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45 Cervicogenic Headache
437
45.3 Case Presentation
The patient is a 58-year-old man with a prior diagnosis of migraine without aura. He
reported experiencing occasional episodes of headache, typically once every year or
two, associated with upper respiratory tract infections characterized by nasal congestion, pharyngitis with erythema, odynophagia, and palpable cervical and retroauricular lymphadenopathy.
During these episodes, he developed a unilateral occipital headache that radiated
anteriorly toward the ipsilateral frontal region. The pain was described as ne, dull,
and bothersome, with a continuous but uctuating intensity rated between 3 and 6
out of 10. Palpation of the ipsilateral occipital or nuchal region exacerbated the pain.
He noted that the pain was worsened by head and neck movement.
These episodes lasted between 1 and 3 days, coinciding with the peak of his
infectious symptoms. Over time, he had experienced at least eight such episodes,
more commonly on the right side. No diagnostic nerve block was performed. The
headaches resolved spontaneously as his systemic symptoms improved.
Given the anatomical overlap between the pain distribution and the sensory territory of the GON, as well as the presence of enlarged lymph nodes during symptomatic periods, we hypothesize that reactive cervical lymphadenopathy caused
intermittent compression of peripheral nerves such as C2, GON, or the lesser occipital nerve, contributing to his headache episodes.
45.4 Clinical Characteristics ofHeadache
Cervicogenic headache (CGH) presents with distinct clinical characteristics,
although some features may overlap with other headache types. The hallmark symptom is a unilateral headache that typically does not shift sides. The pain often follows a characteristic “ram’s horn” pattern, beginning in the occipital region and
potentially radiating toward the frontal, temporal, or orbital areas [7, 8].
Patients almost always report ipsilateral neck pain or discomfort, which is frequently associated with tenderness over the upper cervical spine joints (C1–C3),
restricted cervical range of motion (ROM), and worsening of headache with neck
movement or prolonged awkward head positions. The quality of pain is usually
described as dull, steady, and non-throbbing, with a tightening or pressing sensation, and pain intensity is often moderate [9].
Associated symptoms may include diffuse, vague shoulder, and arm pain on the
same side, which can occasionally exhibit radicular features. There is often muscle
tightness and myofascial trigger points in muscles such as the upper trapezius, levator scapulae, scalenes, and suboccipital muscles. Some patients may also present
with weakness in the deep neck exors and atrophy of suboccipital muscles. If the
greater occipital nerve is involved, sensory changes in the occipital scalp may occur.
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M. Valença and J. R. de Andrade
Neck movements, sustained postures, or external pressure over the upper cervical or occipital region typically exacerbate a headache. As a result, patients often
adopt protective behaviors to minimize or avoid neck motion.
45.5 Diagnosis ofCervicogenic Headache
The diagnosis of cervicogenic headache (CGH) relies on a comprehensive approach
that includes clinical evaluation, established diagnostic criteria, and, when necessary, conrmatory procedures. Because CGH often mimics primary headache disorders such as migraine or tension-type headache, careful clinical assessment is
essential. The diagnostic process begins with a detailed history, focusing on the
onset, duration, and characteristics of the headache, its relationship to neck movement or sustained postures, and the presence of neck pain, trauma, or known cervical pathology. Associated symptoms, such as ipsilateral shoulder or arm pain, are
also essential to document [2, 9].
Physical examination should include palpation of the upper cervical spine and
occipital region for tenderness, evaluation of cervical range of motion (often
restricted), and observation of headache provocation by neck movements or pressure. A neurological exam helps rule out alternative causes. Manual diagnostic tests
such as the cervical exion–rotation test (CFRT), palpation for myofascial trigger
points, and assessment of deep cervical muscle strength and function further support the clinical impression [2, 9, 10].
