CERVICOGENIC HEADACHE

By Dr Jyeshtharaj Patangankar

INTRODUCTION

Cervicogenic headache is a chronic, hemi cranial pain syndrome in which the

sensation of pain originates in the cervical spine or soft tissues of the neck and is
referred to the head so presents as Headache as a Manifestation of Neck
Disorders.

Head pain that is referred from the bony structures or soft tissues of the neck is
commonly called “cervicogenic headache.” It is often a sequel of head or neck
injury but may also occur in the absence of trauma. The clinical features of
cervico-genic headache may mimic those commonly associated with primary
headache disorders such as tension-type headache, migraine, or hemicrania
continua, and as a result, distinguishing among these headache types can be
difficult.

The prevalence of cervicogenic headache in the general population is estimated

to be between 0.4% and 2.5%, but in pain management clinics, the prevalence is
as high as 20% of patients with chronic headache. The mean age of patients with
this condition is 42.9 years, and cervicogenic headache is four times more
prevalent in women. Patients with cervicogenic headache have demonstrated
substantial declines in quality of life measurements that are similar to those in
patients with migraine and tension-type headache when compared with control
subjects, but they demonstrate the greatest loss in domains of physical
functioning when compared with the groups with other headache disorders.

Pathophysiology

Sensory afferent nerve fibers from upper cervical regions have been observed to
enter the spinal column by way of the spinal accessory nerve before entering the
dorsal spinal cord. The close association of sensory motor fibers of the spinal
accessory nerve with the spinal sensory nerves is believed to allow for a
functional exchange of somatosensory, proprioceptive, and nociceptive
information from the trapezius, sternocleidomastoid, and other cervical muscles
to converge in the trigeminocervical nucleus and ultimately resulting in the
referral of pain to trigeminal sensory fields of the head and face.

Types of Cervicogenic Headache

 Occipital Headache:- felt at occiput. Can radiate to the vertex. Corresponds

to posterior rami of C2 & C3.

 Occipito-temporo-maxillary Headache:- located in retro-auricular region,

mastoid, and parietal region. Radiates towards inferior maxilla.
Corresponds to ipsilateral facets of C2-3

 Supra-orbital Headache: -it is the most frequent type of headache of

cervical origin, Pain is referred in supra-orbital region only.
Diagnosis

The Cervicogenic Headache International Study Group developed diagnostic

criteria that have provided a detailed, clinically useful description of the
condition. The diagnosis of cervicogenic headache can often be made without
resorting to diagnostic neural blockade by completion of a careful history and
physical examination.

ICHD-3 diagnostic criteria for cervicogenic headache

A. Any headache fulfilling criterion C

B. Clinical and/or imaging evidence of a disorder or lesion within the

cervical spine or soft tissues of the neck, known to be able to cause

headache

C. Evidence of causation demonstrated by at least two of the following:

1. headache has developed in temporal relation to the onset of the cervical

disorder or appearance of the lesion

2. headache has significantly improved or resolved in parallel with

improvement in or resolution of the cervical disorder or lesion

3. cervical range of motion is reduced and headache is made significantly

worse by provocative maneuvers

4. headache is abolished following diagnostic blockade of a cervical

structure or its nerve supply

D. Not better accounted for by another ICHD-3 diagnosis

Headache Classification Committee of the International Headache Society (IHS) The International Classification of Headache Disorders,

3rd edition. Cephalalgia. 2018 Jan;38(1):1–211 [12]. Copyright © 2018 by International Headache Society. Reprinted by the permission of SAGE

Publications, Ltd.

Diagnostic Testing for Suspected Cervicogenic Headache

Patients with cervicogenic headache will often have altered neck posture or
restricted cervical range of motion. The head pain can be triggered or reproduced
by active neck movement, passive neck positioning especially in extension or
extension with rotation toward the side of pain, or on applying digital pressure to
the involved facet regions or over the ipsilateral greater occipital nerve. Muscular
trigger points are usually found in the suboccipital, cervical, and shoulder
musculature, and these trigger points can also refer pain to the head when
manually or physically stimulated. There are no neurologic findings of cervical
radiculopathy, though the patient might report scalp paresthesia or dysesthesia.

