysis by means of cine phase-contrasted MR imaging. AJNR 2011;32:739-42. PMID 21330393
- 03
- Black DF, Eross EJ, Swanson JW. Post-traumatic low CSF pressure headache presenting as cough headache responsive to indomethacin. Headache 2005;45:836-7.
- 04
- Bougea A, Constantinides V, Anagnostou E, Kararizou E. An uncommon coexistence of primary sexual, cough and exercise headaches: the first three cases from Greece. Hippokratia 2015;19(4):369. PMID 27703312
- 05
- Britton TC, Guiloff RJ. Carotid artery disease presenting as cough headache. Lancet 1988;1:1406-7. PMID 2898088
- 06
- Bruyn GW. Cough headache. In: Vinken PJ, Bruyn GW, editors. Headache and Cranial Neuralgias. Handbook of Clinical Neurology. Amsterdam: North-Holland Publishing Company, 1968:185-7.
- 07
- Calandre L, Hernandez-Lain A, Lopez-Valdes E. Benign Valsalva’s maneuver-related headache: an MRI study of six cases. Headache 1996;36:251-3. PMID 8675432
- 08
- Chen PK, Fuh JL, Wang SJ. Cough headache: a study of 83 consecutive patients. Cephalalgia 2009;29(10):1079-85. PMID 19438909
- 09
- Chen YY, Lirng JF, Fuh JL, Chang FC, Cheng HC, Wang SJ. Primary cough headache is associated with posterior fossa crowdedness: a morphometric MRI study. Cephalalgia 2004;24(9):694-9. PMID 15315524
- 10
- Donnet A, Valade D, Houdart E, et al. Primary cough headache, primary exertional headache, and primary headache associated with sexual activity: a clinical and radiological study. Neuroradiology 2013;55(3):297-305. PMID 23117256
- 11
- Edmeads J. The worst headache ever: 2. innocuous causes. Postgrad Med 1989;86:107-10. PMID 2662150
- 12
- Ekbom K. Cough headache. In: Rose FC, editor. Headache. Handbook of Clinical Neurology. Amsterdam: Elsevier Science Publishers, 1986;4(48):367-71.
- 13
- Ertsey C, Jelencsik I. Cough headache associated with Chiari type-I malformation: responsiveness to indomethacin. Cephalalgia 2000;20:518-20. PMID 11037749
- 14
- Evans RW, Boes CJ. Spontaneous low cerebrospinal fluid pressure syndrome can mimic primary cough headache. Headache 2005;45:374-7. PMID 15836576
- 15
- Ferrante E. Aetiology of primary cough headache. Cephalalgia 2005;25:640. PMID 16033395
- 16
- Ferrante E. P040. Primary Valsalva maneuver headache without primary cough headache. J Headache Pain 2015;16(Suppl 1):A130. PMID 28132258
- 17
- Gupta VK. Metoclopramide aborts cough-induced headache and ameliorates cough—a pilot study. Int J Clin Pract 2007;61(2):345-8. PMID 16787437
- 18
- Hazelrigg RL. IV DHE-45 relieves exertional cephalgia. Headache 1986;26:52. PMID 3781828
- 19
- Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition. Cephalalgia 2018;38(1):1-211. PMID 29368949
- 20
- Jacome DE, Stamm MA. Malignant cough headache. Headache 2004;44(3):259-61. PMID 15012665
- 21
- Kato Y, Hayashi T, Sano H, Kato R, Tanahashi N, Takao M. Cough headache presenting with reversible cerebral vasoconstriction syndrome: a case report. Intern Med 2018;57(10):1459-61. PMID 29321411
- 22
- Killeen A, Roguski M, Chavez A, Heilman C, Hwang S. Non-operative outcomes in Chiari I malformation patients. J Clin Neurosci 2015;22(1):133-8. PMID 25080302
- 23
- Ko J, Rozen TD. Valsalva-induced cluster: a new subtype of cluster headache. Headache 2002;42(4):301-2. PMID 12010390
- 24
- Lance JW. Solved and unsolved headache problems. Headache 1991;31:439-45. PMID 1774158
- 25
- Lance JW, Goadsby PJ. Miscellaneous headaches unassociated with a structural lesion. In: Olesen J, Tfelt-Hansen P, Welch KM, editors. The Headaches. Philadelphia: Lippincott Williams and Wilkins, 2000:751-62.
