- Email: [email protected]
Benign exertional headache
NEUROLOGY/CASE REPORT
Benign Exertional Headache Jason Imperato, MD, MBA Jonathan Burstein, MD Jonathan A. Edlow, MD
We describe 4 patients with benign exertional headache presenting to the emergency department. Consideration of this uncommon cause of headache might facilitate an accurate diagnosis of those patients with headache caused by strenuous exercise.
From the Department of Emergency Medicine, Beth Israel Deaconess Medical Center, Harvard Affiliated Emergency Medicine Residency, Harvard Medical School, Boston, MA.
[Ann Emerg Med. 2003;41:98-103.]
Copyright © 2003 by the American College of Emergency Physicians. 0196-0644/2003/$30.00 + 0 doi:10.1067/mem.2003.21
9 8
ANNALS OF EMERGENCY MEDICINE
41:1
JANUARY 2003
BENIGN EXERTIONAL HEADACHE Imperato, Burstein & Edlow
INTRODUCTION
Patients with headache account for 1% to 2% of visits to the emergency department1-4 and up to 4% of visits to physicians’ offices.5 Although no precise incidence data are available, benign exertional headache is an uncommon cause of head pain. Benign exertional headache can mimic more serious forms of headache, and therefore, investigation of other causes is warranted. We describe 4 patients with benign exertional headache presenting to our ED over a 6-month period. Because there is little description of this disease in the emergency medicine literature,6 our purpose is to familiarize emergency physicians with the common presentation, proposed pathophysiologic characteristics, diagnosis, and treatment of benign exertional headache. CASE REPORTS Patient 1
A 32-year-old man presented to the ED with acute onset of severe occipital headache with a near-syncopal episode while lifting weights a few hours before evaluation. Two days earlier, while lifting weights, the patient had a similar episode that resolved within 20 minutes. This headache, however, was more severe and persisted. He described the headache “like a pop” in the posterior aspect of his head that was so severe that he felt as if he was “going to pass out.” His other complaints included nausea without vomiting. He denied fever, chills, neck pain, photophobia, rash, other neurologic complaints, or previous history of headache. His past medical history was significant for only asthma, for which he used an albuterol metereddose inhaler on an as-needed basis, and he had no allergies. On examination, the patient had a blood pressure of 147/80 mm Hg, a pulse rate of 49 beats/min, a respiratory rate of 16 breaths/min, and a temperature of 36.9°C (98.4°F). The patient was awake, alert, nontoxic appearing, and in moderate distress because of pain. Examination of his head, eyes, ears, nose, and throat
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were unremarkable, and his neck was supple. A complete neurologic examination revealed normal cranial nerves, strength, sensation, gait, and coordination. The remainder of his examination was unremarkable. A noncontrast head computed tomographic (CT) scan showed no evidence of acute intracranial hemorrhage, edema, or mass effect. Lumbar puncture revealed clear and colorless cerebrospinal fluid that had 0 WBCs, 2 RBCs, a protein level of 39 mg/dL, and a glucose level of 61 mg/dL. Fluid Gram stain showed no polymorphonuclear leukocytes and no microorganisms. The patient was discharged home after resolution of the headache with narcotic analgesia. He was given a prescription for oral narcotic analgesics and was instructed to refrain from similar exercises. Subsequent cerebrospinal fluid culture showed no growth of organisms. Patient 2
A 19-year-old man presented to the ED with a severe left occipital headache that began 1 day previously while lifting weights at the gym. He described the sudden onset of a pulsating sensation in the left posterior side of his head that was severe at first but later improved slowly. The pain had nearly resolved at the time of evaluation. He denied fever, chills, neck pain, photophobia, rash, vomiting, other neurologic complaints, or previous history of headache. The patient had no significant medical history, took no medications, and had no allergies. On examination, he had a blood pressure of 121/80 mm Hg, a pulse rate of 90 beats/min, a respiratory rate of 14 breaths/min, and a temperature of 36.7°C (98.1°F). The patient was awake, alert, nontoxic appearing, and in no distress. A complete neurologic examination revealed normal cranial nerves, strength, sensation, gait, and coordination. The remainder of his examination was unremarkable. A noncontrast head CT scan showed no evidence of acute intracranial hemorrhage, edema, or mass effect. An analysis of cerebrospinal fluid revealed clear and colorless fluid without visual xanthochromia with 2 WBCs and 1,180 RBCs in tube 1 and 2 WBCs and 76
