A Problem-Based Learning Discussion

Diagnosing and Treating Cervicogenic Headache: A Problem-Based Learning Discussion

Nov 11, 2024, 16:04 PM by Farrah Asaad, DO, Eric Jones, MD, Kirk Sheplay, MD, Georges Abdelahad, MD

 

31-year-old female with no past medical history presents with chief complaint of right-sided suboccipital headache. Headache episodes began five years ago but worsened six months ago when she began a new job requiring frequent lifting, causing her to seek treatment. Pain described as aching pressure in the neck localized to the base of the skull radiating to the right occipital region. Rated as 4/10 at best, 8/10 at worst. Pain is constant but worsens as the day progresses. Most severe headaches occur about four times per week, lasting three to five hours. The patient notes an associated “popping” sensation in the neck with movement. Denies photophobia, phonophobia, nausea, vomiting, auras preceding headaches, vision changes, or tearing of vision. Aggravating factors include lifting heavy boxes at work, turning head while driving, or sitting for extended periods of time at her desk at work. She is not currently exercising. The only medication she takes for headache relief is combination acetylsalicylic acid (250 mg)/acetaminophen (250 mg)/caffeine (65 mg) when headaches are most severe, about three to four times per week, which helps relieve the pain.

Physical exam is notable for moderate flattening of cervical lordosis with forward-rounded shoulders, cervical range of motion with reduced extension to 40 degrees limited by pain and reproduction of headache symptoms. Full range of motion with cervical flexion. Right cervical lateral bending and rotation is limited at the end of range of motion with noted “popping sensations” and discomfort throughout. Full range of motion with left cervical lateral bending and rotation. Tenderness to palpation over right upper cervical paraspinals. No tenderness to palpation of the greater or less occipital nerves bilaterally.

No imaging or laboratory studies available.

 

Questions:

1. Given this patient’s presentation, what are the most worrisome features? What are the differentials?

The patient has experienced intermittent headaches over the past five years, but these symptoms have worsened in the last six months. This change, along with the increase in headache severity during exercise, raises concerns that warrant further investigation to rule out potential differential diagnosis discussed below.

The most critical differential diagnosis is a dissecting aneurysm of the vertebral or internal carotid arteries, which can present with headaches and neck pain. Additionally, lesions in the posterior cranial fossa should be noted, though they typically present with additional neurological symptoms.10

For unilateral headaches, potential causes include cluster headaches, chronic paroxysmal hemicrania (CPH), and hemicrania continua (HC). Unlike cervicogenic headaches, these three types do not respond to anesthetic blockades. To confirm cervicogenic headaches, specific diagnostic criteria related to anesthetic response must be satisfied. A positive response to indomethacin can help differentiate HC and CPH from cervicogenic headaches, as the latter usually do not respond to this medication.1,2

Migraines are another possibility; however, cervicogenic headaches typically originate in the neck or occipital region, while migraines usually start in the frontotemporal area. Other distinguishing features include the tendency of migraines to shift from side to side during episodes, whereas cervicogenic headaches tend to remain on one side. Lastly, tension-type headaches should also be considered. They differ from cervicogenic headaches in that they are usually bilateral, lack mechanical triggers, and are often described as dull or pressing in quality.1,2

2. What are the different headache types?

Headaches are divided into primary and secondary types. Primary headaches are all those that are not due to another disease including migraine, tension-type headache, and trigeminal autonomic cephalalgias.

Primary Headaches:

Migraine:

Migraine is the third most prevalent disorder and the seventh highest cause of disability worldwide. The pathophysiology is thought to be due to centralized phenomenon that involves an altered sensory processing and excitability of the brain in multiple brain areas, but predominantly in the trigeminovascular system. The symptoms of a migraine are typically unilateral, pulsating, aggravated by movement, bright light, loud noises, and often accompanied by nausea and vomiting. The duration often lasts 4-72 hours and can be episodic or chronic. The overall clinical picture of migraine may be divided into four phases: prodrome, aura, headache phase, and postdrome. Approximately one-third of patients experience migraine with aura. An aura is a fully reversible set of neurological symptoms, most often visual or sensory, that typically develops gradually, recedes, and is then followed by headache accompanied by nausea, vomiting, photophobia, and phonophobia. Less common symptoms include speech/language symptoms, motor or brainstem symptoms, and/or retinal symptoms.18,19

Tension-Type Headache (TTH):

TTH is the most common primary headache disorder, with a lifetime prevalence in the general population of up to 80%. The pathophysiology is multifactorial including pericranial tenderness, a generalized pressure pain hypersensitivity, muscle tightness, and central sensitivity. The symptoms are bilateral, pressing quality often described as a tight headband not aggravated by movement, and no accompanying symptoms. Rarely, these patients may present with photophobia or phonophobia. The duration often lasts from 30 minutes to seven days and can be episodic or chronic.18,19