- 26
- Lane RJ, Davies PT. Modified Valsalva test differentiates primary from secondary cough headache. J Headache Pain 2013;14:31. PMID 23565708
- 27
- Liu H, Cao X, Zhang M, et al. A case report of cough headache with transient elevation of intracranial pressure and bilateral internal jugular vein valve incompetence: a primary or secondary headache? Cephalalgia 2018;38(3):600-3. PMID 28376658
- 28
- Mateo I, Pascual J. Coexistence of chronic paroxysmal hemicrania and benign cough headache. Headache 1999;39:437-8. PMID 11279922
- 29
- Mathew NT. Indomethacin responsive headache syndromes. Headache 1981;21:147-50. PMID 7021474
- 30
- McGeeney BE. Valsalva-induced cluster headache. J Headache Pain 2006;7(6):416-8. PMID 17149564
- 31
- Medrano V, Mallada J, Sempere AP, Fernandez S, Piqueras L. Primary cough headache responsive to topiramate. Cephalalgia 2005;25:627-8. PMID 16033389
- 32
- Mitsikostas DD, Ashina M, Craven A, et al. European headache federation consensus on technical investigation for primary headache disorders. J Headache Pain 2015;17:5. PMID 26857820
- 33
- Mokri B. Spontaneous CSF leaks mimicking benign exertional headaches. Cephalalgia 2002;22:780-3. PMID 12485202
- 34
- Moncada E, Graff-Radford SB. Cough headache presenting as a toothache: a case report. Headache 1993;33:240-3. PMID 8320098
- 35
- Moreno-Ajona D, Villar-Martínez MD, Goadsby PJ, Hoffmann J. Primary cough headache treated with non-invasive vagal nerve stimulation. Neurology 2020;95(13):593-4. PMID 32753438
- 36
- Nightingale S, Williams B. Hindbrain hernia headache. Lancet 1987;1:731-4. PMID 2882140
- 37
- Pascual J, Gonzalez-Mandly A, Martin R, Oterino A. Headaches precipitated by cough, prolonged exercise or sexual activity: a prospective etiological and clinical study. J Headache Pain 2008;9(5):259-66. PMID 18751938
- 38
- Pavão Martins I, Viana P. “Cough hemicrania”–an overlapping form of headache: case reports. Headache 2017;57(5):796-800. PMID 28411371
- 39
- Perini F, Toso V. Benign cough “cluster” headache. Cephalalgia 1998;18:493-4. PMID 9793704
- 40
- Ran Y, Liu H, Zhang M, Dong Z, Yu S. Laugh‐induced headache: clinical features and literature review. Headache 2017;57(10):1498-1506. PMID 28925506
- 41
- Raskin NH. The indomethacin-responsive syndromes. In: Headache. 2nd edition. New York: Churchill Livingstone, 1988:255-68.
- 42
- Raskin NH. The cough headache syndrome: treatment. Neurology 1995;45:1784. PMID 7675251
- 43
- Raskin NH. Short-lived head pains. Neurol Clin 1997;15:143-52. PMID 9058402
- 44
- Raskin NH, Hosobuchi Y, Lamb S. Headache may arise from perturbation of brain. Headache 1987;27:416-20. PMID 3667258
- 45
- Rasmussen BK, Olesen J. Symptomatic and nonsymptomatic headaches in a general population. Neurology 1992;42:1225-31 PMID 1603351
- 46
- Rivera M, del Real MA, Teruel JL, Gobernado JM, Ortuno J. Carotid artery disease presenting as cough headache in a patient on haemodialysis. Postgrad Med J 1991;67:702. PMID 1924072
- 47
- Rooke ED. Benign exertional headache. Med Clin N Am 1968;52:801-8. PMID 5742064
- 48
- Sampaio Rocha-Filho PA. Headache associated with COVID-19: epidemiology, characteristics, pathophysiology, and management. Headache 2022;62(6):650-6. PMID 35545780
- 49
- Sansur CA, Heiss JD, DeVroom HL, Eskioglu E, Ennis R, Oldfield EH. Pathophysiology of headache associated with cough in patients with Chiari I malformation. J Neurosurg 2003;98(3):453-8. PMID 12650413
- 50
- Senegor M, Dohrmann GJ, Wollmann RL. Venous angiomas of the posterior fossa should be considered as anomalous venous drainage. Surg Neurol 1983;19:26-32. PMID 6828991
- 51
- Slavik RS, Rhoney DH. Indomethacin: a review of its cerebral blood flow effects and potential use for controlling intracranial pressure in traumatic brain injury patients. Neurol Res 1999;21:491-9. PMID 10439431
- 52
- Smith WS, Messing RO. Cerebral aneurysm presenting as cough headache. Headache 1993;33:203-4. PMID 8496059
- 53
- Symonds C. Cough headache. Brain 1956;79:557-68. PMID 13396063
- 54
- Symonds C. Cough headache. In: Studies in neurology. London: Oxford University Press, 1970:216-26.