9 9
BENIGN EXERTIONAL HEADACHE Imperato, Burstein & Edlow
RBCs in tube 4. Cerebrospinal fluid protein level was 26 mg/dL, and glucose level was 63 mg/dL. Fluid Gram staining showed no polymorphonuclear leukocytes and no microorganisms. The patient was discharged without medications after spontaneous resolution of his headache. Patient 3
A 45-year-old man presented to the ED with a severe, sudden onset, frontotemoral headache that began 1 day previously while performing leg extension exercises at the gym. He stated that while lifting he “heard a pop” on the left side of his head that resulted in a severe 10 of 10 headache. Additionally, he complained of photophobia, blurred vision in the left eye, and nausea without vomiting. He was evaluated at a community hospital shortly after the headache began. He had a head CT scan with results that he reported as negative; however, he refused a lumbar puncture when recommended by the evaluating physician. He presented to our ED with complaints of persistent headache despite acetaminophen/oxycodone (Percocet) that had reduced his headache from a 10 of 10 to a 4 of 10. He denied fever, chills, neck stiffness, and other neurologic complaints. He reported similar symptoms 3 times in the past while lifting heavy weights but did not seek medical attention for those complaints. His past medical history was significant for untreated hypertension and cholelithiasis. His medications were limited to the acetaminophen/oxycodone prescribed the day before, and he had no allergies. On examination, he had a blood pressure of 153/86 mm Hg, a pulse rate of 81 beats/min, a respiratory rate of 18 breaths/min, and a temperature of 36.4°C (97.5°F). The patient was awake, alert, and in moderate distress caused by pain. His visual acuity was normal bilaterally, extraocular eye movements were intact, visual field testing was normal, and fundoscopic examination revealed sharp optic discs and venous pulsations. His neck was supple, and a complete neurologic examination showed normal cranial nerves, strength, sensation, gait, and coordination. The remainder of his examination was unremarkable.
1 0 0
Lumbar puncture revealed 1 WBC and 1 RBC, with a protein level of 34 mg/dL and a glucose level of 61 mg/ dL. Fluid Gram staining showed no polymorphonuclear leukocytes and no microorganisms. The patient was given intravenous narcotics that resulted in resolution of the headache, and he was discharged with prescriptions for narcotic analgesics. Subsequent cerebrospinal fluid culture showed no growth of organisms. Patient 4
A 19-year-old man presented to the ED as a referral from his primary care physician with a severe headache for 2 days. He stated that 1 day previously he was at the gym performing a leg press exercise when he noted the sudden onset of headache. He described the pain as a constant pressure in the occipital area of his head that was “the worst headache” of his life. The headache subsequently had migrated to cause pain in his entire head. The pain was a 10 of 10 initially but was a 2 of 10 at presentation after taking acetaminophen/caffeine (Excedrin Migraine) at home, which provided minimal relief of symptoms. His other complaints included lightheadedness and nausea without vomiting. He denied fever, chills, neck pain, photophobia, rash, other neurologic complaints, or previous history of headache. The patient had no significant past medical history, took no medications, and had no allergies. On examination, he had a blood pressure of 152/68 mm Hg, a pulse rate of 80 beats/min, a respiratory rate of 14 breaths/min, and a temperature of 36.4°C (97.6°F). The patient was alert and nontoxic appearing. Examination of his head, eyes, ears, nose, and throat was unremarkable, and his neck was supple. A complete neurologic examination revealed normal cranial nerves, strength, sensation, gait, and coordination. The remainder of his examination was normal. A noncontrast head CT scan showed no evidence of acute intracranial hemorrhage, edema, or mass effect. Lumbar puncture revealed clear and colorless cerebrospinal fluid that had 1 WBC and 2 RBCs. Fluid Gram staining showed no polymorphonuclear leukocytes and no microorganisms.
