Primary exercise headache …

Headache & Pain

Updated 03.21.2024
Released 09.26.1996
Expires For CME 03.21.2027

Primary exercise headache

Authors: Calvin Chan MBChB, Peter J Goadsby MD PhD FRS

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Editor: Stephen D Silberstein MD

Primary exercise headache

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Introduction

Overview

Primary exercise headache is a rare syndrome where headache can be induced during or after physical exertion. Intracranial pathology can present with exercise-induced headaches, and therefore, it is imperative to investigate for secondary causes. Other Valsalva-induced headache syndromes, primary cough headache and primary headache associated with sexual activity, are discussed in dedicated MedLink Neurology articles.

Key points

	• Primary exercise headache is a diagnosis of exclusion. Headache induced by exertion is a red flag and imaging is necessary to rule out secondary causes.
	• Phenotypically, the headache begins within 30 minutes of cessation of exertion, typically lasts up to 4 hours, and can be associated with migrainous features.
	• Prevention of headache attacks can be through modification of exercise. Where this is not possible or successful, prophylactic indomethacin can be utilized.
	• Patients typically experience spontaneous resolution.

Historical note and terminology

Headache associated with exercise or exertion has been noted as early as classical Greece when Hippocrates wrote, “one should be able to recognize those who have headaches from gymnastic exercises, or running, or walking or hunting, or any other unseasonable labour, or from immoderate venery…” (02).

The first description in modern medicine was in 1932 by Tinel, who presented four patients with intermittent, paroxysmal headaches following exertion and surmised the cause was distention of the intracranial veins due to increased intrathoracic pressure (44). Symonds’s observations differentiated this further and described headaches induced by Valsalva-like maneuvers and not exertion (42), now classified as primary cough headache (19).

Jokl self-described “an attack of nausea, headache, prolonged weakness, and vomiting” brought on after running that lasted 15 minutes (22).

He later noted several profound cases of “effort migraine” during the Olympic Games in Mexico City (23). He described high altitude, heat, humidity, and perhaps lack of training as predisposing factors. These headaches tended to occur with prolonged running rather than sprints. These highly conditioned athletes developed scotomata, unilateral retro-orbital pain, nausea and vomiting, and, in some cases, a striking prostration. These descriptions are more in keeping with migraine triggered by exertion.

The first large published series on exertion-induced headache or head pain related to exertion came from the Mayo Clinic (38). The cohort included 103 patients with headache precipitated by running, bending over, coughing, sneezing, straining, or heavy lifting. Of their cohort, 90% were diagnosed with “benign exertional headache unassociated with intracranial lesions”; however, the phenotype was not differentiated by either precipitation with sudden Valsalva maneuvers or sustained exertion or exercise.

Primary exercise headache was initially termed “benign exertional headache” in the first edition of the International Classification of Headache Disorders (17). It was subsequently renamed “primary exertional headache” in the second edition (18) and is now described as “primary exercise headache” in the third edition (19).

Clinical manifestations

Presentation and course

The ICHD-3 diagnostic criteria for primary exercise headache are detailed in Table 1.

A variety of sports have been associated with primary exercise headache, including running (22), weightlifting (35), Australian Football League (31), rugby union (49), cycling (46), and swimming (21; 32). It could be noted that two of the three swimming cases reported by Indo and Takahashi may have been cold-stimulus headaches, and two had migrainous features.

Most patients report onset of headache within 30 minutes of cessation of exercise (36; 45). The headache duration can range from minutes to less than 1 day (16; 36; 45). Adolescents may report a shorter headache duration, with most respondents reporting attacks up to 4 hours (04). A more prolonged form of exercise-induced headache has been reported. Attacks can last more than a day, with the longest reported attacks lasting up to 2 days (36).

Most patients report a pulsating headache; however, the nature of the pain can be described as aching, pounding, or throbbing (04; 45). The headache can be either bilateral or unilateral. More recent studies report a higher incidence of bilateral headache (46; 16; 36; 45). The frontal region is the most frequently reported headache location (04; 36; 45). Pain intensity is variable.

Patients who experience primary exercise headache may also report many of the characteristics of migraine, such as nausea, vomiting, photophobia, and phonophobia (04; 46). These symptoms were reported more often in females (04). Patients with comorbid migraine and primary exercise headache are more likely to report headaches lasting more than 1 hour, nausea, photophobia, and phonophobia than patients with primary exercise headache alone (04). However, this finding should be interpreted with caution as it would be difficult to differentiate primary exercise headache with migraine features from migraine triggered by physical activity (19). A principle used in headache classification is that the diagnosis should not be if the phenotype can be accounted for by another headache diagnosis. It could be argued for research purposes that only featureless exercise-triggered headache should be classified as such. Indeed, in clinical practice there is little to be gained in the diagnosis of exercise headache in the presence of migrainous features as treatments for migraine increase over time.

