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Cardiac cephalgia:

A treatable form of exertional headache


R.B. Lipton, MD; T. Lowenkopf, MD; Z.H. Bajwa, MD; R.S. Leckie, MD; S. Ribeiro, MD; L.C. Newman, MD;
and M.A. Greenberg, MD

Article abstract-We report two patients with exertional headaches beginning with vigorous exercise and relieved by
rest. Neurologic evaluation and neuroimaging were normal in both. During exercise stress testing, the onset of the
patients’ typical headaches correlated with ECG changes indicative of myocardial ischemia. In both patients coronary
angiography revealed three-vessel disease, and myocardial revascularization procedures were followed by complete reso-
lution of headaches. Based on these patients, and a review of prior similar reports, we conclude that myocardial ischemia
is a rare and treatable cause of exertional headache. Accurate diagnosis is critical to controlling headaches and preventing
myocardial infarction.
NEUROLOGY 1997;49:813-816

The majority of headaches associated with exertion trast CT and MRI of the head, both of which were
are benign. Within the International Headache Soci- normal. Two weeks later he again developed the
ety classification, most exertional headache can be rapid onset of severe headache with exertion. He
categorized as “miscellaneous headaches unassoci- consulted a second neurologist who performed a lum-
ated with a structural lesion” (reference 1, p. 39).l bar puncture to rule out subarachnoid hemorrhage;
Subtypes include benign cough headache, benign ex- CSF was normal. The patient’s internist sent him for
ertional headache, and headaches associated with pulmonary function tests, which were also normal. A
sexual activity.l Exertional headaches due to an or- resting ECG revealed flattened T waves in V, and
ganic cause may occur in patients with intracranial AVL, an inverted T wave in lead V,, and biphasic T
structural p a t h o l ~ g y .We
~ - ~report two unusual cases waves in V3,4,6.No specific diagnosis was made.
of cardiac ischemia presenting as an exertional head- The patient’s exertional headaches persisted and
ache and review prior published reports. he sought a third neurologic opinion at our center.
To assess the possibility that cardiac ischemia
Patient 1. A 57-year-old man presented with a caused his headaches, we referred him for a exercise
4-month history of exertional headaches beginning 5 stress-thallium test. In stage I1 he developed the
to 10 minutes after the onset of vigorous exercise onset of his typical exertional headache accompanied
and increasing in severity over several minutes. The by ST-segment depression in leads 11, 111, AVF, and
pain was maximum at the vertex, sharp or shooting V4-6. A severe reversible perfusion defect was seen
in quality, and rated as 10 (of 10) in severity. The in the mid and distal anterior wall extending to the
pain persisted when he walked or swam, gradually apex. The termination of exercise was followed by
subsiding over minutes to hours when exercise was the rapid simultaneous resolution of the headache
terminated. The headache also occurred with sexual and ECG changes. The patient was started on dilti-
activity. The pain was accompanied by nausea but azem 30 mg four times a day and aspirin 80 mg
not vomiting. There was no aura, photophobia, or every day with some decrease in his exertional head-
phonophobia. Headaches were not provoked by aches. Coronary angiography revealed severe three-
coughing, sneezing, or straining with bowel move- vessel disease with hypokinesis and hypertrophy of
ments. On a few occasions they were associated with the wall of the left ventricle. Following three-vessel
vague abdominal or chest discomfort but not with coronary artery bypass surgery, his resting ECG nor-
palpitations, dizziness, or diaphoresis. malized and he has returned to his usual exercise
His history was negative for hypertension, diabe- program without exertional headache.
tes, and heart disease. He stopped smoking 1 year
prior to evaluation. Family history was positive for Patient 2. A 67-year-old man presented with a
coronary artery disease in both parents. 9-month history of exertional headache beginning 10
The patient presented to a neurologist with the to 15 minutes after the onset of strenuous exercise,
described history. Initial workup included noncon- including brisk walking. The headaches consisted of
From the Department of Neurology (Drs. Lipton, Lowenkopf, Ribeiro. and Newman) and Medicine (Dr. Greenberg), Albert Einstein College of Medicine, The
Headache Unit, Montefiore Medical Center (Drs. Lipton, Lowenkopf, Ribeiro, and Newman), Bronx, NY: and the Department of Neurology (Dr. Bajwal and
Anesthesiology (Drs. Bajwa and Leckie), Beth Israel Hospital, Harvard Medical School, Boston, MA.
Received September 26, 1996. Accepted on final form March 1. 1997.
Address correspondence and reprint requests to Dr. Richard B. Lipton, Department of Neurologymeadache Unit, Montefiore Medical Center, 111 E. 210th
Street, Bronx, NY 10467.
Copyright 0 1997 by the American Academy of Neurology 813
a bifrontal, squeezing, steady pressure that in- algia” for this disorder. In both our patients, and in
creased with continuous exercise to a rating of 10 (of several earlier reports, the diagnosis was delayed by
10) in severity. Pain gradually resolved over minutes a failure to recognize cardiac ischemia as a cause of
to an hour after the termination of exercise. The headache.
headaches were not associated with nausea, vomit- As exertional headache has a broad differential,