Diagnostic criteria from the International Classication of Headache Disorders
(ICHD-3) [4] require (A) a headache fullling causation criteria, (B) clinical or
imaging evidence of cervical pathology known to cause headache, (C) at least two
indicators of a causal relationship—such as temporal correlation with cervical
pathology, improvement with treatment, worsening with neck movement, or relief
following a diagnostic nerve block—and (D) exclusion of other ICHD-3 diagnoses.
The Cervicogenic Headache International Study Group (CHISG) adds further
specicity with criteria such as precipitation of headache by neck motion or pressure, restricted cervical motion, ipsilateral neck/shoulder/arm pain, consistent onesided headache without shifting, and conrmation by anesthetic block.
Conrmatory diagnostic procedures include imaging techniques like magnetic
resonance imaging (MRI) or computed tomography (CT) to identify cervical spine
lesions or rule out other structural causes. Diagnostic nerve blocks targeting cervical joints or nerve roots are used to conrm the cervical origin if the headache is
abolished. Lastly, differential diagnosis is essential to exclude primary headache
disorders and other secondary causes such as vascular, neoplastic, infectious, or
traumatic conditions.
45 Cervicogenic Headache
439
45.6 Treatment
The diagnosis of cervicogenic headache is often complex due to its diverse etiologies and symptom overlap with primary headaches. In this case, the episodic nature
of the pain, its occipital onset with frontal radiation, exacerbation by neck movement, and aggravation by occipital palpation support the diagnosis of CGH.
The convergence of cervical afferents (C1–C3) and trigeminal inputs within the
trigeminocervical complex allows for the referral of cervical nociceptive input to
cranial regions, particularly the orbit and forehead, as rst proposed by Lance and
Anthony [1, 11]. This neuroanatomical substrate provides a logical explanation for
why cervical pathology can manifest as cranial pain.
Support for this mechanism comes from experimental studies such as that of
Piovesan et al. [2], who investigated referred pain following stimulation of the
GON.In their study, 2mL of distilled water was injected near the right GON in
three human subjects. Two of the three participants experienced referred pain
extending beyond the anatomical territory of the GON, projecting into the ipsilateral ophthalmic division (V1) territory. This phenomenon is a direct clinical correlate to the patient described here, where presumed GON compression by
lymphadenopathy elicited pain that radiated from the occipital to the frontal region.
Their study supports the concept that the convergence of cervical and trigeminal
inputs within the spinal cord and brainstem facilitates the mislocalization of pain.
This convergence theory has formed the basis for understanding not only cervicogenic headache but also its treatment modalities, which target cervical structures.
Interestingly, this case also highlights an underexplored cause of CGH—transient reactive lymphadenopathy that compresses the cervical nerves. Though not
traditionally included in the differential diagnosis of CGH, reactive nodes may pose
a potential entrapment point. The GON, for instance, exits the semispinalis capitis
muscle and ascends through a dense network of connective tissue and musculature
in the suboccipital region, which can be a site for entrapment or compression [8].
There is a growing trend among surgeons to perform decompression surgeries
targeting cranial nerves, particularly the greater occipital nerve, in patients with
refractory headache disorders [12]. Emerging evidence suggests that these nerves
may be subjected to chronic compression from surrounding anatomical structures,
such as hypertrophied muscles, brous bands, or aberrant vascular loops. These
compressive phenomena are believed to contribute to the pathophysiology of certain chronic headaches, including cervicogenic headache and occipital neuralgia.
Interestingly, a signicant proportion of patients who undergo occipital nerve
decompression procedures report substantial and lasting relief from pain, often after
years of failed pharmacological treatments. This growing body of surgical outcomes
is prompting a reevaluation of the role of peripheral nerve compression in chronic
headache syndromes. It supports further investigation into targeted neurosurgical
interventions as part of a comprehensive headache management strategy [13, 14].
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M. Valença and J. R. de Andrade
The anatomical course of the GON and its susceptibility to mechanical irritation
have led to surgical decompression being proposed as a treatment option for refractory occipital neuralgia and chronic migraine, particularly when physical ndings
correlate with tenderness over the nerve.
