Primary cough headache

INTRODUCTION — Cough headache is an uncommon headache syndrome characterized by brief episodes of head pain triggered by cough, straining, or other Valsalva maneuvers.

This topic will discuss primary cough headache. Other uncommon headache syndromes characterized by recurrent episodes of brief head pain are discussed separately.

●(See "Overview of thunderclap headache".)

●(See "Exercise (exertional) headache".)

●(See "Primary headache associated with sexual activity".)

●(See "Hypnic headache".)

●(See "Primary stabbing headache".)

●(See "Cold stimulus headache".)

PATHOPHYSIOLOGY — The etiology of primary cough headache is poorly understood. Increases in intra-abdominal pressure with coughing may be transmitted to cerebrospinal fluid (CSF) and/or intracerebral venous structures and cause nociceptor activation in susceptible patients.

In small studies of patients undergoing cervical myelography, CSF pressure was measured during coughing in both the lumbar region and the cisterna magna [1,2]. None of the cases were found to have complete blockage of the spinal subarachnoid space. However, during coughing, intrathoracic and intra-abdominal pressure caused a pressure wave that was transmitted into the head and then rapidly downwards again. CSF passed from the spine towards the head relatively unimpeded, but the downward rebound from the head towards the spine caused tissue to temporarily obstruct the foramen magnum. This process is postulated to create a pressure gradient between the head and spine, termed the "craniospinal pressure dissociation" [2]. Cough and other Valsalva maneuvers that typically occur during daily activities are insufficient to produce such symptoms in most individuals, suggesting that patients with cough headache have some factor (or factors) that lowers activation thresholds and results in recurrent depolarization of the nociceptive fibers.

Another postulated mechanism is that coughing induces sudden increases in intra-abdominal and intrathoracic pressures transmitted through the valveless venous system into the intracranial venous sinus system, causing activation of intradural or perivascular nociceptive neurons [3,4].

Venous outflow obstruction may also be a promoting factor in some patients with primary cough headache. One study found stenotic abnormalities on magnetic resonance venography in five of seven patients with primary cough headache compared with 0 of 16 headache-free control subjects [3].

EPIDEMIOLOGY — The prevalence and incidence of primary cough headache is unknown, as symptoms are often brief and may be underreported or misattributed to other headache syndromes. One Danish population-based study found that the lifetime prevalence of benign cough headache was 1 percent [5]. The prevalence of cough headache among 679 patients evaluated for cough in a pulmonary clinic was found to be 18 percent [6].

Primary cough headache appears to be more common among males (2.5:1) and most often affects individuals over the age of 40 years with a mean age at presentation of approximately 60 years [7-9]. However, primary cough headache has also been reported to occur in a child as young as seven years old [4].

CLINICAL FEATURES — Primary cough headache is typically provoked by coughing but also may be provoked by straining or another Valsalva-type maneuver such as sneezing or laughing.

Cough headaches are sudden in onset and come to a peak severity within several seconds to a few minutes, although some patients may have a headache for up to two hours [10,11]. They may be sharp or even explosive in character and severe in intensity. Location is typically bilateral and occipital but also may be unilateral or diffuse [8,9].

These headaches are not generally associated with nausea, vomiting, light or sound sensitivity, conjunctival injection, rhinorrhea, or lacrimation [12]. The presence of other neurologic signs or symptoms should prompt evaluation for secondary causes of headache, such as Chiari type I malformation. (See 'Secondary (symptomatic) cough headache' below.)

DIAGNOSIS AND EVALUATION — Primary cough headache should be considered in patients who develop a headache immediately following coughing, straining, or another Valsalva-type maneuver. The diagnosis of primary cough headache is made in patients whose symptoms fulfill diagnostic criteria when alternative causes have been excluded [11].

