Headache Medicine, v.10, n.1, p.32-34, 2019
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ABSTRACT
CASE REPORT
Cardiac cephalalgia: A deadly case report
Paulo Sergio Faro Santos
1
Matheus Kahakura Franco Pedro
2
Ana Carolina Andrade
3
1
Neurologist, Head of Headache and Orofacial
Pain Division, Department of Neurology,
Neurological Institute of Curitiba, Curitiba, PR,
Brazil.
2
Neurologist, Department of Neurology,
Neurological Institute of Curitiba, Curitiba, PR,
Brazil.
3
Resident, Department of Neurology,
Neurological Institute of Curitiba, Curitiba, PR,
Brazil.
*Correspondence
Paulo Faro
E-mail: dr.paulo.faro@gmail.com
Received: December 22, 2018.
Accepted: January 13, 2019.
Cardiac cephalalgia is a nosologic entity that has only been acknowledged by
the turn of the century, and is, consequently, often underdiagnosed, even by
experienced neurologists. Unlike most headaches, however, failing to provide a
proper and timely diagnosis can have deadly consequences. Report of a case
of cardiac headache attended at the emergency department and literature
review. This entity was rst described in 1997; no studies have yet determined
its prevalence, with the literature relying on case reports. The pathophysiology
remains a mystery, with three main hypothesis: spinal convergence of cardiac
visceral afferent nerves with somatic afferent nerves from the head, increase
of intracranial pressure from decrease in cerebral venous return originated
from the reduced cardiac output, and release of inammatory markers during
cardiac ischaemia, such as bradykinin, serotonin and histamin, causing vascular
changes. Distinguishing this pathology from others, especially migraine, with
which it shares many traits, is of paramount importance: vasoconstrictor drugs
such as triptans are absolutely contraindicated, and the outcome can be
dramatic. This case illustrates the need to promptly recognize this rare entity
since failure to diagnose it can have devastating consequences.
Keywords: Cardiac Cephalalgia; Myocardial Ischemia; Cardiac Arrest.
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Headache Medicine, v.10, n.1, p.32-34, 2019
INTRODUCTION
Cardiac cephalalgia is a nosologic entity that has
only been acknowledged by the turn of the century,
and is, consequently, often underdiagnosed, even by
experienced neurologists. Unlike most headaches,
however, failing to provide a proper and timely diagnosis
can have deadly consequences
1
.
CASE REPORT
We aim to report he case of ES, a 62 years
old Caucasian male with no previous history of
headache who went to the ER due to a aching,
holocranial, and intense headache lasting over
two weeks, with few moments of respite in the
meantime, with nausea and emesis but no photo/
phonophobia. He developed angina pectoris at the
exact same time, and was subjected to a series of
cardiac exams in the weeks before the pain, which
appeared normal. His comorbities included having
been subjected to a kidney transplantation in 2004,
being still in dialysis, cardiac pacemaker in 2012, as
well as diabetes, heart failure and hypertension. His
admission laboratory workup showed creatinine of
9.16, troponine of 1,95 and CK-MB of 24.24; six hours
later, the exams showed an increase to 1.85 and
26.83, respectively. His EKG showed no ST-segment
elevation.
The hypothesis of cardiac cephalalgia
was raised and his care was transferred to the
cardiology department. He was admitted to the
Coronary Unit, received ASA and clopidogrel and
the patient underwent a percutaneous intervention,
which subsequently demonstrated critical lesions in
anterior descending and right coronaries as well as
thrombus in circumflex artery. After an emergency
coronary artery bypass, he developed hyperkalemia
and went into cardiac arrest, with unsuccessful
reanimation attempts. He fulfilled criteria for cardiac
cephalalgia, as the headache developed in close
temporal relation to the ischaemia and had both
moderate to severe intensity, nausea and absence
of photophobia (Table 1).
DISCUSSION
This entity was first described in 1997
2
; no
studies have yet determined its prevalence,
with the literature relying on case reports
3
.
The pathophysiology remains a mystery, with
three main hypothesis: spinal convergence of
cardiac visceral afferent nerves with somatic
afferent nerves from the head, increase of
intracranial pressure from decrease in cerebral
venous return originated from the reduced cardiac
output, and release of inflammatory markers during
cardiac ischaemia, such as bradykinin, serotonin and
histamin, causing vascular changes
4
. Distinguishing
this pathology from others, especially migraine,
with which it shares many traits, is of paramount
importance: vasoconstrictor drugs such as triptans
are absolutely contraindicated, and the outcome
can be dramatic. In a review of seven cases, three
had triple arterial lesion as well, but in all cases the
patient survived
4
.
CONCLUSION
This case illustrates the need to promptly recognize
this rare entity since failure to diagnose it can have
devastating consequences.
International Headache Classication - 3
rd
edition Part two - secondary headaches
A. Any headache fullling criterion C.
B. Acute myocardial ischaemia has been demonstrated.
C. Evidence of causation demonstrated by at least two of the following:
1. headache has developed in temporal relation to onset of acute myocardial ischaemia.
2. either or both of the following:
a) headache has signicantly worsened in parallel with worsening of the myocardial ischaemia.
b) headache has signicantly improved or resolved in parallel with improvement in or resolution of the myocardial ischaemia.
3. headache has at least two of the following four characteristics:
a) moderate to severe intensity.
b) accompanied by nausea.
c) not accompanied by phototophia or phonophobia.
d) aggravated by exertion.
4. headache is relieved by nitroglycerine or derivatives of it.
D. Not better accounted for by another ICHD-3 diagnosis.
Table 1. Diagnostic criteria for Cardiac Cephalalgia.
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REFERENCES
1. Headache Classication Committee of the International
Headache Society (IHS). The international classication of
headache disorders, 3rd edition (beta version). Cephalalgia.
2013; 33(9): 629-808.
2. Lipton RB, Lowenkopf T, Bajwa ZH, et al. Cardiac
cephalgia: a treatable form of exertional headache.
Neurology. 1997;49:813–6.
3. Wei JH, Wang HF. Cardiac cephalalgia: case reports and
review. Cephalalgia. 2008;28:892–6.
4. Torres-Yaghi Y, Salerian J, Dougherty C. Cardiac cephalalgia.
Curr Pain Headache Rep (2015) 19:14.
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