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BENIGN EXERTIONAL HEADACHE Imperato, Burstein & Edlow
The patient was subsequently discharged home after complete resolution of the headache with narcotic analgesia. He was instructed to refrain from strenuous exercise and to take acetaminophen (Tylenol) for return of his symptoms. Subsequent cerebrospinal fluid culture revealed no growth of organisms. DISCUSSION
Benign exertional headache should be included in the differential diagnosis of patients presenting to the ED with acute onset of severe headache. Our recent experience with 4 patients over a 6-month period underscores the need for emergency physicians to be familiar with this entity. An association between headache and exercise was first noted by Hippocrates, who, in 450 BC, wrote, “… one should be able to recognize those who have headaches from gymnastic exercises or running or walking or hunting or any other reasonable labour or from immoderate venery.”7,8 In 1932, Tinel9 described 4 patients with an intermittent paroxysmal headache that occurred only with effort, which he called “la cephalee a l’effort.”10 In the first English-language description, Sir Charles Symonds described 27 similar patients with transient severe head pain provoked by coughing, sneezing, straining at stool, laughing, or stooping, which he called “cough headache.”9,11 The term benign exertional headache was first proposed by Rooke12 in 1968. The syndrome included any sudden-onset headache precipitated by exertion that persists for seconds to hours and is not associated with a structural brain lesion. Since the time of Rooke, the entity has only been described in case reports, and the only such similar report in the emergency medicine literature was in 1982.6 Clues to the diagnosis include abrupt onset of severe headache lasting minutes to hours and precipitated by physical exertion.7,9,12-15 The International Headache Society classification of benign exertional headache is depicted in the Figure.7 The entity is twice as common in patients older than 40 years than in younger patients and 4 times more
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common in men than in women.9 The pain is typically described as severe in intensity, having a bursting, explosive, or splitting quality and might be throbbing in nature. The pain typically lasts seconds to hours, and patients are normally free of pain between attacks. It is bilateral in 65% of cases.9 The headache usually begins immediately or within seconds after the precipitating event. Precipitants are mostly Valsalva-type maneuvers and can include heavy lifting, swimming, coughing, sneezing, straining at stool, singing, or orgasm. The headache might be associated with nausea, vomiting, or photophobia, but these findings are nonspecific.9,16 The result of complete neurologic examination in these patients is normal. Several theories in regard to the cause of benign exertional headache have been proposed, but none has been proved. Most investigators believe that these headaches are vascular in origin. According to one theory, they occur because exertion increases cerebral arterial pressure, causing the pain-sensitive venous sinuses at the base of the brain to dilate.13 Additionally, studies of weight lifters demonstrate that with maximal lifting, systolic blood pressure might reach levels of greater than 400 mm Hg and diastolic pressures might reach levels of greater than 300 mm Hg.17 Furthermore, angiographic studies in patients with these headaches have demonstrated vasospasm.18 The throbbing nature of these headaches combined with these findings supports the vascular nature of benign exertional headache.13
Figure.
International Headache Society classification of benign exertional headache. 1—The headache is specifically brought on by physical exercise. 2—The headache is bilateral, throbbing in nature at onset, and might develop migrainous features in those patients susceptible to migraine. 3—The headache only lasts from 5 minutes to 24 hours. 4—The headache is prevented by avoiding excessive exertion, particularly in hot weather or at high altitude. 5—The headache is not associated with any systemic intracranial disorder.
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BENIGN EXERTIONAL HEADACHE Imperato, Burstein & Edlow
The diagnosis of benign exertional headache should be suspected on clinical grounds. The differential diagnosis includes benign forms of headache, such as cough headache, sexual or orgasmic cephalgia, and benign thunderclap headache.9,16,19-21 However, because the differential diagnosis of abrupt onset of severe headache also includes more serious causes, such as subarachnoid hemorrhage, intraparencyhmal hemorrhage, subdural and epidural hematoma, cerebral venous sinus thrombosis, meningitis, and tumor, further emergency workup is warranted. In fact, 2 studies have shown that, of patients with acute severe headache and normal neurologic examination, approximately 15% will have a subarachnoid hemorrhage.22,23 There is no way on the basis of history or physical examination to distinguish benign exertional headaches and other more serious pathologies. As a result, appropriate investigation includes a noncontrast CT scan of the head followed by lumbar puncture to rule out these more serious causes. Opening pressure measurements were not performed in any of the patients described in this case series. However, it should be emphasized that a lumbar puncture should include measurement of an