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44 Cardiac Cephalalgia
425
(continued)
Calcium channel
blockers, aspirin,
myocardial
revascularization,
rotational
atherectomy,
2a,b;
3a,b,c,d)
Exertion Rest Nausea C (1;
out of
10in
severity)
percutaneous
transluminal
coronary
angioplasty
Aspirin, enalapril,
simvastatin,
myocardial
revascularization
3a,d)
vomiting
B; C (1;
2a,b;
discomfort
Rest Chest
temperature
Severe Exertion or
Stent
C (1;
3a,d)
discomfort
denied
Nitrates
B; C (1;
3a,b,d)
discomfort
grafts
Acute Vertex Sharp Severe (10
Male
(57–67)
Lipton etal.
(1997) [1]
(oppressive)
Dull
Male
(73)
Shankar
etal. (2016)
[23]
Oppressive Severe Exertion Rest Chest
bilateral
occipital
Progressive Vertex and
Acute Explosive Severe Cold sweat and
Male
(35)
(2007) [21]
Mathew
(47)
etal. (2015)
[27]
Male
Seow etal.
Severe Exertion Rest C (3a,d) Saphenous vein
Nonpulsatile Severe Exertion Rest or nitrates Chest
temporal
Vertex and
Bilateral
Male
Male
(76)
Grace etal.
Chen etal.
(2004) [24]
occipital
(59)
(1997) [9]
426
aspirin,
clopidogrel and
heparin,
myocardial
Nitroglycerin,
aspirin and
revascularization
calcium channel
blockers
calcium channel
V. da SilvaLessadeOliveira et al.
Beta-blocker,
blocker,
nitroglycerin and
angioplasty
calcium channel
blocker
ICHD-3
Associated
signs and
Triggering
or
aggravating
criteria Intervention
symptoms
factors Relief factors
Exertion Rest C (1,4) Sublingual nitrate,
3b; 4)
Rest Chest pain C (1;
anesthesia,
exertion
Throbbing Severe Local
C (1,4) Beta-blocker,
chest pain
Severe Nitroglycerin Left arm and
Non-
throbbing
Outcome: death
chest pain
3d)
Table 44.1 (continued)
Headache
location Character Intensity
Vertex and
Onset
mode
Male
Sex
(Age)
Costopoulos
Author
(year)
occipital
(55)
(2011) [8]
temporal,
parietal
Acute Bilateral
Female
(52–67)
Cheng etal.
(2010) [22]
Bilateral
temporal
Male
(59)
Bowen and
Oppenheim
(1993) [7]
Headache and
Female,
Takayanagi
Exertion Nitroglycerin Chest pain B; C (1,
Female
Male
(64–67)
Wayne
etal. (1990)
[33]
(>49)
(1986) [34]
44 Cardiac Cephalalgia
Percutaneous
transluminal
coronary
B; C (1,
3d)
angioplasty
Aspirin,
C (1,
metoprolol,
lisinopril,
simvastatin,
heparin
3d)
427
denied
Acute Occipital Throbbing Severe Exertion Rest Chest pain
vomiting and
facial pallor
Acetaminophen Nausea,
Exertion or
rest
Mild-to-
moderate
Throbbing
occipital
and frontal
Acute Bilateral
Occipital Outcome: death
Male
(64)
Ishida etal.
(1996) [10]
Female
Male
(47)
Broner etal.
Auer etal.
(2001) [20]
(72)
(2007) [35]
Age: years
ICHD-3 International Classication of Headache Disorders, 3rd edition
428
V. da SilvaLessadeOliveira et al.

