Cervicogenic Headache/Cervicogenic Orofacial Pain: Evidence based facts

Cervicogenic Headache (CGH)/Cervicogenic Orofacial Pain (COP) is the pain related to cervical cranium and facial are common and are very disabling.
They are often concomitantly present at the same time and share the pathophysiology process, anatomical distribution and structures involved.
In this review, a synopsis of the available evidence of cervicogenic headache will be provided. The basic mechanisms, analyze the evidence on diagnosis and treatment, and recommendations on management will be discussed.

Epidemiology of cervicogenic headache (CGH)

They are not uncommon. 2-4% of general population and up to 20% of chronic headache patients have CGH. It is more common is female (M: F 1:4) and peak age in early 40s. These patients have suboptimal quality of life due to pain. Many patients have overlapping headache disorders. (Figure 1)

Fig 1. Overlapping of head and neck pains

Facial Pain/Orofacial pain (OFP)
This pain is distinct from headache. OFP is defined based on the anatomical location of the pain.
OFP occurs in area under the orbitomeatal line, anterior to pinnae and the above the neck. It has prevalence of 25% among facial pain disorders and often associated with temporomandibular disorder (TMD). 44% of CGH patients have associated TMD also.

Fig 2. Classification of head-face-neck pain

Diagnostic criteria for CGH

There are 2 sets of criteria.
A. The cervicogenic headache international study group criteria (1998)
B. International classification of headache disorders (ICHD) 2018.

Each has somewhat different inclusion and exclusion criteria

Pragmatic diagnostic criteria base on above 2 criteria

1. Headache associated with cervical spine disorders such as bone, soft tissue, disc. (Neck pain may or may not be present)
2. Clinical or imaging evidence of cervical spine or neck pathology.
3. Clinical symptoms are heterogenous.
   • Unilateral side lock headache
   • Neck-related headache triggered by movement or pressure
   • Limited cervical range of motion (ROM)
   • Ipsilateral neck/shoulder/arm pain may be present
4. Evidence of causation
   • Temporal relationship between CGH and cervical spine disorders
   • Improvement of CGH with cervical spine disease treatment
   • Worsening of CGH pain with provocative maneuver
   • Nerve block procedure (occipital nerve or cervical nerve roots) eliminate CGH

 

Differential diagnosis of CGH
Primary headache disorders

• Migraine
• Tension type headache (TTH)
• Hemicrania continua
• Trigeminal autonomic cephalgias (TACs)
• Primary exercise headache
• New daily persistent headache (NDPH)
• Neck-tongue syndrome

Secondary headache disorders

• Headache due to
• Whiplash neck injury
• Arterial dissection
• Chiari 1 malformation
• Infection
• Retropharyngeal tendonitis
• Craniocervical dystonia
• Posterior fossa lesions
• Occipital neuralgia
• TMD
• Myofascial pain
• Rheumatoid arthritis
• Syringomyelia
• Persistent idiopathic facial pain

 

The most important differential diagnosis of cervicogenic headache is dissecting aneurysms of the vertebral or internal carotid arteries, which can present with neck pain and headache. These aneurysms are indicated by the onset of cerebrovascular features, which typically emerge within 1–3 weeks. If this differential diagnosis is not considered, there is a risk of patients being treated with cervical manipulation, with fatal consequences due to aggravation of the aneurysm.

The second most important differential diagnosis is lesions of the posterior cranial fossa, as the dura mater and vessels of the posterior fossa are innervated by upper cervical nerves. These lesions are distinguished by the onset of neurological features or systemic illness. Meningitis of the upper cervical spine can be distinguished from cervicogenic headache by the presence of systemic illness and neck rigidity. Additionally, herpes zoster can produce pain in the occipital region during its prodromal phase; however, the eruption of vesicles distinguishes this disease from cervicogenic headache.

What distinguishes CGH from other headache disorders?

• Side-locked pain (unilateral facial or head pain)
• Provocation of headache by palpation on neck muscles and head movement
• Posterior to anterior radiation of pain (Occipital, Temporal, Frontal, Orbital)
• Nuchal onset of pain in 97% of the cases
• Intensity: Moderate to severe intensity
• Character: Deep pressure and non-throbbing
• Pain can be constant or intermittent or both
• Can have associated with migraine symptoms such as nausea, vomiting, photophobia or phonophobia, but not all of the cases and if presence, they are not severe as like in migraine

Variation in clinical symptoms of CGH

• If bilateral, one side frequently predominates
• Contralateral headache may occur in some patients
• +/- Ipsilateral diffuse shoulder and arm pain
• Numbness in one or both arms
• Dizziness is common
• Chronic and recurrent and difficult to treat with routine therapy

Clinical pearl for CGH

• If migraine headache patient does not respond well to typical migraine headache treatment, think about CGH
• Cervical triggers/precipitants may evidence in history or the examination
• Isolated neck or occipital pain should not be mistaken as CGH
• Minor Imaging abnormalities in upper cervical spine are common in patient with CGH (especially after the age of 40)
• Patients often have past medical history of migraine or TTH
• Do not confuse CGH and whiplash neck pain

Pathophysiology of CGH

Mechanism of pain referral (Figure 3,4,5, and 6)

The mechanism underlying the pain involves convergence between cervical and trigeminal afferents in the trigeminocervical nucleus.
In this nucleus, nociceptive afferents from the C1, C2, and C3 spinal nerves converge onto second-order neurons that also receive afferents from adjacent cervical nerves and from the first division of the trigeminal nerve (V), via the trigeminal nerve spinal tract.
Convergence between cervical afferents allows for upper cervical pain to be referred to regions of the head innervated by cervical nerves (occipital and auricular regions).
Convergence with trigeminal afferents allows for referral into the parietal, frontal, and orbital regions.