Heat, high humidity, changing barometric pressure, exercise at high altitudes, caffeine, poor nutrition, hypoglycemia, and alcohol usage are believed to be contributing factors (36; 45).

Table 1. Diagnostic Criteria for Primary Exercise Headache

Description: Headache precipitated by any form of exercise in the absence of any intracranial disorder.
Diagnostic criteria:
	(A) At least two headache episodes fulfilling criterion B and criterion C
	(B) Brought on by and occurring only during or after strenuous physical exercise
	(C) Lasting less than 48 hours
	(D) Not better accounted for by another ICHD-3 diagnosis
(19)

Prognosis and complications

The prognosis for patients with primary exercise headache is good. Spontaneous resolution has been reported by patients after months to several years (38; 40; 41; 16). In the cohort reported by Rooke, approximately one third of patients with primary exercise headache will experience spontaneous resolution within 5 years of onset, and approximately 80% will have spontaneous remission after 10 years of onset (38). The most recent cohort described by Hanashiro and colleagues found almost all patients had resolution within their 1-year follow-up from diagnosis.

Clinical vignette

A 62-year-old female presented with a 4-year history of headache on 28 out of 30 days a month. The headaches were always associated with strenuous exercise and would occur within 15 minutes of exercise cessation.

The pain was in the left facial temporal regions with pain involvement bilaterally in the throat, neck, shoulder, and upper and lower teeth. The pain typically lasts for 20 minutes with a range of 2 to 60 minutes, where resolution occurs with cessation of exercise. The headache was not associated with aura symptoms, migrainous features, or cranial autonomic symptoms. She did not report premonitory-like or postdromal symptoms.

Paracetamol was not effective in treating the headache.

The headache was not associated with cough or sexual activity.

As a teen, she had headaches, which would be consistent with migraine with visual aura. There was a family history of migraine in her mother.

The neurologic examination was unremarkable. The brain MRI, neck MRI, intracranial and extracranial MRA were normal. She was assessed by cardiology, and she did not have significant coronary artery disease.

Biological basis

	• The pathogenesis of primary exercise headache remains unclear.
	• Imaging evidence suggests altered cerebral venous dynamics may cause primary exercise headache in some patients.

Etiology and pathogenesis

Numerous theories have been proposed to explain headache associated with exertion, but objective data are lacking in most cases, and the pathophysiology of primary exercise headache remains unclear (15).

Powell proposed strain or stretch of the cervical ligaments and tendons with the development of excessive muscle contraction as an explanation for individuals who complain of headache associated with weightlifting (35).

A hexamethylpropyleneamine oxime SPECT of a 15-year-old with exercise-induced headache revealed transient hypoperfusion in the frontal lobes bilaterally during the headache (03), suggesting changes in cerebral hemodynamics.

Heckmann and colleagues observed abnormal cerebrovascular autoregulation via stress transcranial Doppler in one patient with exercise-induced headache (20). They observed a marked elevation in blood flow velocity with minimal changes in calculated resistance, which they suggested indicates an abnormal vascular myogenic response.

Cerebral arterial vasospasm has been observed in one patient with both primary headache associated with sexual activity and primary exercise headache (39).

Primary exercise headache has been proposed to be a venous disorder (10). The prevalence of jugular valve incompetence leading to a transient increase of intracranial pressure during exertion has been shown to be much higher (70%) in patients with primary exercise headache when compared with controls (20%), suggesting that intracranial venous congestion caused by retrograde jugular venous flow may play a role (09). The prevalence of cerebral venous stenosis has been shown to be increased in patients with exercise-induced headaches in a study that included patients with cough, exercise, and sexual headaches, as compared to controls (11).

Calcitonin gene-related peptide (CGRP) has been established as an important neurotransmitter in the pathogenesis of migraine (12). Calcitonin gene-related peptide has been demonstrated to increase with exercise (25). Although calcitonin gene-related peptide has been hypothesized to play a role in primary exercise headache, the one study that explored this found no difference in calcitonin gene-related peptide rise between runners without a background of headache, migraine sufferers, and runners with primary exercise headache (43).

Differential diagnosis

Associated or underlying disorders

A comorbid diagnosis of migraine is found in one third to two thirds of patients with primary exercise headache (04; 16).

Approximately one third and 17% of patients with primary exercise headache also have a diagnosis of primary headache associated with sexual activity and primary cough headache, respectively. This observation may be due to shared pathophysiology. Cerebral venous stenosis was observed in patients with either primary cough, primary exercise, or primary headache associated with sexual activity (11). However, the pathogeneses of these disorders remain unclear.

An adequate history will usually reveal the diagnosis of primary exercise headache, but patients with mass lesions, structural malformations, and vascular malformations can also have headaches with exertion. About one third of cases of headaches precipitated by exercise are secondary to an underlying pathology (34). Pheochromocytoma, arterial dissection, hypoplasia of the aortic arch after successful coarctation repair, elongated styloid process, and fusiform aneurysms of the vertebral artery, although rare, have been reported as responsible for exercise-induced headache (05; 27; 30). Rarely, exercise headache is a symptom of middle cerebral artery dissection (01). The diagnosis of primary exercise headache can be made only after a thorough examination to rule out intracranial disease or structural malformation.