Trigeminal Autonomic Cephalalgias (TACs):

The TACs are a group of disorders including cluster headache (CH), paroxysmal hemicrania (PH), short-lasting unilateral neuralgiform headaches with conjunctival injection and tearing (SUNCT), short-lasting unilateral neuralgiform headaches with cranial autonomic features (SUNA), and hemicrania continua (HC). They are characterized by a lateralized symptom of prominent headache in the orbital, supraorbital, and temporal regions (but may include other sites within the trigeminal nerve distribution) and accompanied by ipsilateral cranial autonomic features of conjunctival injection, periorbital edema, facial flushing, optic fullness, lacrimation, and rhinorrhea. An important distinction from other primary headaches is the unilaterality of photophobia or phonophobia ipsilateral to the pain, and it may be up to ten times as common in TACs than in migraine. Another hallmark of the TACs is the presence of agitation in patients who experience them, likely caused by activation of the posterior hypothalamus.18.19

Other Primary Headaches:

These include headaches associated with exertion such as cough, exercise, sexual activity or “thunderclap” headache. It also includes epicranial headaches such as pain over the scalp and from an outside stimulus like cold or external pressure. These headaches are grouped in this subcategory as they all share a poorly understood pathogenesis.20

Secondary Headaches:

Secondary headaches are due to an underlying medical condition and are classified into eight major headache classes according to the International Classification of Headache Disorders 3rd edition (ICHD-3), and further subdivided into more than 100 secondary headaches. The eight major classes include:17

  1. Headache Attributed to Trauma or Injury to the Head and/or Neck
  2. Headache Attributed to Cranial or Cervical Vascular Disorder
  3. Headache Attributed to Non-Vascular Intracranial Disorder
  4. Headache Attributed to a Substance or its Withdrawal
  5. Headache Attributed to Infection
  6. Headache Attributed to Disorder of Homeostasis
  7. Headache or Facial Pain Attributed to Disorder of the Cranium, Neck, Eye, Ears, Nose, Sinuses, Teeth, Mouth, or Other Facial or Cervical Structure
  8. Headache Attributed to Psychiatric Disorders
  9. Painful lesions of the Cranial Nerves and other Facial Pain
  10. Other Headache disorders

3. What criteria make up the diagnosis of a cervicogenic headache for this patient?

Agreement on the clinical diagnostic criteria for cervicogenic headaches has been difficult to reach throughout the years; however, there are a handful of reliable features. Pain should begin in the neck and radiate to the fronto-temporal region, pain that radiates to the ipsilateral shoulder and arm and pain that worsens with neck movement.10 A panel of seven features was created as a way to approach a clinical diagnosis which states patients that have unilateral headaches and pain that starts at the neck and any of three additional features in the panel, then a probable diagnosis of cervicogenic headache could be made.10

Clinical Criteria for the diagnosis of cervicogenic headache:

  1. Unilateral headache without side shift
  2. Symptoms and signs of neck involvement: pain triggered by neck movement or sustained awkward posture and/or external pressure of the posterior neck or occipital region; ipsilateral neck, shoulder and arm pain; reduced range of motion
  3. Pain episodes of varying duration or fluctuating continuous pain
  4. Moderate, non-excruciating pain, usually of non-throbbing nature
  5. Pain starting in the neck, spreading to oculo-fronto-temporal areas
  6. Anesthetic blockades abolish the pain transiently provided complete anesthesia is obtained, or occurrence of sustained neck trauma shortly before onset
  7. Various attack-related events: autonomic symptoms and sings, nausea, vomiting, ipsilateral oedema and flushing in the periocular area, dizziness, photophobia, phonophobia, or blurred vision in the ipsilateral eye

4. The patient asks you about what types of conservative treatment options are available.

Physical and manipulative therapy is considered the first line of treatment, and may include cervical and thoracic spinal manipulation, massage with myofascial release, and neck and shoulder stretching and exercises. Low-load resistance exercise protocol that aims to strengthen the deep muscles of the neck and train the scapular muscles for six weeks of treatment, with eight to 12 sessions, is recommended. According to a study by Jull et al., both manipulative therapy and low-load resistance exercise therapy significantly reduced headache frequency and intensity in 72% of patients at the 12-month follow-up, 42% of these patients reported 80% or higher relief.13 These manipulative maneuvers stimulate neural inhibitory systems at various levels in the spinal cord and activate descending inhibitory pathways. High velocity manipulation is not recommended however, due to the associated risk of vertebral artery dissection and stroke in these patients. Physical therapy may initially worsen the headache, so it is important to educate the patient that this is a possibility, and treatment should be slowly advanced.