44.6 Diagnostic Approach

Accurate diagnosis of cardiac cephalalgia necessitates distinguishing its unique characteristics from other headache types, including migraine and secondary head­aches [6]. Recognizing the epidemiological prole of this condition is crucial for clinical awareness in potentially affected patients. Cardiac cephalalgia primarily affects individuals over 50 years of age, and a male predominance is observed, attributed potentially to a higher prevalence of cardiovascular risk factors [4, 16]. Concurrent cardiovascular risk factors, such as hypertension, coronary artery dis­ease, and diabetes mellitus, are common [14].
Distinguishing cardiac headache from other disorders can be challenging; how­ever, the simultaneous onset of headache and cardiac symptoms is a key diagnostic clue. Cardiac headache typically presents with a sudden onset, reaching peak inten­sity rapidly and maintaining a stable severity. Autonomic symptoms, such as nau­sea, may be present, while photophobia and phonophobia are usually absent. Exacerbation can occur with both physical activity and rest [2, 3, 6]. These features should be meticulously documented in the patient’s history. Clinical evaluation should focus on conrming the headache’s characteristics, including onset, loca­tion, triggering, associated, and relieving factors, as previously described. Conrmatory diagnosis relies on demonstrating acute myocardial ischemia through ancillary testing. Resting or stress electrocardiography (ECG), stress testing, and myocardial perfusion imaging can reveal ischemic changes. Cardiac biomarkers, such as cretine kinase muscle brain (CK-MB) and troponins, further support the diagnosis [6, 11, 14, 19]. Denitive diagnosis is established by the resolution of headache following treatment of the underlying coronary artery disease. Therapeutic strategies include addressing the coronary pathology, which alleviates the headache [4, 16]. Triptans, commonly used for migraine, are contraindicated due to their vasoconstrictive effects, which can exacerbate coronary ischemia. Vasodilators, such as nitroglycerin, are preferred for their coronary vasodilatory properties and relief of referred pain. Revascularization procedures, including percutaneous coro­nary intervention (PCI) and coronary artery bypass grafting (CABG), restore coro­nary ow and promptly resolve the headache, conrming the diagnosis.

44.7 Management

Based on the synthesized pathophysiology, cardiac cephalalgia is characterized by headaches originating from underlying cardiac ischemia. Therefore, the primary therapeutic objective is to address acute cardiac ischemia, thereby alleviating the headache. Consequently, conventional treatments for primary headaches, such as
44 Cardiac Cephalalgia
429
non-steroidal anti-inammatory drugs (NSAIDs), are not indicated. Triptans are formally contraindicated [2, 6]. Treatment modalities for cardiac cephalalgia range from intravenous medications, such as nitroglycerin, to invasive procedures, includ­ing percutaneous coronary intervention (PCI). Literature reviews consistently high­light nitrates, particularly nitroglycerin, as the rst-line treatment for cardiac headache relief. Nitroglycerin can be administered via sublingual or intravenous routes [1, 6]. For sublingual administration, a 0.4mg dose is recommended, with response observed within approximately 30 minutes. Intravenous administration should begin with 5–10μg/minute, titrated based on patient response, utilizing an infusion pump. The maximum dose is typically 400μg/minute. Continuous moni­toring of blood pressure and heart rate is essential during administration. Nitroglycerin’s efcacy stems from its vasodilatory properties, which reduce car­diac afterload and improve venous return. This enhances blood ow to ischemic myocardial cells, alleviating the referred headache [36]. The response to vasodila­tors, particularly nitrates such as nitroglycerin, is a pivotal aspect in supporting the diagnosis of cardiac headache and differentiating it from conditions like migraine [4, 16]. Cardiac headache, originating from myocardial ischemia, typically resolves with coronary vasodilation. This response contrasts sharply with migraine, where vasodilators are generally ineffective and usually trigger or exacerbate symptoms [4, 16]. Therefore, a positive response to vasodilators not only provides therapeutic benet but also serves as a crucial diagnostic clue, helping to distinguish cardiac headache from other primary headache disorders. Additional treatments for acute myocardial ischemia, as described in the literature, include brinolytic agents and invasive procedures like PCI.These interventions aim to restore coronary blood ow, thereby resolving headaches through the previously described pathophysio­logical mechanisms [4].

44.8 Conclusion

Discussing cardiac cephalalgia is crucial for raising clinical awareness, particularly given that acute headache as the initial presentation of myocardial ischemia is exceedingly rare. This atypical presentation signicantly increases the risk of missed diagnoses of acute cardiac ischemia, underscoring the importance of consid­ering cardiac origins in patients presenting with sudden-onset headaches, even in the absence of typical chest pain. Furthermore, it is essential to recognize that car­diac cephalalgia is not necessarily an exertional headache, as it can occur at rest, further complicating its identication. A heightened index of suspicion, coupled with thorough clinical evaluation and appropriate ancillary testing, is paramount to ensuring timely and accurate diagnosis, thereby improving patient outcomes.
430
V. da SilvaLessadeOliveira et al.