Although this patient did not undergo diagnostic nerve blockade, which is a
requirement for CGH diagnosis per ICHD-3, the temporal relationship between
infection, lymphadenopathy, and headache resolution supports a causal
relationship.
45.7 Conclusion
This case illustrates a possible variant of cervicogenic headache triggered by
mechanical irritation of the greater occipital nerve from reactive cervical lymphadenopathy. It highlights the need to consider uncommon yet plausible causes of cervicogenic pain, particularly in patients with episodic occipital-frontal headaches in
the setting of infection.
Further studies are needed to validate whether lymph node-induced nerve compression should be formally considered among the etiologies of CGH.This case
also underscores the importance of detailed anatomical and clinical assessment in
the diagnosis and management of secondary headache disorders.
References
1. Lance JW, Anthony M.Neck-tongue syndrome on sudden turning of the head. J Neurol
Neurosurg Psychiatry. 1980;43:97–101. https://doi.org/10.1136/jnnp.43.2.97.
2. Piovesan E, Kowacs P, Tatsui C, Lange M, Ribas L, Werneck L. Referred Pain After Painful
Stimulation of the Greater Occipital Nerve in Humans: Evidence of Convergence of Cervical
Afferences on Trigeminal Nuclei. Cephalalgia [Internet]. 2001;21(2):107–9. Available from:
https://journals.sagepub.com/doi/10.1046/j.1468-2982.2001.00166.x
3. Bartsch T, Goadsby PJ.The trigeminocervical complex and migraine: current concepts and synthesis. Curr Pain Headache Rep. 2003;7:371–6.
4. The International Classication of Headache Disorders, 3rd edition (beta version). Cephalalgia.
2013;33:629–808. https://doi.org/10.1177/0333102413485658.
5. Bogduk N, Govind J.Cervicogenic headache: an assessment of the evidence on clinical diagnosis, invasive tests, and treatment. Lancet Neurol. 2009;8:959–68. https://doi.org/10.1016/
S1474- 4422(09)70209- 1.
6. Sjaastad O, Fredriksen TA, Pfaffenrath V.Cervicogenic headache: pathophysiology, diagnostic
criteria and treatment. Agri. 2005;17(3):1–7.
7. Rubio-Ochoa J, Benítez-Martínez J, Lluch E, Santacruz-Zaragozá S, Gómez-Contreras P,
Cook CE.Physical examination tests for screening and diagnosis of cervicogenic headache:
a systematic review. Man Ther. 2016;21:35–40. https://doi.org/10.1016/j.math.2015.09.008.
8. Fredriksen TA, Hovdal H, Sjaastad O. “Cervicogenic headache”: clinical manifestation.
Cephalalgia. 1987;7:147–60.
https://doi.org/10.1046/j.1468- 2982.1987.0702147.x.
https://doi.org/10.1007/s11916- 003- 0036- y.
45 Cervicogenic Headache
9. Leone M, D'Amico D, Grazzi L, Attanasio A, Bussone G.Cervicogenic headache: a critical review of the current diagnostic criteria. Pain. 1998;78(1):1–5. https://doi.org/10.1016/
S0304- 3959(98)00116- X.
10. Putilina MV. Cervicogenic headache: pathogenesis, clinical features, and diagnosis. Neurol
Neuropsychiatr Pol. 2011;45(5):456–60.
11. Anthony M.Cervicogenic headache: prevalence and response to local steroid therapy. Clin
Exp Rheumatol. 2000;18:S59–64.
12. Hammond S.Headache caused by neuralgias and structural disorders of the neck. In: Olesen
J, Tfelt-Hansen P, Welch KMA, editors. The headaches. 2nd ed. Lippincott Williams &
Wilkins; 2005.
13. Jansen J.Surgical treatment of non-responsive cervicogenic headache. Clin Exp Rheumatol.
2000;18:S67–70.