- 55
- Wall M, Silberstein SD, Aiken RD. Headache associated with abnormalities in intracranial structure or function: high cerebrospinal fluid pressure headache and brain tumor. In: Silberstein SD, Lipton RB, Dalessio DJ, editors. Wolff’s Headache and Other Head Pain. Oxford: Oxford University Press, 2001:393-416.
- 56
- Wang SJ, Fuh JL, Lu SR. Benign cough headache is responsive to acetazolamide. Neurology 2000;55:149-50. PMID 10891932
- 57
- Williams B. Cerebrospinal fluid pressure changes in response to coughing. Brain 1976;99:331-46. PMID 990900
- 58
- Williams B. Cough headache due to craniospinal pressure dissociation. Arch Neurol 1980;37:226-30. PMID 7362488
- 59
- Wolff HG. Pain-sensitive structures within the cranial cavity. In: Headache and Other Head Pain. 2nd edition. New York: Oxford University Press, 1963:53-95.
- 60
- Zhang Y, Zhao X, Wang Y, Dong Z, Yu S. Prevalence and characteristics of cough headache in a Chinese respiratory clinic. Cephalalgia 2021;41(3):366-74. PMID 33175591
Contributors
All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.
Author
-
Brittany Heckel MD
Dr. Heckel of Thomas Jefferson University Hospitals received a consulting fee from AbbVie.
See Profile
Editor
-
Stephen D Silberstein MD
Dr. Silberstein, Director of the Jefferson Headache Center at Thomas Jefferson University has no relevant financial relationships to disclose.
See Profile
Former Authors
- Sameer Jain MD
- Ketan Jhunjhunwala MD
- Christopher J Boes MD, Manjit S Matharu PhD MRCP, Peter J Goadsby MD PhD, Cynthia E Armand MD, and Matthew S Robbins MD
Patient Profile
- Age range of presentation
-
- 19 to 64 years
- Sex preponderance
-
- male>female, >2:1
- Heredity
-
- none
- Population groups selectively affected
-
- None selectively affected
- Occupation groups selectively affected
-
- None selectively affected
ICD & OMIM codes
- ICD-9
-
- Headache: 784.0
- ICD-10
-
- Other specified headache syndromes: G44.8
This is an article preview.
Start a Free Account
to access the full version.
-
Nearly 3,000 illustrations, including video clips of neurologic disorders.
-
Every article is reviewed by our esteemed Editorial Board for accuracy and currency.
-
Full spectrum of neurology in 1,200 comprehensive articles.
-
Listen to MedLink on the go with Audio versions of each article.
Questions or Comment?
MedLink®, LLC
3525 Del Mar Heights Rd, Ste 304
San Diego, CA 92130-2122
Toll Free (U.S. + Canada): 800-452-2400
US Number: +1-619-640-4660
Support: service@medlink.com
Editor: editor@medlink.com
ISSN: 2831-9125
Also in This Category
-
-
Neuropharmacology & Neurotherapeutics
Acupuncture
Sep. 09, 2024
-
Headache & Pain
Migraine in childhood
Sep. 04, 2024
-
Headache & Pain
Headache attributed to head trauma
Aug. 28, 2024
-
Headache & Pain
Migraine: pathogenesis and pathophysiology
Aug. 24, 2024
-
Headache & Pain
Tension-type headache
Aug. 23, 2024
-
Headache & Pain
Benign paroxysmal vertigo
Aug. 23, 2024
-
Headache & Pain
Vagal nerve stimulation for headaches
Aug. 23, 2024