Cough, exertional, and sexual headaches

Headache provoked by cough, exercise, and sexual activity can be symptomatic of nonintracranial disorder or an unpleasant but harmless inconvenience. The headache classification of the International Headache Society (IHS) includes within the group of "miscellaneous headaches unassociated with structural lesion'' the entities of benign cough headache, benign exertional headache, and headache associated with sexual activity. ^[1] Benign cough headache is defined as a bilateral headache of sudden onset, lasting less than 1 minute, and precipitated by coughing in the absence of any intracranial disorder. Benign exertional headache is a bilateral, throbbing headache, lasting from 5 minutes to 24 hours, specifically provoked by physical exercise and unassociated with any systemic or intracranial disorder. "Headache associated with sexual activity'' describes bilateral headaches precipitated by masturbation or coitus, also in the absence of any intracranial disorder. Three types of sexual headache are recognized in the IHS classification. ^[1] The first, the dull type, is described as a dull ache in the head and neck that intensifies as sexual excitement increases. Type 2, also known as vascular or explosive, is a sudden, severe, explosive headache occurring at orgasm. Type 3 is postural headache, resembling that of low CSF pressure, developing after coitus. Following the IHS criteria, Rasmussen and Olesen ^[2] have shown that the lifetime prevalences of these headaches are 1% in each case (95% CI, 0 to 2%).

In 1932, Tinel ^[3] described four patients with intermittent paroxysmal headache that occurred only with effort, which he called "la cephalee a l'effort.'' Sir Charles Symonds, ^[4] in 1956, reported 27 similar patients with transient severe head pain provoked by coughing, sneezing, straining at stool, laughing, or stooping. He subdivided the patients into two groups. The first included six subjects with structural cranial disease, and the second included 21 patients without any clinical evidence of a spaceoccupying lesion. ^[4] In his landmark paper, Rooke, ^[5] in 1968, proposed the term "benign exertional headache'' for any headache precipitated by "exertion'' (such as running, bending, coughing, sneezing, heavy lifting, or straining at stool) that has an acute onset and is unassociated with structural brain disease. Even though the IHS classification separates headache precipitated by coughing from that precipitated by exercise, ^[1] the influence of Symonds ^[4] and Rooke's ^[5] remains; Sands et al., ^[6] in 1991, lumped together the 219 cases of cough and exertional headache previously reported. Despite most of these cases having been studied before the advent of modern cranial imaging, Sands et al. found that one of five cases were secondary to structural lesions, mainly of posterior fossa.

Since the original descriptions, ^[7-10] benign sexual headache has become a well-defined entity with three types described according to the presumed clinical pathophysiologic mechanisms. ^[1] The most frequent, the vascular type or type 2, begins suddenly at the time of orgasm and is thought to be related to hemodynamic changes. ^[6,9,11] The relationship between sexual headache and exertional headache is uncertain. In the experience of Lance ^[9] physical exertion did not seem to play an important role in patients with sexual headache. However, Silbert et al. ^[12] found that 40% of patients with sexual headache also experience benign exertional headache.

From these conflicting data the need seems clear to report new series of patients with these headaches, which have been studied with modern neuroradiologic procedures. We present our experience with these provoked headaches, evaluate the relationships between them, and examine the possible symptomatic cases.

Patients and methods.

The medical charts of patients consulting our service in the last 15 years whose main complaint was headache precipitated by coughing, physical exercise, or sexual excitement were reviewed. Our service receives, directly from general practitioners, all patients in our region requiring neurologic advice. There is no private neurologic practice in our health area. ^[13] Patients were included in this study only if, after an appropriate follow-up, the final diagnosis had been performed by one of the staff neurologists according to history, examination, and, at times, special investigations. We excluded patients with headache in whom this was not the main symptom for consultation.

For all patients, we obtained information about age at onset of headache, sex, medical antecedents, and personal or immediate family history of migraine and other headaches. We also recorded in detail the general clinical characteristics of headaches in all patients (precipitants, localization, quality, severity, duration, frequency, associated symptoms, and effects of treatments).

Statistical analysis was performed using the Student t test (age) and chi-square test (sex). Values are expressed as mean +/- SD.

Results.

Seventy-two patients had attended our service due to headache precipitated by coughing (n = 30), physical exercise (n = 28), or sexual excitement (n = 14). These 72 patients represent 1.9% of all consultations due to headache in our unit in the same period of time. ^[14] Of these 72 patients, 42 (58%) patients fulfilled present criteria for the diagnosis of benign cough, exercise, or sexual headache, and 30 (42%) had cough or exercise headache symptomatic of a variety of intracranial lesions Table 1.