opening pressure. Such measurement might allow the clinician to discover causes of headache in the setting of a normal CT scan of the head and negative cerebrospinal fluid analysis. These entities include pseudotumor cerebri and cerebral venous sinus thrombosis, which might manifest with high opening pressures, or spontaneous intracranial hypotension, which might manifest with low pressure. Rare patients with acute, severe headache with normal pressures and cerebrospinal fluid analysis could have vertebral dissection or symptomatic unruptured cerebral aneurysms and might require further evaluation. In Rooke’s study,12 103 patients with the complaint of exertional headache but without detectable organic intracranial disease were followed up for at least 3 years. Of these, 93 had benign exertional headache that was never associated with significant intracranial lesions. However, a lesion was identified through operation or reoentgenography in 10 patients over time, including Arnold-Chiari deformity, subdural hematoma, platyba-
1 0 2
sia, tumor, and basilar impression. However, because this study was published before the availability of CT scanning, we believe that it is highly likely that these lesions would have been identified at the time of initial presentation had such technology been available. In a later study, when neuroimaging tests were more readily available, Pascual et al16 investigated 28 patients with headaches precipitated by physical exercise. These patients represented referrals from general practitioners to neurologists after the cause of those patients’ headaches was unclear. After further investigation, 16 of the 28 met criteria for benign exertional headache. However, 12 of those patients with headache caused by physical exercise were found to have symptomatic exertional headaches. The causes of these headaches were found to be subarachnoid hemorrhage in 10 patients, pansinusitis in 1 patient, and multiple brain metastasis in 1 patient. This study underscores the need for serious investigation as to the potential cause for headaches brought on by exertion because they can be classified as benign only after intracranial pathology has been excluded. Furthermore, it emphasizes the need for the emergency physician to arrange follow-up with the primary medical physician or headache specialist after treating patients for a presumed benign exertional headache. The treatment of benign exertional headache is even less well studied. The prophylactic treatments of choice are currently considered avoidance or reduction of the precipitating factor. Although several pharmacologic therapies have been proposed, indomethacin 3 times daily is most frequently suggested in the literature. However, no controlled clinical trials have been done to support this point, and therefore, the best treatment for this type of headache remains unknown. All 4 of our patients were discharged from the ED on resolution of their symptoms. Patient 1 remained asymptomatic 4 months after his original diagnosis, and patient 3 was without headache at follow-up 1 month after presentation. Patient 2, however, returned to the ED 2 days after the initial evaluation with worsening headache on standing. He was given a diagnosis of postlumbar puncture headache and was treated with
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crystalloid intravenous hydration and intravenous caffeine with complete resolution of headache. He remained asymptomatic at follow-up after 2 months. Follow-up was not available for patient 4. Benign exertional headache, although uncommon, should be considered in patients with headache. We believe that, although the investigation of more severe forms of headache is essential, consideration of the disease entity might help alleviate the concerns of patients with severe headache caused by exertion.
19. Paulson GW, Klawans HL. Benign orgasmic cephalgia. Headache. 1974;13:181-187. 20. Porter M, Jankovic J. Benign coital cephalalgia. Arch Neurol. 1981;38:710-712. 21. Lance JW. Headaches related to sexual activity. J Neurol Neurosurg Psychiatry. 1976;39:1226-1230. 22. Morgenstern LB, Luna-Gonzales H, Huber JC Jr, et al. Worst headache and subarachnoid hemorrhage: prospective modern computed tomograpy and spinal fluid analysis. Ann Emerg Med. 1998;32:297-304. 23. Linn FH, Wijdicks EF, van der Graff Y, et al. Prospective study of sentinel headache in aneurysmal subarachnoid haemorrhage. Lancet. 1994;344:590-593.
Received for publication May 20, 2002. Revision received July 30, 2002. Accepted for publication August 9, 2002. Reprints not available from the authors. Address for correspondence: Jonathan A. Edlow, MD, Department of Emergency Medicine, Beth Israel Deaconess Medical Center, One Deaconess Road, Boston, MA 02215; 617-754-2329, fax 617-754-2350; E-mail [email protected].
REFERENCES 1. Dhopesh V, Anwar R, Herring C. A retrospective assessment of emergency department patients with complaint of headache. Headache. 1979;19:37-42. 2. Evans RW. Diagnostic testing for the evaluation of headaches. Neurol Clin. 1996;14:1-26. 3. Leicht MJ. Non-traumatic headache in the emergency department. Ann Emerg Med. 1980;9:404-409. 4. Ramirez-Lassepas M, Espinosa CE, Cicero JJ, et al. Predictors of intracranial pathologic findings in patients who seek emergency care because of headache. Arch Neurol. 1997;54:1506-1509. 5. Frishberg BM. The utility of neuroimaging in the evaluation of headache in patients with normal neurologic examinations. Neurology. 1994;44:1191-1197. 6.