Diagnostic imaging such as radiography, magnetic resonance imaging (MRI), and

computed tomography (CT) myelography cannot confirm the diagnosis of
cervicogenic headache but can lend support to its diagnosis. One study reported
no demonstrable differences in the appearance of cervical spine structures on
MRI scans when 24 patients with clinical features of cervicogenic headache were
compared with 20 control subjects. Cervical disc bulging was reported equally in
both groups (45.5% vs 45.0%, respectively).

A comprehensive history, review of systems, and physical examination including a

complete neurologic assessment will often identify the potential for an underlying
structural disorder or systemic disease. Imaging is then primarily used to search
for suspected secondary causes of pain that may require surgery or other more
aggressive forms of treatment. The differential diagnosis in cases of suspected
cervicogenic headache could include posterior fossa tumor, Arnold-Chiari
malformation, cervical spondylosis or arthropathy, herniated intervertebral disc,
spinal nerve compression or tumor, arteriovenous malformation, vertebral artery
dissection, and intramedullary or extramedullary spinal tumors.

A laboratory evaluation may be necessary to search for systemic diseases that

may adversely affect muscles, bones, or joints (ie, rheumatoid arthritis, systemic
lupus erythematosus, thyroid or parathyroid disorders, primary muscle disease,
etc).

Diagnostic Block is used to confirm the diagnosis of cervicogenic headache and

predict the treatment modalities that will most likely provide the greatest
efficacy. The first three cervical spinal nerves and their rami are the primary
peripheral nerve structures that can refer pain to the head.

a. The suboccipital nerve (dorsal ramus of C1) innervates the atlanto-occipital

joint; therefore, a pathologic condition or injury affecting this joint is a
potential source for head pain that is referred to the occipital region.
b. The C2 spinal nerve and its dorsal root ganglion have a close proximity to
the lateral capsule of the atlantoaxial (C1–2) zygapophyseal joint and
innervate the atlantoaxial and C2–3 zygapophyseal joints; therefore,
trauma to or pathologic changes around these joints can be a source of
referred head pain. Neuralgia of C2 is typically described as a deep or dull
pain that usually radiates from the occipital to parietal, temporal, frontal,
and periorbital regions. A paroxysmal sharp or shock like pain is often
superimposed over the constant pain. Ipsilateral eye lacrimation and
conjunctival injection are common associated signs. Arterial or venous
compression of the C2 spinal nerve or its dorsal root ganglion has been
suggested as a cause for C2 neuralgia in some cases.
c. The third occipital nerve (dorsal ramus C3) has a close anatomic proximity
to and innervates the C2–3 zygapophyseal joint. This joint and the third
occipital nerve appear most vulnerable to trauma from acceleration-
deceleration (“whiplash”) injuries of the neck. Pain from the C2–3
zygapophyseal joint is referred to the occipital region but is also referred to
the frontotemporal and periorbital regions. Injury to this region is a
common cause of cervicogenic headache. The majority of cervicogenic
headaches occurring after whiplash resolve within a year of the trauma.

Of interest are reports that patients with chronic headache had experienced
substantial pain relief after discectomy at spinal levels as low as C5–6.
Diagnostic anesthetic blockade for the evaluation of cervicogenic headache can
be directed to several anatomic structures such as the greater occipital nerve
(dorsal ramus C2), lesser occipital nerve, atlanto-occipital joint, atlantoaxial joint,
C2 or C3 spinal nerve, third occipital nerve (dorsal ramus C3), zygapophyseal
joint(s) or intervertebral discs based on the clinical characteristics of the pain and
findings of the physical examination. Fluoroscopic or interventional MRI-guided
blockade may be necessary to assure accurate and specific localization of the pain
source.