Discussion.

Apart from that of Rooke, ^[5] this is the largest series of headaches of sudden onset provoked by cough, physical exercise, or sexual excitement. In contrast to Rooke's series, in which only 10% of patients had intracranial lesions, almost half of our cases (42%) had intracranial lesions. With cough headache, symptomatic cases (57%) were even more frequent than benign cases. Because our series includes patients referred directly from the general practitioners in our health area, these differences do not seem to be related to referral bias. The explanation for this finding is the current ease of diagnosing of tonsillar descent with MRI, which was not available for Rooke.

The etiology of symptomatic provoked headaches was different for each category. Chiari type I malformation was the only cause for symptomatic cough headache ^[16,17]; subarachnoid hemorrhage, sinusitis, and brain metastases were the etiologies for symptomatic exertional headache; and subarachnoid hemorrhage was the cause for the only case of symptomatic type 2 sexual headache.

Although the precipitants were the same for both categories, benign and symptomatic cases differed in several clinical aspects. Symptomatic cough headache began earlier in life and tended to last longer than benign cough headache. Three patients, who underwent MRI demonstrated tonsillar descent, initially only had isolated cough headache, but they did not have any response to indomethacin. In addition, all patients with symptomatic cough headache developed posterior fossa manifestations and the headaches were not relieved by indomethacin as were the benign cases. However, because MRI was not performed in all benign cough headache patients in this series, some could have had tonsillar descent. We recommend MRI in all patients with cough headache, and mandate it when there is no response to indomethacin, in patients with posterior fossa signs, or in those under 50. The pathophysiology of these headaches remains obscure. While the degree of tonsillar descent correlates with the presence of cough headache in Chiari type I patients, ^[16] Raskin ^[18] has recently reported the response of some patients with benign cough headache to lumbar puncture. This author has proposed that benign cough headache could be due to the sensitization of the receptors sensitive to the increase in intracranial pressure occurring with coughing, lifting, or straining. ^[18]

Symptomatic exertional and sexual headaches began later in life and lasted longer than benign exertional and sexual headaches. Male predominance was not present in the symptomatic exertional headache group. Furthermore, all patients with these symptomatic headaches had manifestations of meningeal irritation or intracranial hypertension. Those with subarachnoid bleeding had only had one headache episode. Although neuroradiologic studies could be avoided in cases with clinically typical benign sexual or exertional headaches (men around the third decade of life, with short-duration, multiple episodes of pulsating pain, response to ergotamine or to preventive beta-blockers, and normal examination), the remaining patients must have brain CT and CSF examination if the CT scan is normal.

Benign cough and exertional headaches are separate conditions. Besides the different precipitants (sudden Valsalva maneuvers for cough headache and sustained physical exercise for exertional headache) benign cough headache began later than benign exertional headache. Cough headache started 43 years later, on average, than exertional headache and, while the youngest patient with benign cough headache in this series was 44 years old, the oldest patient with benign exertional headache was 48. Benign cough headache tended to be shorter than benign exertional headache and the pain quality and responses to treatment were different. Benign cough headache was described as sharp or stabbing and responded to indomethacin, whereas benign exertional headache was pulsating, tended to last longer, and improved with ergotamine or propranolol.

Until the recent paper by Silbert et al., ^[12] reports of patients experiencing both benign sexual headache and benign exertional headache were very infrequent. ^[8,9,19] In the original series ^[9] of 21 patients with types 1 and 2 benign sexual headache, two patients with benign vascular headache also experienced exertional headaches, though of different quality, as were the exertional headaches in the patient described by Paulson and Klawans. ^[8] Silbert et al., ^[12] however, found a definite relationship between type 2 benign sexual headache and benign exertional headache in 40% of their patients. Although we did not find a relationship between benign exertional headache and benign cough headache, our findings with sex and exertional headaches are in agreement with Silbert et al. ^[12] Four of our 13 patients (31%) consulting us due to benign sexual headache admitted to episodes fulfilling the IHS criteria for benign exertional headaches. Furthermore, male predominance, general characteristics (duration, frequency, persistence, and quality) of the pain attacks, and treatment response were very similar. The only significant difference between these two entities, the age at onset (24 years in exertional headache and 41 years in vascular sexual headache), does not necessitate an actual difference, since younger people carry out more "exertional'' activities whereas the middleaged may have more regular "sexual'' activity. Thus, both entities may share the same vascular hyperreactivity provoked by exercise, sexual excitement, or both depending on the habits of the patient at different ages.

Acknowledgment

We are indebted to John Hawkins for the stylistic revision of the manuscript.