Powell B. Weight lifter’s cephalgia. Ann Emerg Med. 1982;11:449-451.
7.
McCrory P. Headaches and exercise. Sports Med. 2000;30:221-229.
8.
Hippocrates. Hippocratic Writings. London, United Kingdom: Penguin Books; 1950.
9. Tinel J. Un syndrome d’algie veineuse intracranienne. La cephalee a l’effort. Prat Med Fr. 1932;13:113-119. 10. Sands GH, Newman L, Lipton R. Cough, exertional, and other miscellaneous headaches. Med Clin North Am. 1991;75:733-747. 11. Symonds C. Cough headache. Brain. 1956;79:557-568. 12. Rooke D. Benign exertional headache. Med Clin North Am. 1968;52:801-808. 13. McCrory P. Recognizing exercise-related headache. Physician Sports Med. 1997;25:33-43. 14. Perry WJ. Exertional headache. Physician Sports Med. 1985;13:95-98. 15. Paulson GW. Weightlifters headache. Headache. 1983;23:193-194. 16. Pascual J, Iglesias F, Oterino A, et al. Cough, exertional, and sexual headaches. Neurology. 1996;46:1520-1524. 17. MacDougall JD, Tuxen D, Sale DG, et al. Arterial blood pressure response to heavy resistance exercise. J Appl Physiol. 1985;58:785-790. 18. Silbert PL, Hankey GJ, Prentice DA, et al. Angiographically demonstrated arterial spasm in a case of benign sexual headache and benign exertional headace. Aust N Z J Med. 1989;19:466-468.
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Benign Exertional Headache Jason Imperato, MD, MBA Jonathan Burstein, MD Jonathan A. Edlow, MD
We describe 4 patients with benign exertional headache presenting to the emergency department. Consideration of this uncommon cause of headache might facilitate an accurate diagnosis of those patients with headache caused by strenuous exercise.
From the Department of Emergency Medicine, Beth Israel Deaconess Medical Center, Harvard Affiliated Emergency Medicine Residency, Harvard Medical School, Boston, MA.
[Ann Emerg Med. 2003;41:98-103.]
Copyright © 2003 by the American College of Emergency Physicians. 0196-0644/2003/$30.00 + 0 doi:10.1067/mem.2003.21
9 8
ANNALS OF EMERGENCY MEDICINE
41:1
JANUARY 2003
BENIGN EXERTIONAL HEADACHE Imperato, Burstein & Edlow
INTRODUCTION
Patients with headache account for 1% to 2% of visits to the emergency department1-4 and up to 4% of visits to physicians’ offices.5 Although no precise incidence data are available, benign exertional headache is an uncommon cause of head pain. Benign exertional headache can mimic more serious forms of headache, and therefore, investigation of other causes is warranted. We describe 4 patients with benign exertional headache presenting to our ED over a 6-month period. Because there is little description of this disease in the emergency medicine literature,6 our purpose is to familiarize emergency physicians with the common presentation, proposed pathophysiologic characteristics, diagnosis, and treatment of benign exertional headache. CASE REPORTS Patient 1
A 32-year-old man presented to the ED with acute onset of severe occipital headache with a near-syncopal episode while lifting weights a few hours before evaluation. Two days earlier, while lifting weights, the patient had a similar episode that resolved within 20 minutes. This headache, however, was more severe and persisted. He described the headache “like a pop” in the posterior aspect of his head that was so severe that he felt as if he was “going to pass out.” His other complaints included nausea without vomiting. He denied fever, chills, neck pain, photophobia, rash, other neurologic complaints, or previous history of headache. His past medical history was significant for only asthma, for which he used an albuterol metereddose inhaler on an as-needed basis, and he had no allergies. On examination, the patient had a blood pressure of 147/80 mm Hg, a pulse rate of 49 beats/min, a respiratory rate of 16 breaths/min, and a temperature of 36.9°C (98.4°F). The patient was awake, alert, nontoxic appearing, and in moderate distress because of pain. Examination of his head, eyes, ears, nose, and throat
JANUARY 2003
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were unremarkable, and his neck was supple. A complete neurologic examination revealed normal cranial nerves, strength, sensation, gait, and coordination. The remainder of his examination was unremarkable. A noncontrast head computed tomographic (CT) scan showed no evidence of acute intracranial hemorrhage, edema, or mass effect. Lumbar puncture revealed clear and colorless cerebrospinal fluid that had 0 WBCs, 2 RBCs, a protein level of 39 mg/dL, and a glucose level of 61 mg/dL. Fluid Gram stain showed no polymorphonuclear leukocytes and no microorganisms. The patient was discharged home after resolution of the headache with narcotic analgesia. He was given a prescription for oral narcotic analgesics and was instructed to refrain from similar exercises. Subsequent cerebrospinal fluid culture showed no growth of organisms. Patient 2