Occipital neuralgia is a specific pain disorder characterized by pain that is isolated

to sensory fields of the greater or lesser occipital nerves. The classic description of
occipital neuralgia includes the presence of constant deep or burning pain with
superimposed paroxysms of shooting or shock like pain. Paresthesia and
numbness over the occipital scalp are usually present. It is often difficult to
determine the true source of pain in this condition. In its classic description, the
pain of occipital neuralgia is believed to arise from trauma to or entrapment of
the occipital nerve within the neck or scalp, but the pain may also arise from the
C2 spinal root, C1–2, or C2–3 zygapophyseal joints or pathologic change within
the posterior cranial fossa.

Occipital nerve blockade, as it is typically done in the clinic setting, often results in
a nonspecific regional blockade rather than a specific nerve blockade and might
result in a misidentification of the occipital nerve as the source of pain. This “false
localization” might lead to unnecessary interventions aimed at the occipital nerve,
such as surgical transection or other neurolytic procedures.5

A regional myofascial pain syndrome (MPS) affecting cervical, pericranial, or

masticatory muscles can be associated with referred head pain.
Treatment of Cervicogenic

Headache

The successful treatment of cervicogenic headache usually requires a

multifaceted approach using pharmacologic, nonpharmacologic, manipulative,
Interventional and occasionally surgical interventions. Medications alone are
often ineffective or provide only modest benefit for this condition.

Anesthetic injections can temporarily reduce pain intensity but have their
greatest benefit by allowing greater participation in physical treatment
modalities.

Developing an individualized treatment plan enhances successful outcomes.

Non-Pharmacological Treatments

Non-pharmacologic management strategies for both ON and CGH include

massage, cool compresses, cranio-cervical exercises, and physiotherapy to
improve posture. Transcutaneous electrical nerve stimulation therapy

(TENS) has been used in the conservative management of

CGH, with reported benefit.

Pharmacologic Treatment

Pharmacologic treatment modalities for cervicogenic headache include many

medications that are used for the preventive or palliative management of tension-

type headache, migraine, and “neuropathic” pain syndromes. The listed

medications have neither been approved by the US Food and Drug Administration
(FDA) nor rigorously studied in controlled clinical trials for the treatment of
cervicogenic headache and are only suggested as potential treatments based on
the anecdotal experiences of clinicians who treat this condition or similar pain
disorders.
Analgesics—Simple analgesics such as acetaminophen or nonsteroidal anti-
inflammatory drugs (NSAIDs) may be used as regularly scheduled medications for
round-the-clock management of chronic pain or as needed for the management
of acute pain

Antidepressants—The TCAs have long been used for management of various

neuropathic, musculoskeletal, head, and face pain syndromes. Analgesic dosages
are typically lower than those required for the treatment of patients with
depression. The serotonin and norepinephrine reuptake inhibitors (SNRIs)

such as venlafaxine hydrochloride and duloxetine hydrochloride have been

observed helpful in the prophylactic management of migraine.

Antiepileptic Drugs—The antiepileptic drugs (AEDs) are believed to be

modulators or stabilizers of peripheral and central pain transmission and are
commonly used for the management of neuropathic, head, and face pain
syndromes. Divalproex sodium is indicated for the preventive management of
migraine headache and may be effective for cluster headaches as well as other
neurogenic pain syndromes. Serum drug levels can be used as a therapeutic
dosing guide. Monthly monitoring of liver transaminase levels and of complete
blood cell (CBC) counts for evidence of toxicity is recommended, especially during
the first 3 to 4 months of treatment or whenever dosages are escalated.
Topiramate is indicated for migraine prophylaxis and has been reported effective
in the management of painful diabetic neuropathy and cluster headache.
Intermittent monitoring of serum electrolyte levels might be needed because of
this medication’s diuretic effect through carbonic anhydrase inhibition.

