Cervicogenic Headache, Post-meningeal Puncture Headache, and Spontaneous Intracranial Hypotension

Cervicogenic Headache

Definition

The International Headache Society (IHS) defines cervicogenic headache as “Headache caused by a disorder of the cervical spine and its component bony, disc and/or soft tissue elements, usually but not invariably accompanied by neck pain.”

Epidemiology

The incidence of cervicogenic headache is 2% when using the IHS criteria and up to 4% when using the Cervicogenic Headache International Study Group (CHISG) criteria. Furthermore, in studies conducted using the CHISG criteria for diagnosis, cervicogenic headaches were 66% more common in women. It encompasses 17.7% of all severe headaches. Epidemiologic studies are limited because of the wide variation in the diagnostic criteria. Cervicogenic headaches represent 25% of headache cases seen in pain management clinics.

Pathophysiology

The pathogenesis of cervicogenic headaches can be explained by the overlap between the first three cervical nerves, the trigeminal nucleus, and the cervical plexus. This is illustrated in Fig. 39.1 . The first three cervical nerves supply other structures, such as the upper cervical synovial joints and muscles, the dura mater of the upper spinal cord and posterior cranial fossa, and the intervertebral discs. The nociceptive afferents from the first three cervical nerves overlap with the second-order neurons that also receive afferents from the trigeminal nerve through the trigeminal nerve spinal tract. This overlap occurs in the trigeminocervical nucleus and allows for referral of cervical pain to the occipital and auricular regions as well as frontal, parietal, and orbital regions because of trigeminal afferents. The trapezius, sternocleidomastoid, and splenius capitis are also innervated by the cervical nerves, and trigger points within these muscles can cause cervicogenic headaches. This is because nociceptive afferents from these muscles also converge with the trigeminocervical nucleus.

Figure 39.1, Nociceptive afferents of the trigeminal and upper three cervical spinal nerves converge onto second-order neurons in the trigeminocervical nucleus in the upper cervical spinal cord. This convergence mediates the referral of pain signals from the neck to regions of the head innervated by cervical nerves or the trigeminal nerve.

Summary of Cervical Nerve Anatomy

First Cervical Nerve
Second Cervical Nerve
Third Cervical Nerve

Diagnostic Criteria

Cervicogenic headaches can be diagnosed based on clinical symptoms that meet the criteria or diagnostic block.

A. Clinical Diagnosis

Table 39.1 describes the clinical criteria put forth by the CHISG for the diagnosis of cervicogenic headache. In Table 39.2 , the IHS International Classification of Headache Disorders 3 diagnostic criteria are described.

B. Diagnostic Block

TABLE39.1

Cervicogenic Headache International Study Group (CHISG) criteria

Major Symptoms	Pain Characteristics	Other Important Criteria	Minor Symptoms and Signs
Unilateral pain	Non-clustering episodes	Female sex	Autonomic symptoms and signs
Symptoms and signs of neck involvement *	Varying duration	Head or neck trauma	Dizziness
	Moderate, non-throbbing, non-excruciating	Pain abolished by C2 or major occipital nerve	Phonophobia and Photophobia
	Starting in the neck and spreading to the oculo-frontotemporal region		Difficulty swallowing

* a) Provocation of attacks i) Pain triggered by neck movement; ii) pain elicited by external pressure over the ipsilateral upper, posterior neck, or occipital region. b) Reduced range of motion of the cervical spine c) Ipsilateral neck, shoulder, and arm pain of a rather vague, non-radicular nature Both major criteria must be present for diagnosis: unilateral pain with at least one sign or symptom of neck involvement. Other important criteria strongly support this diagnosis. Adapted from Sjaastad O, Fredriksen TA, Pfaffenrath V. Cervicogenic headache: diagnostic criteria. Headache . 1990;30(11):725–726.

TABLE39.2

IHS International Classification of Headache Disorders 3 Diagnostic Criteria

A
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.
C
Evidence of causation demonstrated by at least two of the following:
- Headache developed in a temporal relation to the onset of cervical disorder or appearance of the lesion
- Headache significantly improved or resolved in parallel with improvement in or resolution of cervical disorders or lesions
- Cervical range of motion is reduced
- Headache is made significantly worse by provocative maneuvers
- Headache was not explained by any other International Classification of Headache Disorders, 3 ^rd edition (ICHD-3).
D
Headache abolished following diagnostic blockade of a cervical structure or its nerve supply

Adapted from the Headache Classification Committee of the International Headache Society. The international classification of headache disorders, third Edition. Cephalalgia . 2018;38(1):1–211.