A 19-year-old man presented to the ED with a severe left occipital headache that began 1 day previously while lifting weights at the gym. He described the sudden onset of a pulsating sensation in the left posterior side of his head that was severe at first but later improved slowly. The pain had nearly resolved at the time of evaluation. He denied fever, chills, neck pain, photophobia, rash, vomiting, other neurologic complaints, or previous history of headache. The patient had no significant medical history, took no medications, and had no allergies. On examination, he had a blood pressure of 121/80 mm Hg, a pulse rate of 90 beats/min, a respiratory rate of 14 breaths/min, and a temperature of 36.7°C (98.1°F). The patient was awake, alert, nontoxic appearing, and in no distress. A complete neurologic examination revealed normal cranial nerves, strength, sensation, gait, and coordination. The remainder of his examination was unremarkable. A noncontrast head CT scan showed no evidence of acute intracranial hemorrhage, edema, or mass effect. An analysis of cerebrospinal fluid revealed clear and colorless fluid without visual xanthochromia with 2 WBCs and 1,180 RBCs in tube 1 and 2 WBCs and 76
9 9
BENIGN EXERTIONAL HEADACHE Imperato, Burstein & Edlow
RBCs in tube 4. Cerebrospinal fluid protein level was 26 mg/dL, and glucose level was 63 mg/dL. Fluid Gram staining showed no polymorphonuclear leukocytes and no microorganisms. The patient was discharged without medications after spontaneous resolution of his headache. Patient 3
A 45-year-old man presented to the ED with a severe, sudden onset, frontotemoral headache that began 1 day previously while performing leg extension exercises at the gym. He stated that while lifting he “heard a pop” on the left side of his head that resulted in a severe 10 of 10 headache. Additionally, he complained of photophobia, blurred vision in the left eye, and nausea without vomiting. He was evaluated at a community hospital shortly after the headache began. He had a head CT scan with results that he reported as negative; however, he refused a lumbar puncture when recommended by the evaluating physician. He presented to our ED with complaints of persistent headache despite acetaminophen/oxycodone (Percocet) that had reduced his headache from a 10 of 10 to a 4 of 10. He denied fever, chills, neck stiffness, and other neurologic complaints. He reported similar symptoms 3 times in the past while lifting heavy weights but did not seek medical attention for those complaints. His past medical history was significant for untreated hypertension and cholelithiasis. His medications were limited to the acetaminophen/oxycodone prescribed the day before, and he had no allergies. On examination, he had a blood pressure of 153/86 mm Hg, a pulse rate of 81 beats/min, a respiratory rate of 18 breaths/min, and a temperature of 36.4°C (97.5°F). The patient was awake, alert, and in moderate distress caused by pain. His visual acuity was normal bilaterally, extraocular eye movements were intact, visual field testing was normal, and fundoscopic examination revealed sharp optic discs and venous pulsations. His neck was supple, and a complete neurologic examination showed normal cranial nerves, strength, sensation, gait, and coordination. The remainder of his examination was unremarkable.
1 0 0
Lumbar puncture revealed 1 WBC and 1 RBC, with a protein level of 34 mg/dL and a glucose level of 61 mg/ dL. Fluid Gram staining showed no polymorphonuclear leukocytes and no microorganisms. The patient was given intravenous narcotics that resulted in resolution of the headache, and he was discharged with prescriptions for narcotic analgesics. Subsequent cerebrospinal fluid culture showed no growth of organisms. Patient 4
A 19-year-old man presented to the ED as a referral from his primary care physician with a severe headache for 2 days. He stated that 1 day previously he was at the gym performing a leg press exercise when he noted the sudden onset of headache. He described the pain as a constant pressure in the occipital area of his head that was “the worst headache” of his life. The headache subsequently had migrated to cause pain in his entire head. The pain was a 10 of 10 initially but was a 2 of 10 at presentation after taking acetaminophen/caffeine (Excedrin Migraine) at home, which provided minimal relief of symptoms. His other complaints included lightheadedness and nausea without vomiting. He denied fever, chills, neck pain, photophobia, rash, other neurologic complaints, or previous history of headache. The patient had no significant past medical history, took no medications, and had no allergies. On examination, he had a blood pressure of 152/68 mm Hg, a pulse rate of 80 beats/min, a respiratory rate of 14 breaths/min, and a temperature of 36.4°C (97.6°F). The patient was alert and nontoxic appearing. Examination of his head, eyes, ears, nose, and throat was unremarkable, and his neck was supple. A complete neurologic examination revealed normal cranial nerves, strength, sensation, gait, and coordination. The remainder of his examination was normal. A noncontrast head CT scan showed no evidence of acute intracranial hemorrhage, edema, or mass effect. Lumbar puncture revealed clear and colorless cerebrospinal fluid that had 1 WBC and 2 RBCs. Fluid Gram staining showed no polymorphonuclear leukocytes and no microorganisms.