Additionally, certain medications have been shown not to have a significant effect on treatment of cervicogenic headaches. Acetaminophen and NSAIDs may provide temporary relief; however, there have not been any clinical studies that determine their efficacy. Similarly, morphine only has a marginal effect and is generally not indicated for cervicogenic headaches.2 Ergot derivative medications play a large role in treatment of migraines but have been shown to have no effect in the treatment of cervicogenic headaches.2 Triptans, while effective for treating migraines, their effectiveness in treating cervicogenic headaches has not been established and would require further studies.2 Lastly, infliximab, an anti TNF-𝛼 biologic agent has been recently studied in a small pilot study of six patients. Treatment with infliximab provided rapid and sustained relief for cervicogenic headache, which now warrants further longer-term studies.14

Lastly, the use of transcutaneous electrical nerve stimulation (TENS) therapy is an additional conservative treatment option for cervicogenic headaches. TENS therapy works by using low voltage electrical currents to relieve pain by blocking or changing one’s perception of pain.15 In a randomized trial of TENS therapy, Tarhan and Inan found significant improvement in the treatment group after three months compared to placebo.2

5. The patient then returns six weeks later, noting minimal relief with conservative management, and interested in learning about additional treatment options.

Additional interventional options for patients with cervicogenic headaches depend on the primary etiology and can include botulinum toxin injections, local anesthetic injections, vascular decompression of C2, radiofrequency treatment, epidural steroid injections and dorsal cervical laminotomy and laminoplasty.2

Botulinum toxin type A acts by inhibiting the release of acetylcholine at the neuromuscular junction, leading to muscle relaxations. Treatment with botulinum toxin A for chronic tension-type headaches and migraines have been shown to be safe and efficacious. There have been several case reports for the use of botulinum toxin A injections for cervicogenic headaches with shown to have substantial benefits and reduction in pain scores on the visual analog scale (VAS).2

Local injections with local anesthetic around greater occipital nerves have also been used for the management of cervicogenic headaches. In a study of a subgroup of patients, they were reported to have significant relief of headache pain in a seven-day period following an injection with 0.5% bupivacaine; however, it was not considered a long-term modality.3

Different surgical therapies have been proposed to resolve cervicogenic headache pain. In two similar studies, patients underwent vascular decompression of varicose veins or arterial loops around the C2 root was performed with positive postoperative results.4,5 Neurolysis of the greater occipital nerve in the musculature surrounding the neck was thought to be beneficial due to entrapment of occipital nerve being considered as a possible mechanism of action for cervicogenic headaches. In an uncontrolled study with 50 patients, however, most of them redeveloped their pain within one week.5 Similarly, a controlled study was done on the efficacy of cervical epidural corticosteroid injections. Short term results showed significantly lower scores on the numeric pain intensity scale, however patients experienced clinical worsening within their six-month follow up.6,7 Conversely, several studies have been done on the effectiveness of radiofrequency lesion therapy. Sjaastad et. al. performed percutaneous radiofrequency treatment of the periosteum of the external surface of the occipital bone, and five of seven patients reported improvement over a four-and-a-half-year follow up period.8 Lastly, a more invasive treatment, dorsal decompressive laminotomy and laminoplasty have been shown to have good results; however, in the study report by Janson, the follow up period was only two and a half months post-op, therefore making it difficult to assess true efficacy of this therapy.9

6. The patient asks how she can expect her headaches to feel in the future. What are some prognostic indicators, or preventative measures she can take?

Certain factors have been shown to correlate with reduced burden of cervicogenic headache pain and improved function. These include increased age, relief or provocation of symptoms with movement, and gainful employment. In patients whose headaches are relieved or provoked by movement, reports have shown decreased frequency and intensity of headaches. This gives way to the finding from multiple studies that physical therapy interventions aimed at increasing cervical spine mobility provide benefit to patients with cervicogenic headaches. Maintaining employment status has been shown to specifically decrease headache frequency.16

While cervicogenic headaches cannot be entirely prevented, you can lower your risk by following some strategies. These include using proper safety precautions, maintaining good posture, sleeping in the right position, and exercising regularly.12

The relief from cervicogenic headaches largely depends on the treatments the patient receives. Medications may offer immediate relief, while injections can help, they may only provide temporary relief, therefore possibly warranting multiple injections over time.12 Following a home exercise program after initial physical therapy intervention has also been shown to improve physical and functional outcomes.16

 

 


 

Sources:

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  2. Martelletti, P., van Suijlekom, H. Cervicogenic Headache. CNS Drugs 18, 793–805 (2004). https://doi.org/10.2165/00023210-200418120-00004
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