Diagnostic criteria — Diagnostic criteria for primary cough headache by the International Classification of Headache Disorders, 3^rd edition (ICHD-3) include all of the following [10]:

●(A) At least two headache episodes that fulfill criteria B to D

●(B) Headache brought on by and occurs only associated with coughing, straining, and/or other Valsalva maneuvers

●(C) Sudden onset

●(D) Headache lasts between one second and two hours

●(E) Not better accounted for by another ICHD-3 diagnosis

Probable primary cough headache may be diagnosed for patients either with one headache episode that fulfills other diagnostic criteria or two cough-related headache episodes that are either sudden in onset or last for <2 hours in duration.

Neuroimaging evaluation — We suggest neuroimaging evaluation for all patients with new headaches that are triggered by or associated with cough. Symptoms that fulfill diagnostic criteria for primary cough headache do not exclude the possibility of an underlying structural cause to symptoms [10,11]. Neuroimaging is required for patients with cough-related headaches and neurologic signs or symptoms.

We perform brain magnetic resonance imaging (MRI) with gadolinium contrast for patients with new cough-related headaches to evaluate for structural causes. Computed tomography (CT) of the headache with contrast may be performed as a less sensitive alternative for patients unable to undergo brain MRI.

For patients with unilateral cough-provoked headache and contralateral transient focal neurologic symptoms, we also suggest a neurovascular evaluation with CT or magnetic resonance angiography of the head and neck to evaluate for arterial dissection.

Differential diagnosis — Headaches provoked by coughing may occur either as a primary headache disorder or as a secondary headache. Specific clinical features and neuroimaging may help distinguish among these entities [13].

Secondary (symptomatic) cough headache — Structural lesions that have been associated with cough headache include the following:

●Chiari type I malformation with or without syringomyelia [1,13-15] (see "Chiari malformations", section on 'Chiari I clinical features')

●Carotid artery dissection or occlusion [16-18] (see "Cerebral and cervical artery dissection: Clinical features and diagnosis", section on 'Head and neck pain')

●Acute sinusitis [8] (see "Acute sinusitis and rhinosinusitis in adults: Clinical manifestations and diagnosis", section on 'Clinical features')

●Spontaneous intracranial hypotension from spinal cerebrospinal fluid leak [19,20] (see "Spontaneous intracranial hypotension: Pathophysiology, clinical features, and diagnosis", section on 'Associated symptoms')

●Posterior fossa tumors [12,21] (see "Brain tumor headache", section on 'Clinical features')

●Reversible cerebral vasoconstriction syndrome [22] (see "Reversible cerebral vasoconstriction syndrome", section on 'Clinical presentation and course')

●Unruptured cerebral aneurysms [23] (see "Unruptured intracranial aneurysms", section on 'Clinical manifestations')

Secondary etiologies may be present in approximately one-half of patients who have cough-provoked headaches, most commonly associated with Chiari type I malformations [24]. In one series that included 30 patients who presented with headaches precipitated by coughing, headaches were secondary to another cause in 17 patients (57 percent) [13]. All 17 had a Chiari type I malformation, with cerebellar tonsillar descent of >3 mm below the foramen magnum by MRI. Fourteen of these patients also had signs or symptoms related to the posterior fossa/foramen magnum region, and five had evidence of syringomyelia. In another series of patients with spontaneous intracranial hypotension due to cerebrospinal fluid-venous fistula, cough or Valsalva-induced headache was a presenting feature in 88 percent [20].

Secondary cough headaches due to a structural lesion typically persist longer than primary cough headaches (hours rather than seconds), are more likely to be occipital in location, and may be associated with brainstem neurologic signs and symptoms [25]. A response to indomethacin is more typical for primary cough headache than for secondary forms (see 'Treatment' below). However, the lack of response to indomethacin is not reliable as a sole distinguishing feature for secondary cough headache, since the cough headache associated with Chiari I malformation may also respond to indomethacin [15].

Other primary headaches — Cough, straining, or other Valsalva maneuvers may trigger other primary headache syndromes. It may be difficult to distinguish primary cough headache from these other syndromes. The clinical setting and pattern of headaches may help to discriminate.