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BENIGN EXERTIONAL HEADACHE Imperato, Burstein & Edlow
The patient was subsequently discharged home after complete resolution of the headache with narcotic analgesia. He was instructed to refrain from strenuous exercise and to take acetaminophen (Tylenol) for return of his symptoms. Subsequent cerebrospinal fluid culture revealed no growth of organisms. DISCUSSION
Benign exertional headache should be included in the differential diagnosis of patients presenting to the ED with acute onset of severe headache. Our recent experience with 4 patients over a 6-month period underscores the need for emergency physicians to be familiar with this entity. An association between headache and exercise was first noted by Hippocrates, who, in 450 BC, wrote, “… one should be able to recognize those who have headaches from gymnastic exercises or running or walking or hunting or any other reasonable labour or from immoderate venery.”7,8 In 1932, Tinel9 described 4 patients with an intermittent paroxysmal headache that occurred only with effort, which he called “la cephalee a l’effort.”10 In the first English-language description, Sir Charles Symonds described 27 similar patients with transient severe head pain provoked by coughing, sneezing, straining at stool, laughing, or stooping, which he called “cough headache.”9,11 The term benign exertional headache was first proposed by Rooke12 in 1968. The syndrome included any sudden-onset headache precipitated by exertion that persists for seconds to hours and is not associated with a structural brain lesion. Since the time of Rooke, the entity has only been described in case reports, and the only such similar report in the emergency medicine literature was in 1982.6 Clues to the diagnosis include abrupt onset of severe headache lasting minutes to hours and precipitated by physical exertion.7,9,12-15 The International Headache Society classification of benign exertional headache is depicted in the Figure.7 The entity is twice as common in patients older than 40 years than in younger patients and 4 times more
JANUARY 2003
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ANNALS OF EMERGENCY MEDICINE
common in men than in women.9 The pain is typically described as severe in intensity, having a bursting, explosive, or splitting quality and might be throbbing in nature. The pain typically lasts seconds to hours, and patients are normally free of pain between attacks. It is bilateral in 65% of cases.9 The headache usually begins immediately or within seconds after the precipitating event. Precipitants are mostly Valsalva-type maneuvers and can include heavy lifting, swimming, coughing, sneezing, straining at stool, singing, or orgasm. The headache might be associated with nausea, vomiting, or photophobia, but these findings are nonspecific.9,16 The result of complete neurologic examination in these patients is normal. Several theories in regard to the cause of benign exertional headache have been proposed, but none has been proved. Most investigators believe that these headaches are vascular in origin. According to one theory, they occur because exertion increases cerebral arterial pressure, causing the pain-sensitive venous sinuses at the base of the brain to dilate.13 Additionally, studies of weight lifters demonstrate that with maximal lifting, systolic blood pressure might reach levels of greater than 400 mm Hg and diastolic pressures might reach levels of greater than 300 mm Hg.17 Furthermore, angiographic studies in patients with these headaches have demonstrated vasospasm.18 The throbbing nature of these headaches combined with these findings supports the vascular nature of benign exertional headache.13
Figure.
International Headache Society classification of benign exertional headache. 1—The headache is specifically brought on by physical exercise. 2—The headache is bilateral, throbbing in nature at onset, and might develop migrainous features in those patients susceptible to migraine. 3—The headache only lasts from 5 minutes to 24 hours. 4—The headache is prevented by avoiding excessive exertion, particularly in hot weather or at high altitude. 5—The headache is not associated with any systemic intracranial disorder.