14. van Suijlekom JA, Weber WE, van Kleef M.Cervicogenic headache: techniques of diagnostic
nerve blocks. Clin Exp Rheumatol. 2000;18:S39–44.
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Chapter 46
Headache Attributed toCraniocervical
Dystonia
MíriamCarvalho Soares andPedroAugustoSampaio Rocha-Filho
46.1 Introduction
Headache attributed to craniocervical dystonia (HACCD) is listed in the third edition of the International Classication of Headache Disorders (ICHD-3) among
headaches attributed to disorders of the neck [1]. This type of headache is closely
related to dystonia, which is a movement disorder characterized by sustained or
intermittent muscle contractions that lead to abnormal, often repetitive movements
and postures. These movements are typically patterned and twisting, and may
include tremors. Diagnosis is primarily clinical, with distinctive features such as
“sensory tricks”—specic maneuvers that temporarily alleviate dystonic symptoms—crucial in conrming the condition [2]. When muscle contractions extend to
adjacent areas, such as the neck, oromandibular region, tongue, or larynx, the condition is termed craniocervical dystonia (CCD), a type of segmental dystonia [3].
CCD is a complex and often debilitating neurological disorder marked by sustained
or intermittent abnormal postures and movements of the head and neck, typically
accompanied by painful muscle contractions.
While the primary symptoms of CCD are motor disturbances, an often overlooked aspect of the condition is the signicant presence of pain [4]. Headaches in
individuals with dystonia are not well-documented in the literature, but they are
commonly described as either starting or worsening in conjunction with dystonic
episodes. HACCD can severely impact quality of life and present considerable challenges in terms of both diagnosis and treatment [1].
Several mechanisms may contribute to headaches in CCD patients. These include
pain resulting from abnormal cervical muscle contractions, altered postural alignment, or potential involvement of the central nervous system and its sensitization [4,
M. Carvalho Soares · P. A. Sampaio Rocha-Filho (*)
Federal University of Pernambuco (UFPE), Recife, Brazil
e-mail: pedro.rochalho@ufpe.br
Switzerland AG 2026
D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_46
443© The Author(s), under exclusive license to Springer Nature
444
M. Carvalho Soares and P. A. Sampaio Rocha-Filho
5]. In patients with cervical dystonia (CD), headache attributed to dystonia may
present with a migraine-like or tension-type pattern. Additionally, headaches—such
as migraine, tension-type, or cervicogenic headache—may coexist independently,
without a temporal relationship to the dystonic movements, further complicating the
diagnostic process [6]. Therefore, understanding the interplay between CCD and
headache is essential for optimizing patient management and improving outcomes.
This chapter provides a comprehensive overview of HACCD, exploring its
pathophysiology, diagnostic challenges, and treatment strategies. A clinical case
will be presented to illustrate key aspects of HACCD.By examining this comorbidity, we aim to emphasize the importance of a multidisciplinary approach to managing both the motor and non-motor manifestations of this complex disorder.
46.2 Pathophysiology
The pathophysiology of pain in dystonia involves both “muscle-based” and “nonmuscle- based” mechanisms. Preclinical studies suggest that sustained muscle contractions in dystonia can lead to muscle ischemia, triggering the release of
inammatory mediators, including substance P, bradykinin, and ATP.These mediators increase the excitability of A-delta and C bers, potentially leading to abnormal
nociceptive transmission and processing. Some hypotheses also propose that trigger
points in dystonic muscles contribute to pain through excessive local release of
these inammatory mediators, which can further exacerbate muscle contractions [7].
Additionally, dysfunction in descending pain inhibitory pathways, as well as
structural and functional changes in the basal ganglia, cortex, and other areas
involved in sensorimotor integration, may contribute to the pain experience in CCD
patients. Individuals with dystonia may experience abnormal cortical sensorimotor
integration and processing, further complicating the pain experience. Pain in dystonia is likely multifactorial, inuenced by factors such as the severity and chronicity
of dystonic postures, as well as comorbid orthopedic complications (e.g., spinal
degeneration, disc herniation, or radiculopathy), which may also contribute to the
development of pain [8]. Nevertheless, pain does not always correlate with the
severity of motor symptoms. Some individuals with severe dystonic movements
report little or no pain, while others with similar levels of motor involvement experience signicant discomfort [5].
