INTRODUCTION — Cough headache is an uncommon headache syndrome characterized by brief episodes of head pain triggered by cough or straining.
This topic will discuss primary cough headache. Other uncommon headache syndromes characterized by recurrent episodes of brief 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".)
CLINICAL FEATURES — Sometimes referred to as benign cough headache or Valsalva maneuver headache, primary cough headache is provoked by coughing or straining in the absence of any intracranial disorder [1].
Primary cough headache most often affects people over the age of 40. Cough headaches are sudden in onset, bilateral in distribution, and usually last from seconds to a few minutes, although some patients may have headache for up to two hours [1,2]. These headaches are not associated with nausea, vomiting, light or sound sensitivity, conjunctival injection, rhinorrhea, or lacrimation [3].
The true prevalence and incidence of primary cough headache is unknown, but one Danish population-based study found that the lifetime prevalence of benign cough headache was 1 percent [4].
DIFFERENTIAL DIAGNOSIS — Headaches provoked by coughing may occur either as a benign primary headache disorder or as a secondary headache. Structural lesions should be ruled out with neuroimaging before making the diagnosis of primary cough headache [1,2].
Several structural lesions have been associated with cough headache, including the following:
●Chiari type 1 malformations with or without syringomyelia [5-8]
●Posterior fossa lesions [3,9]
●Unruptured cerebral aneurysms [10]
●Intracranial hypervolemia [11]
●Spontaneous intracranial hypotensions (with positional headaches) [12]
●Unilateral carotid artery occlusion [13,14]
●Electrode implantation in the periaqueductal gray [15]
Limited evidence suggests that secondary etiologies are present in approximately one-half of patients who have cough-provoked headaches. In one series that included 30 patients who presented with headaches precipitated by coughing, the headaches were secondary to another cause in 17 patients (57 percent) [8]. 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.
Given these data, we suggest a neuroimaging evaluation, preferably with a contrast-enhanced brain MRI, for all patients presenting with de novo headache precipitated by coughing. We recommend such an evaluation for patients with posterior fossa symptoms or signs. In addition, we recommend a neurovascular evaluation (using CT or magnetic resonance [MR] 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.
A few of the secondary causes of cough-provoked headache are discussed in detail elsewhere. (See "Unruptured intracranial aneurysms" and "Spontaneous intracranial hypotension: Pathophysiology, clinical features, and diagnosis".)
Distinguishing primary and secondary forms of cough headache — Several clinical differences may help distinguish between benign primary cough headache and secondary cough headache [8]. These differences include a longer typical duration for secondary cough headache than for the primary form (under one minute for benign headaches versus seconds to days for secondary headaches), and the presence of posterior fossa signs or symptoms in secondary cough headache.
A response to indomethacin is more typical for primary cough headache than for secondary forms. However, the lack of response to indomethacin is probably not reliable as a sole distinguishing feature for secondary cough headache, since the cough headache associated with Chiari I malformation may respond to indomethacin [7].
In addition, headaches that occur in the context of other primary disorders, such as migraine, cluster headache, and hemicrania continua, may be intensified by coughing or Valsalva maneuvers [3]. The presence of associated symptoms suggestive of other primary headache disorders allows them to be distinguished from primary cough headache.
Distinguishing cough headache and exertional headache — Because Valsalva maneuvers frequently occur in the context of many forms of physical exertion, it is sometimes difficult to distinguish primary cough headache from primary exertional headache. (See "Exercise (exertional) headache".)
However, several clinical differences between benign cough and benign exertional headache have been identified that aid in distinguishing the two disorders [8]:
●Cough headache is triggered by Valsalva maneuvers, while exertional headache is triggered by more sustained physical exercise
●The average age of patients afflicted with benign cough headache is 67 (± 11), while the average age of patients with benign exertional headache is 24 (± 11)
●Primary cough headache typically has a faster onset than benign exertional headache, which builds up over minutes or longer
●Primary cough headaches are shorter than benign exertional headaches
●Primary cough headaches are typically sharp and stabbing in quality while benign exertional headaches are described as pulsatile
PATHOPHYSIOLOGY — The etiology of primary cough headache is poorly understood. One postulated mechanism is that coughing induces sudden increases in intra-abdominal and intrathoracic pressures that are transmitted through the valveless venous system into the intracranial venous sinus system, causing activation of intradural or perivascular nociceptive neurons.