1 0 1
BENIGN EXERTIONAL HEADACHE Imperato, Burstein & Edlow
The diagnosis of benign exertional headache should be suspected on clinical grounds. The differential diagnosis includes benign forms of headache, such as cough headache, sexual or orgasmic cephalgia, and benign thunderclap headache.9,16,19-21 However, because the differential diagnosis of abrupt onset of severe headache also includes more serious causes, such as subarachnoid hemorrhage, intraparencyhmal hemorrhage, subdural and epidural hematoma, cerebral venous sinus thrombosis, meningitis, and tumor, further emergency workup is warranted. In fact, 2 studies have shown that, of patients with acute severe headache and normal neurologic examination, approximately 15% will have a subarachnoid hemorrhage.22,23 There is no way on the basis of history or physical examination to distinguish benign exertional headaches and other more serious pathologies. As a result, appropriate investigation includes a noncontrast CT scan of the head followed by lumbar puncture to rule out these more serious causes. Opening pressure measurements were not performed in any of the patients described in this case series. However, it should be emphasized that a lumbar puncture should include measurement of an opening pressure. Such measurement might allow the clinician to discover causes of headache in the setting of a normal CT scan of the head and negative cerebrospinal fluid analysis. These entities include pseudotumor cerebri and cerebral venous sinus thrombosis, which might manifest with high opening pressures, or spontaneous intracranial hypotension, which might manifest with low pressure. Rare patients with acute, severe headache with normal pressures and cerebrospinal fluid analysis could have vertebral dissection or symptomatic unruptured cerebral aneurysms and might require further evaluation. In Rooke’s study,12 103 patients with the complaint of exertional headache but without detectable organic intracranial disease were followed up for at least 3 years. Of these, 93 had benign exertional headache that was never associated with significant intracranial lesions. However, a lesion was identified through operation or reoentgenography in 10 patients over time, including Arnold-Chiari deformity, subdural hematoma, platyba-
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sia, tumor, and basilar impression. However, because this study was published before the availability of CT scanning, we believe that it is highly likely that these lesions would have been identified at the time of initial presentation had such technology been available. In a later study, when neuroimaging tests were more readily available, Pascual et al16 investigated 28 patients with headaches precipitated by physical exercise. These patients represented referrals from general practitioners to neurologists after the cause of those patients’ headaches was unclear. After further investigation, 16 of the 28 met criteria for benign exertional headache. However, 12 of those patients with headache caused by physical exercise were found to have symptomatic exertional headaches. The causes of these headaches were found to be subarachnoid hemorrhage in 10 patients, pansinusitis in 1 patient, and multiple brain metastasis in 1 patient. This study underscores the need for serious investigation as to the potential cause for headaches brought on by exertion because they can be classified as benign only after intracranial pathology has been excluded. Furthermore, it emphasizes the need for the emergency physician to arrange follow-up with the primary medical physician or headache specialist after treating patients for a presumed benign exertional headache. The treatment of benign exertional headache is even less well studied. The prophylactic treatments of choice are currently considered avoidance or reduction of the precipitating factor. Although several pharmacologic therapies have been proposed, indomethacin 3 times daily is most frequently suggested in the literature. However, no controlled clinical trials have been done to support this point, and therefore, the best treatment for this type of headache remains unknown. All 4 of our patients were discharged from the ED on resolution of their symptoms. Patient 1 remained asymptomatic 4 months after his original diagnosis, and patient 3 was without headache at follow-up 1 month after presentation. Patient 2, however, returned to the ED 2 days after the initial evaluation with worsening headache on standing. He was given a diagnosis of postlumbar puncture headache and was treated with
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crystalloid intravenous hydration and intravenous caffeine with complete resolution of headache. He remained asymptomatic at follow-up after 2 months. Follow-up was not available for patient 4. Benign exertional headache, although uncommon, should be considered in patients with headache. We believe that, although the investigation of more severe forms of headache is essential, consideration of the disease entity might help alleviate the concerns of patients with severe headache caused by exertion.
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Received for publication May 20, 2002. Revision received July 30, 2002. Accepted for publication August 9, 2002. Reprints not available from the authors. Address for correspondence: Jonathan A. Edlow, MD, Department of Emergency Medicine, Beth Israel Deaconess Medical Center, One Deaconess Road, Boston, MA 02215; 617-754-2329, fax 617-754-2350; E-mail [email protected].
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