Although the exact mechanisms linking dystonia and headache—particularly
migraine—remain unclear, shared pathogenic pathways, such as cortical spreading
depression and ion channel gene dysfunction (e.g., CACNA1A, ATP1A2, ATP1A3,
PRRT2, PNKD), suggest a phenotypic overlap [9]. Additionally, pain may result
from local muscle contraction, genetic susceptibility, and secondary sensitization.
46 Headache Attributed toCraniocervical Dystonia
445
46.3 Case Presentation
A 60-year-old male was diagnosed with cervical dystonia at the age of 32, initially
presenting with involuntary movements in the cervical muscles. Over the past
20years, he has been undergoing botulinum toxin type A therapy (BoNT-A). In
addition to his dystonia, he has experienced cervical pain and headache for the past
10years, coinciding with a worsening of the dystonic movements. Brain magnetic
resonance imaging (MRI) did not show any structurally signicant alteration.
Cervical spine computed tomography (CT) was unremarkable as well. No alternative secondary etiologies for dystonia were found.
On physical examination, the patient exhibited right torticollis, left laterocollis,
and anterocollis (Fig.46.1). Latent trigger points were identied bilaterally in the
superior trapezius and the left suboccipital region. The patient rated his pain as
moderate, scoring 7 out of 10 on the Visual Analog Scale (VAS). The pain was primarily localized in the occipital and nuchal regions bilaterally coinciding with areas
of greater muscle contraction. It was described as a pressing sensation, with additional descriptors of tiredness, annoyance, and tightness (McGill Pain Questionnaire).
The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) was administered, revealing scores of 19in the severity subscale (indicating moderate severity);
11in disability (moderate functional impact); and 5in pain (minimal).
The patient reported experiencing headaches on an average of 20days per month.
He denied nausea or phonophobia but noted mild photophobia. He also indicated
that his headaches were not exacerbated by physical exertion. Despite the frequency
of his headache, he had not been using prophylactic treatment or overusing analgesics. His Headache Impact Test (HIT-6) score was 56, suggesting a signicant
impact on his quality of life due to headaches. The headache pattern was consistent
with a tension-type headache.
abc
Fig. 46.1 Patient with cervical dystonia. (a) Right torticollis and left laterocollis (b) Noticeable
anterocollis. (c) Latent trigger points in the bilateral superior trapezius and in the left suboccipital
region. (Created in BioRender. Soares (2025) https://BioRender.com/2sllw1q)
446
M. Carvalho Soares and P. A. Sampaio Rocha-Filho
Regarding his cervical dystonia, the patient reported a subjective improvement
of approximately 70% in cervical movements with BoNT-A, with effects lasting
around 4 months following previous injections. One month after his most recent
BoNT-A injection, the patient reported no headaches, and his muscle contractions
were notably improved, as reected in his TWSTRS score—severity decreased to 7,
disability to 6, and pain was reduced to 0. On physical examination, the trigger
points in the trapezius muscles had resolved, though the trigger point in the left
suboccipital region remained. The patient noted that as the effects of the BoNT-A
began to wear off, his pain gradually returned. BoNT-A treatment was administered
using abobotulinum toxin, with the specic injected muscles and dosages detailed
in Table46.1.
Cervical dystonia (CD) is more common in women than in men, with a prevalence ranging from 5 to 20 per 100,000 individuals. It is the most common form of
focal dystonia and is often referred to as spasmodic torticollis when conned to the
cervical region [10]. The heterogeneous nature of CD arises from the varied patterns
of muscle involvement, which can signicantly impact diagnosis and treatment
[11]. Regarding etiology, primary CD may be idiopathic or, in approximately 12%
of cases, hereditary. Secondary CD results from other underlying pathologies,
which may be inherited or acquired. Secondary causes of CD include medication
side effects, vascular injury, or neurodegenerative diseases such as Huntington’s
disease or Parkinson’s disease [12].