Other Medications—Muscle relaxants, especially those with central activity such

as tizanidine hydrochloride and baclofen, may provide some analgesic efficacy.
Botulinum toxin, type A injected into peri-cranial and cervical muscles is a
promising treatment for patients with migraine and cervicogenic Headache but
further clinical and scientific study is needed.

Proinflammatory mediators such as cytokines and TNF-α have been hypothesized

to be involved in the pathophysiology of CGH. A small open-label pilot study in six
patients with CGH found that infliximab treatment, which targets TNF-α, was
associated with rapid and sustained effects on headache pain scores and self-
administered analgesic consumption. However, further studies are certainly
needed to cautiously explore these results.

Minimally Invasive Interventions

Anesthetic block of the greater and/or lesser occipital nerves are often used both
diagnostically and therapeutically. However, evidence is limited as most studies
are non-controlled. Occipital nerve blocks with or without corticosteroids yield
transient benefit in most, with 15–36% sustaining extended relief for several
months. Facet block or anesthetic block of the upper cervical nerves with
corticosteroid has also been used as a therapeutic approach in CGH. Intra-
articular corticosteroid injections may be beneficial in reducing short-term pain,
but may have lesser benefit long-term.

For patients failing the above interventions, minimally invasive surgical options
include neuromodulation with subcutaneous occipital nerve stimulation (ONS), or
pulsed radiofrequency (PRF) therapy. PRF therapy exposes the nerve to high-
voltage radiofrequency pulses, which is hypothesized to induce an inhibitory
electrical field around the nociceptive afferents, disrupting pain transmission and
potentiation.

A more recent study including patients with CGH who had failed all other
conservation therapies suggested that RF ablation of the C2 dorsal root ganglion
and/or third occipital nerve may provide greater than 50% pain relief in the vast
majority of recipients, lasting up to 5–6 months. However, the rate of adverse
events was not insignificant, ranging from 12 to 13%.

Occipital Nerve Stimulation involves the subcutaneous insertion of electrodes in

the C1/C2 region of the posterior cervical spine and is attractive as a
nondestructive, reversible therapeutic approach for refractory ON. The
therapeutic mechanism is aligned with the gate theory of pain. Studies have
generally been concordant in showing a 62.5–100% improvement in
symptomatology post procedure. Complications related to ONS include lead
migration (4%), post-surgical infection (12%), and less commonly, lead fracture or
disconnection (2%).

Third Occipital Nerve Neurolysis

The third occipital nerve is the superficial medial branch of C3 dorsal ramus. It
supplies the C2–C3 zygapophysial joint while crossing the joint laterally. Also, it
supplies part of the semispinalis capitis muscle and its cutaneous branch supplies
a small area of skin below the occiput.
Third occipital radiofrequency neurolysis was shown to be effective in the
treatment of headache stemming from the C2–C3 joint. The most common side
effect is incomplete lesioning of the third occipital nerve because of its variable
anatomy. Using the three-needle-technique accommodates all variation in the
anatomy of the third occipital nerve from just lateral to the joint line to above or
below the joint, and creating consecutive lesions no more than one electrode
width from adjacent lesion markedly improved the results.

Invasive

More invasive surgical approaches such as neurolysis, posterior partial rhizotomy,

and dorsal root entry zone lesioning, have mixed results which should be weighed
against the possibility for poor longevity and frequent, often significant, side
effects.

References

1. Biondi DM: Cervicogenic headache: mechanisms, evaluation, and treatment

strategies. J Am Osteopath Assoc. 2000;100(9 Suppl):S7-14.)
2. Rebecca Barmherzig1,2 & William Kingston et al; Occipital Neuralgia and
Cervicogenic Headache: Diagnosis and Management, Current Neurology
and Neuroscience Reports (2019) 19: 20
3. Basics of Pain Management by Dr. Gautam Das
4. “Cervicogenic Headache” Samer Narouze MD, PhD, in Essentials of Pain
Medicine (Fourth Edition), 2018