Fig 3. Mechanism of pain referral from the cervical spine to the head
Pain stimulation and receiving is vice versa in nature. (Figure 4 and 5)

Fig. 4. Noxious stimulation of greater occipital nerve in the neck induces increased excitability of supratentorial afferents

Fig. 5. Increased cervical nociception after stimulation of supraorbital nerve

Pattern of pain referral (Figure 6)
Noxious stimulation of more rostral structures in the cervical spine elicited referred pain in the occipital region and more distant regions, such as the frontal region and orbit.
Stimulation of more caudal structures (such as the at lanto-occipital, the lateral at lanto-axial joints, the C2–3 zygapophysial joint, and the C2–3 intervertebral disc) elicit pain in the neck, which could be referred only to the occipital regions, but not to distant regions of the head.

Fig 6. Pattern of pain referral

Red arrow: Point of pain stimulation
Number in circles: the percentage of individual who reported pain in the areas shown after stimulation at each segmental level.
The more cephalad the site of stimulation, the more likely that pain is referred to the distant regions of the head.

Diagnosis of CGH

1.Physical exam

1. Cervical spine range of motion (Decreased ROM)
2. Cervical muscle tone (Increased muscle tone)
3. Greater occipital nerve tenderness
4. C2-C3 facets area tenderness
5. Forward head neck posture (Janda’s upper cross syndrome -Figure 7)
6. Trigeminal autonomic features
7. Posterior scalp paresthesia or numbness
8. Increased magnitudes of thoracolumbar posture displacement

CGH can be diagnosed with 100% sensitivity and 94% specificity by examining neck dysfunction in the following three points.

1. Cervical range of motion: pattern of reduced movement by cervical extension
2. Manual examination: painful upper cervical joint dysfunction by palpation
3. Impaired muscle function in craniocervical flexion test

Janda’s upper cross syndrome (Figure-7)
The upper crossed syndrome consists of inhibited or weakened deep neck flexors, lower trapezius, and serratus anterior. Tightened muscles of the upper crossed syndrome include sternocleidomastoid, pectoral muscles, upper trapezius, and levator scapulae.

Fig 7. Janda’s upper cross syndrome

Summary of physical examination finding in CGH includes-

1. Impaired muscle function (of flexor and extensor neck muscle with/without axioscapular muscle impairment.
2. Painful joint dysfunction of C0-C4 (altered joint motion and local/referred pain)
3. Restricted neck motion of cervical spine (by flexion rotation and extension rotation test)

Fig 8. physical examination finding in CGH

2.Laboratory Investigation

It is not always required unless suspicion of rheumatological diseases such as rheumatoid arthritis or infections.
CP, ESR, CRP and RF can be ordered.

3.Imaging investigation

Radiology investigations such as X-ray, CT, Ultrasound or MRI are not always required unless suspicion of cervical radiculopathy.
Treatment of CGH

1. Non-Pharmacologic treatment
2. Pharmacological treatment
3. Injection treatment
4. Interventional treatment
5. Surgical treatment

1.Non-pharmacologic treatment

• Physical therapy
• Cool compress
• Massage
• TENS
• Cranio-cervical exercise and therapy
• Dry needling
• Acupuncture
• Spinal manipulation

2.Pharmacological treatment

1. Antidepressants (tricyclic antidepressant and SNRIs such as Duloxetine and Venlafaxine)
2. Antiseizure medications (gabapentin, pregabalin, carbamazepine, topiramate, sodium valproate)
3. Muscle relaxants (Tizanidine, Baclofen, Cyclobenzaprine)
4. Non-steroidal anti-inflammatory drugs (NSAIDs)

3.Injection treatment
Trigger points injection (Figure-9)

Fig 9. Common sites for trigger point injection
Neurotoxin injection (Figure-10)

Fig 10. Common sites for Botulinum toxin injection

4.Interventional procedure treatment
Anesthetic nerve blockade (Figure-11)

Fig 11. Indirect cervical nerve block

Neuromodulation
Neuromodulation approaches range from non-invasive techniques such as transcranial magnetic stimulation to implanted devices, such as a spinal cord stimulation or a deep brain stimulation system.
Methods of neuromodulation

Invasive

1. Spinal cord stimulation
2. Motor cortex stimulation
3. Deep brain stimulation

Non-invasive

1. Transcranial magnetic stimulation
2. Transcranial current stimulation

 

 

Neurolytic procedure
A neurolytic procedure is used to target the destruction of a nerve or nerve plexus.
Chemicals, radiofrequency (heat application) ablation (RFA), cryoablation (cold freezing of nerves), nerve disruption using pulsed radiofrequency and neurosurgical procedures are often used with fluoroscopy to improve the precision and efficiency of targeted neurolysis.
Patients often received substantial reductions in pain.

Fig.12. Chemical neurolysis

Cervical surgery
Primary indications for surgery are (1) cervical compressive myelopathy and (2) cervical radiculopathy.

References

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Author Information

Dr. Thar Thar Oo
MBBS, MD, MPH, FAAN
Senior Consultant Neurologist