In support of this hypothesis, one investigator measured cerebrospinal fluid pressure from the lumbar region and the cisterna magna during coughing in 16 patients requiring cervical myelography [16]. None of the cases were found to have 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 caused a pressure wave that was transmitted into the head and then rapidly downwards again [5].
These studies suggest that the upward passage of fluid from the spine towards the head is relatively unimpeded, but that the downward rebound from the head towards the spine is sufficient to cause tissue to jam in the foramen magnum. This creates a pressure gradient between the head and spine, termed the "craniospinal pressure dissociation" [16]. However, cough and Valsalva maneuvers that occur in the course of daily life are usually insufficient to activate intracranial nociceptive fibers, suggesting that patients with cough headache have some factor (or factors) that lowers activation thresholds and results in recurrent depolarization of the nociceptive fibers.
One study found stenotic abnormalities on magnetic resonance venography in 5 of 7 patients with primary cough headache, compared with 0 of 16 headache-free control subjects [17]. This finding suggests that venous outflow obstruction could be a promoting factor in some patients with primary cough headache.
PROGNOSIS — The prognosis for spontaneous recovery is fair at best. In the first reported series of 21 cases, nine had spontaneous recovery within 18 months to 12 years [18]. Two additional patients had improvement after lumbar puncture. Another two had relief after tooth extraction. The remaining patients continued to have cough headache until they were lost to follow-up.
TREATMENT — First, any pulmonary disease causing chronic coughing should be identified and treated if possible [19]. (See "Causes and epidemiology of subacute and chronic cough in adults" and "Evaluation and treatment of subacute and chronic cough in adults".)
The treatment of choice for primary cough headache is indomethacin. A small, double-blind, controlled study found that indomethacin (150 mg daily) was effective treatment [20]. In other series, indomethacin doses up to 250 mg per day have been required [21].
Indomethacin decreases intracranial pressure [22], and that may be why it is effective in this condition as compared with other nonsteroidal antiinflammatory drugs. Because of the strong potential for gastrointestinal irritation with chronic indomethacin use, the addition of a proton pump inhibitor is probably prudent in those patients requiring long-term indomethacin treatment. (See "NSAIDs (including aspirin): Primary prevention of gastroduodenal toxicity".)
Some patients may lose their tendency for cough headache with time, suggesting that periodic indomethacin withdrawal after six to 12 months of therapy is also wise.
Other medications reported to be effective for primary cough headache in open-label trials or case series include acetazolamide [11], propranolol [23], methysergide [24], naproxen [25], ergonovine, intravenous dihydroergotamine, and phenelzine [26].
In addition to medical therapy, several authors have reported occasional benefit from cerebrospinal fluid removal by lumbar puncture [18,27]. One author reported that patients who have been unresponsive to indomethacin may improve after high volume (40 mL) cerebrospinal fluid removal [21].
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
●Primary cough headache most often affects people older than age 40 and is provoked by coughing or straining in the absence of any intracranial disorder. (See 'Clinical features' above.)
●For patients presenting with de novo headache precipitated by coughing, we suggest a neuroimaging evaluation, preferably with a contrast-enhanced brain MRI. We recommend such an evaluation for patients who have posterior fossa symptoms or signs. In addition, we recommend a neurovascular evaluation with CT or magnetic resonance (MR) 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.)
●Because Valsalva maneuvers frequently occur in the context of many forms of physical exertion, it is sometimes difficult to distinguish primary cough headache from primary exertional headache. (See 'Distinguishing cough headache and exertional headache' above.)
●For patients with primary cough headache, we suggest treatment with indomethacin (150 to 250 mg daily) (Grade 2C). Alternative medications that may be effective include acetazolamide, propranolol, methysergide, naproxen, ergonovine, intravenous dihydroergotamine, and phenelzine. Any pulmonary disease causing chronic coughing should be identified and treated if possible. (See 'Treatment' above.)