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431
Part VIII
Headache or Facial Pain Attributed to
Disorder of the Cranium, Neck, Eyes,
Ears, Nose, Sinuses, Teeth, Mouth
or Other Facial or Cervical Structure
Chapter 45
Cervicogenic Headache
MarceloValença andJulianaRamosde Andrade

45.1 Introduction

Cervicogenic headache (CGH) is a secondary headache disorder characterized by pain referred from a source in the cervical spine or surrounding structures [1]. The condition has gained increasing recognition since it was rst dened more clearly in the late twentieth century [13]. In the 1980s, Australian neurologist Dr. James Lance and British neurologist Dr. Martin Anthony played pivotal roles in establish­ing the foundational concept that cervical structures, especially the upper cervical nerves, could be sources of headache through convergent input onto the trigemino­cervical complex. Their observations of head pain associated with cervical pathol­ogy laid the groundwork for subsequent clinical and anatomical studies.
The International Classication of Headache Disorders (ICHD) has progres­sively rened the diagnostic criteria for CGH, now listed in its third edition (ICHD-3) as a distinct clinical entity [4]. However, despite its formal recognition, CGH remains underdiagnosed and often misclassied, particularly due to its symptom overlap with primary headache disorders such as migraine and tension-type head­ache. The underlying mechanisms of CGH are believed to involve the convergence of afferent bers from the upper cervical roots (C1–C3) and the trigeminal nerve within the trigeminocervical complex.
We present a case of a man who developed episodic occipital headaches during upper respiratory tract infections. The clinical features and presumed pathophysio­logical mechanisms of his pain are consistent with cervicogenic headache triggered by lymphadenopathy compressing the greater occipital nerve (GON). This case serves to explore a possible yet underrecognized etiology of CGH and to highlight the complex neuroanatomical interactions involved.
M. Valença (*) · J. R. de Andrade Federal University of Pernambuco, Recife, Pernambuco, Brazil
Switzerland AG 2026 D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_45
435© The Author(s), under exclusive license to Springer Nature
436
M. Valença and J. R. de Andrade

45.2 Pathophysiology

Cervicogenic headache (CGH) arises from nociceptive sources within the cervical spine, primarily involving structures innervated by the upper cervical nerves (C1– C3). The fundamental pathophysiological mechanism is the convergence of afferent bers from the upper cervical spinal nerves and the trigeminal nerve within the tri­geminocervical complex—a region in the upper cervical spinal cord where sensory input from both the cervical and trigeminal territories is integrated [5, 6]. This ana­tomical convergence enables nociceptive stimuli originating in the cervical spine to be perceived as pain in the head and face, explaining the referred nature of CGH.
Key anatomical sources of nociception implicated in CGH include:
• Zygapophyseal (facet) joints, especially the C2–C3 joint, which is the most com-
monly identied pain generator.
• Atlantoaxial (C1–C2) and atlanto-occipital joints, which can refer pain to the
posterior fossa and occipital region.
• Cervical intervertebral discs, particularly at the C2–C3 level.
• Cervical muscles and ligaments, especially those innervated by C1–C3, such as
the suboccipital muscles.
• Greater occipital nerve (GON), a signicant branch of the C2 dorsal ramus,
which is susceptible to irritation or compression, leading to occipital pain that
may radiate anteriorly.
• Dura mater of the upper spinal cord and posterior cranial fossa, which can also
contribute to pain referral.
Mechanisms of pain generation and referral [5, 6]:
• Peripheral sensitization: Local inammation, trauma, degenerative changes, or
mechanical irritation (e.g., by reactive lymphadenopathy, as in the presented
case) can activate nociceptors in cervical structures.
• Central sensitization: Repeated or intense peripheral nociceptive input can sensi-
tize second-order neurons in the trigeminocervical complex, amplifying pain
perception and broadening the pain referral zone.
• Experimental evidence: Stimulation of the C2–C3 facet joint or the GON in
humans can reproduce characteristic CGH pain patterns, including referral to
frontal and orbital regions, supporting the clinical relevance of trigeminocervical
convergence.
Triggering factors include:
• Degenerative changes (e.g., arthritis of the C2–C3 facet joint)
• Trauma (e.g., whiplash injury)
• Muscle dysfunction or trigger points in the upper cervical and suboccipi-
tal muscles
• Mechanical compression (e.g., by hypertrophied muscles, connective tissue, or
reactive lymphadenopathy)