ing, aura, photophobia, phonophobia, abdominal or which includes both secondary and primary head-
chest discomfort, palpitations, dizziness, diaphoresis, ache syndromes, the evaluation of exertional head-
or shortness of breath. They were not provoked by ache should seek to identify or exclude serious treat-
coughing, sneezing, or straining with bowel move- able etiologies. In general, patients with exertional
ments. The patient also reported the onset of bilat- headache require neuroimaging to rule out struc-
eral calf claudication with walking and stair climb- tural disease. Magnetic resonance imaging is the
ing. preferred imaging modality as pathology in exer-
His history was negative for diabetes, heart dis- tional headache tends to be in the posterior fossa.l0
ease, and hypercholesteremia. He stopped smoking Sands et a1.l0 reviewed 233 published cases of exer-
30 years prior to presentation and had been physi- tional or cough headache and found that 22 had
cally active his entire adult life. For the past year he structural disease. Pascual et aL4 reviewed 28 cases
took nifedipine 30 mg twice a day, metoprolol 50 mg of exercise headache and found 10 with subarach-
twice a day, and enalapril 10 mg twice a day for noid hemorrhage, one with brain metastases, and
hypertension, as well as aspirin 80 mg every day. one with pansinusitis. If the exertional headaches
His family history was positive for diabetes, coronary are of recent onset or if the pain is paroxysmal, se-
artery disease, peripheral vascular disease, and vere, or long-lasting lumbar puncture is required to
cancer. rule out subarachnoid hemorrhage. Occasionally,
His family physician ordered noncontrast CT of pseudotumor cerebri,ll cortical vein thrombosis, or
the head and noninvasive vascular studies of his sagittal sinus thrombosis may present as exertional
lower extremities; both studies were normal. A neu- headache.12 Once secondary causes are excluded, a
rologic consultation did not identify a cause for head- specific primary headache disorder should be diag-
ache and the patient was referred for a cardiac eval- nosed.
uation. His cardiac exam was normal but his ECG The primary headache disorders in the differen-
showed sinus bradycardia and a possible old inferior tial diagnosis of cardiac cephalgia include benign ex-
wall myocardial infarct. During his cardiac exercise ertional headache, benign cough headache, and
stress-thallium test he developed his typical bifron- headache associated with sexual activity.l Exertional
tal headache with simultaneous inferolateral ST- headache caused by angina should be relatively easy
segment depression and a small area of inferolateral to differentiate from benign cough headache, which
reversible hypoperfusion. The patient was instructed is of sudden onset, generally lasts less than 1
to take sublingual nitroglycerin prior to exercise. He minute, and is usually prevented by ind0metha~in.l~
found that this regimen prevented his exertional Raskin13 demonstrated that this disorder also remits
headache. On a repeat stress test he again developed with lumbar puncture. Benign exertional headache
his typical bifrontal headache with inferolateral ST- is potentially more problematic, as the headache
segment depression on ECG. The headache and ECG may last from 5 minutes to 24 hours. Patients at risk
changes simultaneously reversed with the adminis- for cardiac cephalgia may require a cardiac workup
tration of nitroglycerin and the termination of exer- as discussed later. Exertional migraine could present
cise. A coronary angiogram revealed three-vessel dis- a diagnostic difficulty in that typical migraine may
ease with 90% left circumflex artery stenosis. The begin after a period of strenuous exercise.14
patient underwent rotational atherectomy and per- Distinguishing cardiac cephalgia from migraine is
cutaneous transluminal coronary angioplasty. A re- of considerable importance. Both disorders can pro-
peat stress-thallium test 1month after the procedure duce severe head pain accompanied by nausea and
failed to reproduce headache or ECG changes consis- both can be triggered by exertion.14Vasoconstrictors
tent with ischemia. The patient continues to exercise (sumatriptan, ergotarnine, and dihydroergotamine)
regularly without exertional headache. are indicated in the treatment of migraine but con-
traindicated in patients with cardiac cephalgia due
Discussion. We summarize the new patients and to their ability to constrict coronary arteries.15 Con-
five prior reports in table 1. In all seven patients, versely, the vasodilator nitroglycerin can induce
exertional headache was the most prominent mani- headache in normal subjects, and may have an espe-
festation of well-documented myocardial ischemia. cially potent headache-inducing effect in migraine
The onset of headache with exercise, the association sufferers.16In four of the patients with cardiac ceph-
of headache onset with documented simultaneous algia (see the table), treatment with nitrates relieved
myocardial ischemia, and the resolution of headache exertional headache.
with medical (Patients 1, 2, 4, 5, 6, 7) and surgical The key to identifying cardiac cephalgia is the
(Patients 3, 5, 6, 7) therapy support our contention temporal profile of the pain. The headache typically
that myocardial ischemia may present with exer- begins in close proximity to the onset of vigorous
tional headache. We suggest the term “cardiac ceph- exercise, and subsides with rest and with antiangi-
814 NEUROLOGY 49 September 1997
Table Patient reports of cardiac cephalgia