Cardiac cephalalgia. Rarely, patients with arteriosclerotic coronary artery disease may have exercise-induced headaches that begin with exertion and are relieved by rest. This syndrome was first described in 1997 (26), and 35 cases of cardiac cephalalgia have been reported; these were reviewed in 2017 (47).

Cardiac cephalgia (ECG)

Patient is a 74-year-old woman. ST descent coincidental with severe pulsating headache at rest, which resolved several minutes after nitrites were administered. (Contributed by Dr. Julio Pascual.)

Patients who present with this headache often report symptoms of cardiac ischemia, such as chest pain and dyspnea; however, an absence of these symptoms does not exclude the diagnosis.

This headache is most often a severe bilateral occipital headache with associated nausea in a third of cases (47). Photophobia and phonophobia would be unexpected associated features and each of the symptoms has been reported once in the 35 case reports of cardiac cephalalgia (47).

Cardiac cephalalgia occurring at rest has been described (14; 13).

The International Headache Society defines cardiac cephalalgia as headache usually, but not always, aggravated by exercise, occurring during an episode of myocardial ischemia and relieved by nitroglycerin (19).

Treadmill testing can reveal S-T segment electrocardiographic changes appearing with the headache.

Management of the underlying coronary disease with percutaneous intervention or coronary bypass surgery results in the resolution of headaches (48).

Failure to recognize and correctly diagnose cardiac cephalalgia can have serious consequences. Distinguishing this disorder from migraine without aura is crucial, particularly because vasoconstrictor medications (eg, triptans or ergots) are indicated for the treatment of an acute migraine attack but contraindicated for patients with cardiovascular disease (08).

Table 2. Diagnostic Criteria for Cardiac Cephalalgia

Description: Migraine-like headache, usually but not always aggravated by exercise, occurring during an episode of myocardial ischemia. It is relieved by nitroglycerine.
Diagnostic criteria:
(A) Any headache fulfilling criterion C
(B) Acute myocardial ischemia has been demonstrated
(C) Evidence of causation demonstrated by at least two of the following:
	(1) Headache has significantly worsened in parallel with worsening of the myocardial ischemia
	(2) Either or both or the following:
		(a) Headache has significantly worsened in parallel with worsening of the myocardial ischemia
		(b) Headache has significantly improved or resolved in parallel with improvement in or resolution of the myocardial ischemia
	(3) Headache has at least two of the following characteristics:
		(a) Moderate to severe intensity
	(b) Accompanied by nausea
		(c) Not accompanied by photophobia or phonophobia
		(d) Aggravated by exertion
(D) Headache is relieved by nitroglycerine or derivatives of it
(E) Not better accounted for by another ICDH-3 diagnosis
(19)

References

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Contributors

All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.

Authors

Calvin Chan MBChB

Dr. Chan of King's College London received honorariums from Eli Lilly as a speaker and from AbbVie as an advisory board member.
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Peter J Goadsby MD PhD FRS

Dr. Goadsby of King’s College London received consulting fees from Abbvie, Aeon Biopharma, Amgen, Eli Lilly, Epalex, Lundbeck, Novartis, Pfizer, Sanofi, Satsuma, Shiratronics, and Teva Neurosciences and a grant from Celegene.
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Editor

Stephen D Silberstein MD

Dr. Silberstein, Director of the Jefferson Headache Center at Thomas Jefferson University has no relevant financial relationships to disclose.
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Former Authors

Robert A Davidoff MD
Julio Pascual MD

Patient Profile

Age range of presentation: 13 to 65+ years
Sex preponderance: female>male
Heredity: none
Population groups selectively affected: none selectively affected
Occupation groups selectively affected: none selectively affected

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Primary exercise headache

Differential Diagnosis

brain tumor
mass lesions
middle cerebral artery dissection
intracranial disease
structural malformation at the base of the brain
- vascular malformations
- subarachnoid hemorrhage
- metastatic disease
- cardiac cephalalgia

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Epidemiology of headache
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Migraine: clinical aspects
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Clinical Categories

Headache & Pain

	• Exercise modification may be the only management required to prevent headache onset.
	• Indomethacin can be utilized prophylactically before initiating exercise to prevent headache onset.

Primary exercise headache

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Table 1. Diagnostic Criteria for Primary Exercise Headache

Prognosis and complications

Clinical vignette

Biological basis

Etiology and pathogenesis

Epidemiology

Prevention

Differential diagnosis

Associated or underlying disorders

Table 2. Diagnostic Criteria for Cardiac Cephalalgia

Diagnostic workup

Management

Outcomes

Special considerations

Pregnancy

Anesthesia

Media

References

Contributors

Authors

Editor

Former Authors

Patient Profile

ICD & OMIM codes

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Questions or Comment?

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