While clinical diagnostic criteria help diagnose cervicogenic headaches, a true diagnostic block can completely relieve headaches. It can be used to confirm the diagnosis of cervicogenic headache, rather than relying on clinical diagnosis alone. The third occipital nerve block can be used to diagnose pain arising from C2–C3 zygapophysial joint disease and is performed under fluoroscopic guidance. Patients who obtain complete relief of their headache after controlled blocks of the third occipital nerve can be treated successfully by radiofrequency neurotomy of the nerve.

Diagnostic medial branch block (MBB) of C3 and C4 can also be performed together with a third occipital nerve block to help with the associated component of neck pain. The techniques for C3 and C4 MBB and third occipital nerve block are described in detail under interventional management of cervicogenic headache.

Differential Diagnosis

Serious conditions should be ruled out in the presence of alarming features or red flags. Red flags include fever and chills, age >50 years, history of cancer, night pain leading to awakening, and weight loss. Special attention should be paid to high-risk patients, such as immunocompromised individuals, as they are more susceptible to more serious conditions, such as infections. The differential diagnosis includes:

Internal Carotid or Vertebral Artery Dissection

The vertebral and internal carotid arteries are innervated by the cervical nerves, and dissection of these vessels can result in headaches.
May have stroke-like symptoms within one to three weeks.
Treatment with cervical manipulation can be fatal.

Tumor in the Posterior Fossa, Arnold Chiari Formation

The dura mater in the posterior cranial fossa is supplied by the sinuvertebral nerves C1–3 .

Herniated Cervical Vertebral Disc

The C3 vertebral branch supplies the intervertebral discs of C2/C3. Discogenic pain may be an underlying cause.

Spinal Nerve Compression or Tumor, Intramedullary or Extramedullary Spinal Tumor

The dura mater of the upper cervical spinal cord is supplied by the sinuvertebral nerves C1–3 ⁵ .

Occipital Neuralgia

Greater occipital nerve entrapment.
The greater occipital nerve can be entrapped in its course between the obliquis capitis inferior and the semispinalis capitis. This is a commonly accepted cause of cervicogenic headaches resulting from occipital neuralgia.

C2 Nerve Lesions

Nerve-vessel compression on the C2 nerve root.
This nerve runs across the capsule of the lateral atlantoaxial joint, and its roots are covered in the dura mater and a plexus of the epiradicular veins. Inflammatory lesions of the joint and lesions of the dura or surrounding vessels may result in compression of this nerve. C2 neuralgia is characterized by intermittent and lancinating pain, unlike cervicogenic headache, because of C2/C3 facet disease, which is a constant and dull ache in nature.

Migraines and Tension-Type Headaches

Cervicogenic headaches can be differentiated from migraine and tension-type headaches. These include:
- Side-locked pain.
- Can be elicited by digital pressure on neck muscles and by head movement.
- Radiation of pain in a posterior-to-anterior radiation fashion.
- Lesser degree of nausea, vomiting, photophobia, and phonophobia. ^,
- Not throbbing and no side shifts, unlike migraine headaches.
- Lack of response to ergotamine and triptans, unlike migraine headaches.

Investigations

A full history and physical examination are necessary for the diagnosis of cervicogenic headache. History should include:

History of trauma (e.g. whiplash injury) or cervical disease.
The presence of associated neurologic symptoms or stroke-like symptoms (may indicate more serious diagnoses).

Physical examination should include the range of motion of the neck and attempt to produce a typical headache with head movement or head positioning. There should be a reasonable suspicion of cervicogenic headache prior to imaging studies. Imaging modalities, such as ultrasound, computed tomography (CT), and magnetic resonance imaging, may be helpful. Ultrasound may detect entrapment of the occipital nerve in occipital neuralgia. However, user variability affects reliability. CT imaging can be used to identify the bony pathology of the cervical spine. MRI is the modality of choice because it can visualize the bony pathology, soft tissue, and nerves of the cervical spine. Overall, it is important to remember that the imaging findings were not diagnostic. There should be a temporal relationship between positive findings and headaches to prove causation.

Treatment

Non-pharmacologic Treatment

Although there have been no controlled studies proving the efficacy of non-pharmacologic methods in the treatment of cervicogenic headaches, the potential benefits should still be considered.

Massage
Cold compress
Physical therapy, including manual therapy
- Exercises to improve posture
- Cranio-cervical exercises
Transcutaneous electrical nerve stimulation therapy
Biofeedback/relaxation therapy
Psychotherapy

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