Author Headache Result of


(patient agehex) character Cardiac symptoms Workup Treatment therapy

Fleetcroft and Frontal Tightness of chest ECG: 1 ST AVF, Vl-z Isosorbide Headache
Maddocks5 (78IF) dinitrate resolved
nifedipine
Blacky et aL6 (40/M) Bitemporal None Stress-thallium: inferolateral Antianginal Headache
& ST associated with headache; therapy resolved
reversible inferoapical
perfusion defect; cath: left
circumflex obstructed, RCA
stenosis
Lefkowitz and Biller7 Vertex Retrosternal pain E C G anterolateral & ST; stress CABG Headache
(62/M) test: & ST associated with resolved
headache; cath: anterolateral
wall hypokinesia, three-vessel
disease
Vernay et aL8 (71/M) Occipital, Chest tightness ECG. anteroapical J ST; stress Isosorbide Headache
parietal, test: & ST associated with dinitrate resolved
frontal headache
Bowen and Bitemporal, Rare left-arm ECG: anterior 1 ST; cath: RCA Nitroglycerin, Headache
Oppenheimer9(59/M) left-sided painful total occlusion, proximal OM, PTCA resolved
predominance pressure; chest 95% stenosis
pain once
Lipton” (57/M) Severe vertex Occasional ECG: T-wave flattening I, AVF; Diltiazem, Symptoms
pain sensation of biphasic T-wave Vz4; stress- aspirin decreased
chest heaviness/ thallium: anterior & ST CABG Headache
nausea associated with headache; resolved
anterior hypoperfusion defect;
cath: three-vessel disease
Lipton” (67/M) Bifrontal, None Stress-thallium: inferolateral Nitroglycerin, Headache
squeezing & ST, associated with rotational resolved
pressure headache; inferolateral atherectomy,
perfusion defect; cath: three- PTCA
vessel disease
-

:k Current report.
F = female; M = male; AVF = arteriovenous fistula; CABG = coronary artery bypass graft; cath = cardiac catheterization; OM = ob-
tuse marginal; PTCA = percutaneous transluminal coronary angioplasty; RCA = right coronary artery; stress test = cardiac exercise
stress test; J ST = depressed ST segment.

nal treatment. The diagnosis should be suspected in nial pressure. Although changes in intracranial pres-
patients with headache onset after age 50 and in sure during angina have not been documented, head-
patients with risk factors for cardiac disease. ache and increased intracranial pressure have been
Workup with a stress thallium test should usually described in patients with decreased cranial venous
confirm or exclude the diagnosis. If cardiac cephalgia return secondary to jugular or superior vena caval
is a possibility, prompt workup is essential before obstruction.z0,21Das22reported a patient with head-
treatment with vasoactive migraine drugs. ache and a defective cardiac pacemaker. Synchro-
The mechanism of cardiac cephalgia is specula- nous atrial and ventricular contraction caused a sud-
tive. Indeed, the bases of ischemic chest pain or pres- den increase in right atrial pressure that was
sure and extrathoracic cardiac ischemic pain are transmitted intracranially t o produce headache.
poorly understood.17One possible explanation of car- Headache from sudden, transient, increased intra-
diac cephalgia is anatomic: the heart’s sympathetic cranial pressure is also proposed as an explanation
fibers are supplied by cervical and thoracic ganglia. for cough headache. An increase in intra-abdominal
Because fibers from these ganglia also supply struc- and intrathoracic pressure resulting from the cough
tures of the eye, face, neck, and cerebrovascu1ature,l8 impedes venous return to the right atrium.13
referral of pain along these pathways might account A third explanation for cardiac cephalgia posits an
for headache symptoms. A second possibility is that as yet unidentified mediator released secondary to
the decrease in cardiac output and the increase in cardiac ischemia that might act on intracranial pain-
left ventricular and right atrial pressure associated sensitive structures. Serotonin, bradykinin, hista-
with anginal9cause a decrease in venous return from mine, and substance P have been proposed as medi-
the brain and subsequently an increase in intracra- ators of ischemic painz3 and might also have distant
September 1997 NEUROLOGY 49 815
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816 NEUROLOGY 49 September 1997


Cardiac cephalgia: A treatable form of exertional headache
R. B. Lipton, T. Lowenkopf, Z. H. Bajwa, et al.
Neurology 1997;49;813-816
DOI 10.1212/WNL.49.3.813

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