What is occipital neuritis?

Occipital Neuralgia: Diagnosis, Pathophysiology, and Management

Occipital neuralgia is a disorder characterized by sharp, electrical, paroxysmal pain originating from the occiput and extending along the posterior scalp in the distribution of the greater, lesser, and/or third occipital nerves. 1

Pathophysiology and Etiology

• Occipital neuralgia typically arises from nerve compression by fibrosis, surrounding anatomic structures, or osseous pathology such as bone spurs or hypertrophic atlanto-epistropic ligament 2
• The greater occipital nerve (GON) is particularly vulnerable to compression due to its long course, peculiar anatomy, and location in a mobile region of the neck 2
• Most cases present with unilateral pain, though bilateral pain can occur and may radiate to the frontal region and face 3

Clinical Presentation

• Characterized by paroxysmal shooting or stabbing pain in the dermatomes of the greater or lesser occipital nerve 4
• Pain is typically described as sharp, electric shock-like sensations in the distribution of the occipital nerves 5
• Pain originates in the occiput and extends along the posterior scalp 1
• Physical examination often reveals tenderness over the greater and/or lesser occipital nerves 3

Diagnostic Approach

• Diagnosis is primarily clinical, based on the characteristic pattern of pain in the occipital region 3
• Physical examination is the first step in management, with patients typically demonstrating tenderness over the occipital nerves 3
• Diagnostic nerve blocks using local anesthetics (1-2% lidocaine or 0.25-0.5% bupivacaine) can help confirm the diagnosis 3
• It's crucial to differentiate occipital neuralgia from other conditions that may present with occipital pain:
  • Cervicogenic headache
  • Migraine with occipital pain
  • Optic neuritis (which affects vision and requires different management) 6

Imaging Considerations

• If visual symptoms persist or worsen independently of head pain, MRI of the orbits and head with contrast is warranted to rule out optic neuritis 6
• Unlike occipital neuralgia, optic neuritis shows abnormal enhancement and signal changes within the optic nerve on MRI 6

Treatment Options

First-Line Treatments

• Local nerve blocks with anesthetics (1-2% lidocaine or 0.25-0.5% bupivacaine) combined with anti-inflammatory corticosteroids 3
• Medical therapy with antiepileptics and tricyclic antidepressants is often effective 7
• Physical therapy to address any contributing musculoskeletal factors 7

Second-Line Interventions

• For medically refractory cases, occipital nerve stimulation (ONS) is a treatment option with Level III recommendation 1
• Multiple studies have shown significant pain reduction with ONS:
  • In one study, 91% of patients decreased their analgesic medication use, and 64% reported fewer headaches after ONS implantation 1
  • Another study showed 86% of patients experienced 100% improvement in pain following ONS 1

Refractory Cases

• Pulsed radiofrequency treatment may provide relief, though pain tends to recur during follow-up 4
• More invasive surgical options for medically refractory cases include:
  • C2 ganglionectomy
  • C2-C3 rhizotomy
  • C2-C3 root decompression
  • Neurectomy and neurolysis 4
• Surgical decompression through resection of the obliquus capitis inferior may be considered as definitive treatment, though it carries significant risks 3
• Alternative approaches like acupuncture have shown promise in some cases 5

Important Considerations and Caveats

• Diagnostic nerve blocks, while helpful, can also be effective in migraine headache, potentially leading to false positives 7
• Destructive surgical procedures carry grave risks - once an anatomic structure is destroyed, it cannot be easily recovered 4
• Surgical interventions may lead to the development of painful neuroma or causalgia, which can be even more difficult to control than the original complaint 4
• The most common technical complication with ONS is lead migration, occurring in 13.9% to 24% of cases 1

Treatment Algorithm

1. Initial approach: Trial of conservative management with medications (antiepileptics, tricyclic antidepressants) and physical therapy 7
2. If inadequate response: Proceed to diagnostic and therapeutic nerve blocks with local anesthetics and corticosteroids 3
3. For persistent symptoms: Consider occipital nerve stimulation, which has shown efficacy in multiple studies 1
4. For refractory cases: Evaluate for more invasive interventions such as pulsed radiofrequency or surgical options, weighing the benefits against potential risks 4