●Exercise (exertional) headache – Exertional headache is typically triggered by sustained physical exercise rather than a transient Valsalva-type maneuver. Headaches are pulsatile in character and tend to occur in younger adults, unlike primary cough headaches which are stabbing in character and more common in older adults [13]. In addition, exercise headaches typically develop more slowly (over minutes) and last longer (hours) than primary cough headache. (See "Exercise (exertional) headache".)

●Primary stabbing headache – Primary stabbing headaches are characterized by brief episodes of sudden head pain but are typically unprovoked and may recur up to several times each day, unlike primary cough headache which are always associated with cough or Valsalva maneuvers. (See "Primary stabbing headache".)

●Primary headache associated with sexual activity – The explosive type of primary headache associated with sexual activity is characterized by sudden-onset, severe head pain that occurs just prior to or at the moment of orgasm. Head pain may be severe and brief, lasting several minutes, similar to primary cough headache. However, patients with primary headache associated with sexual activity may also have a residual dull headache for up to 72 hours. (See "Primary headache associated with sexual activity".)

●Idiopathic intracranial hypertension – The headache associated with idiopathic intracranial hypertension (IIH) may be triggered or exacerbated by cough or other Valsalva maneuvers. In addition, some patients with primary cough headache have been reported to improve with acetazolamide, a treatment commonly used to reduce cerebrospinal fluid (CSF) production in IIH [26]. However, the headache in IIH can occur without cough or other provocation and may be persistent or chronic in duration. Patients with IIH may also have other associated symptoms such as diplopia, vision loss, and/or tinnitus. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Clinical features and diagnosis".)

In addition, migraine and cluster headache may be exacerbated by coughing or Valsalva maneuvers [12]. However, coughing is an uncommon trigger for migraine or cluster headaches. The presence of associated features suggestive of these other primary headache disorders also allows them to be distinguished from primary cough headache. (See "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults", section on 'Clinical features' and "Cluster headache: Epidemiology, clinical features, and diagnosis", section on 'Clinical features'.)

TREATMENT — Management of patients with primary cough headache involves addressing any causes of chronic cough or straining that provoke headaches and using preventive medications.

●Treat causes of chronic cough – Some patients with primary cough headache may report relief with successful treatment of underlying causes to chronic cough. Pulmonary, cardiac, and gastrointestinal disorders causing chronic coughing should be identified and treated if possible [27]. These may include asthma, bronchitis, gastroesophageal reflux, and heart failure. (See "Causes and epidemiology of subacute and chronic cough in adults" and "Evaluation and treatment of subacute and chronic cough in adults".)

Patients taking angiotensin-converting enzyme inhibitors who have chronic cough may warrant switching to an alternative medication, such as an angiotensin-receptor blocker. (See "Major side effects of angiotensin-converting enzyme inhibitors and angiotensin II receptor blockers", section on 'Cough'.)

●Initial preventive treatment with indomethacin – For patients with persistent or bothersome headaches, we suggest treatment with indomethacin. Treatment with indomethacin may be effective for cough headache due to its effect of decreasing intracranial pressure [28].

We start at 25 mg three times daily, then increase each dose by 25 mg every three days as needed to a typical total daily dose of 150 mg and a maximum total daily dose of 225 mg, given in three divided doses. We typically continue maintenance therapy using the lowest effective dose for at least two to three months. Sustained resolution of symptoms frequently indicates remission; for these patients, we attempt to wean indomethacin. Indomethacin may be resumed if symptoms recur.

Adverse effects of indomethacin include gastritis, bleeding, hepatic dysfunction, kidney impairment, hypertension, and congestive heart failure. Because of the strong potential for gastrointestinal irritation with chronic indomethacin use, we suggest using gastroprotection with proton pump inhibitors, or histamine H2-receptor blockers for all patients requiring long-term indomethacin treatment. (See "NSAIDs (including aspirin): Primary prevention of gastroduodenal toxicity".)