CD can present as either painless or painful, with pain being a signicant source
of disability for many patients. Several instruments are used to assess the severity of
dystonia, including the Toronto Western Spasmodic Torticollis Rating Scale
(TWSTRS), a validated tool that evaluates the severity of cervical dystonic contractions, functional disability, and pain intensity [13]. The condition signicantly
impairs quality of life, causing both functional and psychosocial disabilities. Pain is
often the primary reason patients seek treatment, with approximately two-thirds of
CD patients requiring analgesics during the disease. Studies report that up to 66%
of CD patients experience signicant pain, and in some studies, pain prevalence is
reported as high as 90% [4].
Given the high prevalence of pain among CD patients, recent studies have
explored its specic manifestations and comorbidities, including the association
with migraine. Zolin etal. examined 58 patients with comorbid CD and migraine.
Most were female (88%), with migraine preceding CD in 72% of cases. Laterocollis
Table 46.1 Botulinum toxin
dosage and muscles selected
(abobotulinum toxin)
Muscles Dosage (units)
Left splenius capitis 40
Left levator scapulae 60
Right sternocleidomastoid 20
Left sternocleidomastoid 60
Right trapezius (pars descendens) 20
Left trapezius (pars descendens) 60
46 Headache Attributed toCraniocervical Dystonia
447
was the most prevalent phenotype (98%), often coexisting with torticollis (60%)
and no correlation was observed between migraine frequency and dystonia severity.
Nonetheless, dystonic movements were common migraine triggers. Treatment with
BoNT-A led to migraine improvement in 63% of patients at 12months, suggesting
a therapeutic role for botulinum toxin in this subgroup [14]. The study highlights
how specic dystonic patterns may be linked to headache manifestations.
In this clinical case, the patient experienced a headache that began 18years after
the onset of cervical dystonia. The onset of this headache coincided with the worsening of the dystonia. The headache improved with the improvement in the dystonia, after each cycle of botulinum toxin. Therefore, we can classify it as HACCD.The
treatment of this headache consisted of treating the dystonia with BoNT-A.
46.4 Clinical Characteristics
The ICHD-3 recognizes that CCD, in the context of HACCD, encompasses not only
classical spasmodic torticollis (isolated cervical dystonia) but also other forms of
segmental dystonia, such as pharyngeal, mandibular, and lingual dystonia, which
may contribute to the headache [1]. The prevalence of HACCD varies between studies, ranging from 1.3 (using ICHD-2 criteria) [6] to 29% (using ICHD-3 criteria)
[15]. This variability in prevalence may be partly attributed to evolving diagnostic
criteria, as reected in the current denition of HACCD in ICHD-3 [1] (Table46.2).
46.5 Differential Diagnosis
HACCD is a secondary headache disorder that typically manifests in regions
affected by dystonic muscle contractions—most commonly the occipital, suboccipital, or temporal areas. Diagnostic clues include the temporal and topographical
relationship between the onset of headache and dystonic activity, as well as improvement in headache following effective dystonia treatment, most notably with BoNT-A
Table 46.2 HACCD diagnostic criteria by ICHD-3 [1]
A.Neck and posterior head pain fullling criterion C.
B.CCD is demonstrated by abnormal movements or defective posturing of the neck and/or head
due to muscular hyperactivity.
C.Evidence of causation, as indicated by at least two of the following:
1. Pain has developed in close temporal relation to the onset of CCD.
2. Pain has signicantly worsened in parallel with progression of the CCD.
3. Pain has signicantly improved or resolved in parallel with improvement in or resolution of
the CCD.
4. Pain location corresponds to the location of the dystonic muscle(s).
D.Not better accounted for by another ICHD-3 diagnosis.