Indomethacin (50 mg three times daily) was first found to be effective at suppressing symptoms in a blinded, case-control series involving two patients with cough-induced headache [29]. In other series, indomethacin doses up to 250 mg per day have been reported as effective [30]. Many patients report resolution of symptoms within a week of starting therapy, even at initial indomethacin doses. In one series of patients with primary cough headache, symptoms resolved with indomethacin in 44 percent, and partial improvement was reported in nearly 75 percent [8].

●Alternative preventive options – Other medications reported to be effective for primary cough headache in open-label trials or case series include:

•Propranolol [31]

•Naproxen [32]

•Topiramate [33]

•Acetazolamide [26]

•Methysergide [34]

•Intravenous dihydroergotamine [35]

•Phenelzine [35]

In addition to pharmacotherapy, cerebrospinal fluid (CSF) removal by lumbar puncture has also been used [36,37]. High-volume CSF removal (40 mL) may be effective when symptoms are unresponsive to indomethacin or other medications [30]. Noninvasive vagal nerve stimulation has also been reported effective in one case report [38].

PROGNOSIS — Limited data suggest the prognosis is good for sustained resolution of symptoms due to primary cough headache. In a series of 83 patients with cough headache, remission was reported in 84 percent at a mean of 51 months [8]. Among patients whose symptoms responded initially to indomethacin, 94 percent remained headache free at follow-up.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Migraine and other primary headache disorders".)

SUMMARY AND RECOMMENDATIONS

●Definition – Primary cough headache is an uncommon headache syndrome characterized by brief episodes of head pain triggered by cough or straining, not due to an underlying structural cause. It appears to be more common among males (2.5:1), and most present at a mean age of approximately 60 years. (See 'Introduction' above and 'Epidemiology' above.)

●Clinical features – Primary cough headaches are sudden in onset, come to a peak severity within several seconds to a few minutes, and may persist for up to two hours. Pain may be severe; the location is typically bilateral and occipital but also may be diffuse. (See 'Clinical features' above.)

●Diagnosis and evaluation – The diagnosis of primary cough headache is made in patients with at least two episodes of sudden-onset headache that are triggered by coughing, straining, and/or other Valsalva maneuvers and last between one second and two hours, after alternative causes have been excluded.

We suggest neuroimaging evaluation for patients with new headaches that are triggered by or associated with cough to exclude underlying structural causes to symptoms. We typically perform brain MRI with gadolinium contrast for all patients. In addition, we recommend a neurovascular evaluation with CT or magnetic resonance angiography of the intracranial and extracranial vessels if the patient has a recent history of unilateral cough-provoked headache with contralateral transient focal neurologic symptoms. (See 'Differential diagnosis' above.)

●Differential diagnosis – Headaches provoked by coughing may occur either as a primary headache disorder or as a secondary headache.

•Other primary headache disorders that may be triggered by cough or Valsalva include exercise headache, primary stabbing headache, primary headache associated with sexual activity, and idiopathic intracranial hypertension. The specific clinical features and setting may be used to discriminate among these headaches.

•Secondary etiologies may be present in approximately one-half of patients who have cough-provoked headaches, most commonly associated with Chiari type I malformations. Other entities include carotid artery dissection, sinusitis, spontaneous intracranial hypotension, and intracranial tumors. Specific clinical features and neuroimaging may help distinguish among these entities.

●Treatment – The initial management of patients with primary cough headache involves addressing any causes of chronic cough or straining that provoke headaches. For patients with persistent or bothersome headaches, we suggest treatment with indomethacin (Grade 2C). We titrate as needed to a typical daily dose of 150 mg and typical maximum dose of 225 mg, divided in three doses. Alternative treatments that may be effective include propranolol, naproxen, topiramate, acetazolamide, methysergide, dihydroergotamine, phenelzine, or high-volume lumbar puncture. (See 'Treatment' above.)