Occipital neuralgia (ONa) is a distinct type of headache caused by injury or inflammation of the peripheral nerves of the posterior head, resulting in severe pain of the C2 and C3 dermatomal distribution. Pain can be unilateral or bilateral and usually paroxysmal, resulting in episodes of stabbing pain that last from seconds to minutes. ONa can present to ophthalmologists because the pain can be referred to behind the ipsilateral eye.
Etiology and Pathophysiology
Occipital neuralgia is caused by irritation/compression of cervical spinal nerves stemming from both the dorsal and ventral rami in the C2 and C3 region. Compression can be due to musculoskeletal disorders or trauma; rare causes include vasculitis or mass lesions. Impacted nerves include the lower occipital nerve (LON), greater occipital nerve (GON), or third occipital nerve (TON); symptoms can be caused by irritation to one nerve or a combination of multiple nerves. Approximately 90 percent of patients with ONa suffer from GON neuralgia.In rare situations, the TON will contribute to symptomatic pain; pain from this nerve can be difficult to isolate due to shared subbranches between the GON and LON. A study of facial pain in the Netherlands found the incidence of occipital neuralgia to be 3.2 per 100,000, with a mildly higher incidence in females that was not statistically significant.
Compared to other common headache and pain syndromes, ONa is a lesser known cause of posterior head and neck pain. The diagnosis of ONa requires a thorough history and examination to isolate occipital nerve symptoms and identify co-existing headache, migraine or pain disorders.
Signs and Symptoms
Pain from ONa is frequently characterized as intermittent sharp or stabbing, and can radiate up to the vertex of the head. It is frequently accompanied by symptoms related to cranial nerve nuclei VIII, IX, and X; these include visual impairment, dizziness, nausea, and nasal congestion. Pain from occipital neuralgia is mostly unilateral, but can occasionally spread to a bilateral distribution in up to 1/3 of cases. On physical exam, some patients exhibit a positive Tinel’s sign, which is numbness and tingling upon gentle tapping of the nerve between occipital protuberance and mastoid process; this is due to the emergence of the GON from the base of the skull.
Criteria have been published by the International Classification of Headache Disorder (ICHD-3). Diagnosis includes:
1. Stabbing, paroxysmal pain distributed over the GON, LON or TON that may or may not have episodes of aching in between.
2. Tenderness of impacted nerve distribution, sometimes accompanied by dysesthesias or allodynia.
3. Alleviation of pain via administration of a local nerve block to the GON, LON, or TON or their respective branches.
4. Inability to adequately explain symptoms from another disorder.
A diagnostic nerve block is required for confirmation of occipital neuralgia and to distinguish it from other similar conditions. While nerve blocks alleviate pain, it is also important to note that symptomatic relief can be a false positive in up to 40 percent of cases; this can be due to migraine or cluster headaches. A second nerve block is sometimes performed to increase confidence in the diagnosis.
Depending on the distribution of the affected nerve, various muscle groups in the upper back and posterior head can be symptomatically impacted. This is dependent on anatomical variations of C2 and C3 nerve innervation from both the dorsal and ventral rami of the respective spinal nerves. Multiple anesthetic injection sites have been detailed in the literature for diagnosis and pain relief, describing targets that are inferolateral to the external occipital protuberance.
MRI is the modality of choice to evaluate soft tissue pathology or joint instability. Detailed imaging can help with the accurate determination of nerve compression and/or irritation. In the setting of peripheral nerve entrapment, ultrasound has also been shown to assist with diagnosis and etiology; it can be used at the bedside to identify compressed nerves and precisely guide anesthetic injection. Most cases have no visible pathology on MRI.
Disorders causing headache, musculoskeletal pain or neuropathy can mimic symptoms of occipital neuralgia. Migraine headaches and cluster headaches are commonly mistaken for occipital neuralgia; repeat nerve block testing is important for confirmation of diagnosis. Tumors and congenital malformations can also trigger symptoms and should not be missed during workup. While most cases are thought to be idiopathic, it has been suggested that occipital neuralgia could occur as a result of cervical trauma or even lesions to the dorsal root ganglia associated with the C2/C3 distribution.
It is important to distinguish occipital neuralgia from cervicogenic headache. Cervicogenic headache can produce a similar pain profile in the occipital region, but these symptoms originate from a lesion within neck structures or the cervical spine. Due to these variations in etiology, a diagnostic nerve block is crucial to rule out cervicogenic headache.
There is currently no definitive course of treatment for occipital neuralgia, and therapy is generally tailored to the needs and severity of each case. Treatment often follows a step-wise approach, starting with conservative management and medical therapy; persistent pain may require ablation or surgical decompression as a last resort.
Occipital neuralgia can be initially treated with a combination of posture control and anti-inflammatory drugs to reduce inflammation and irritation on the impacted nerve. Anticonvulsants (carbamazepine, oxcarbazepine, gabapentin) and selective serotonin reuptake inhibitors also have demonstrated efficacy in the treatment of symptoms.
Nerve blocks can alleviate pain temporarily; some patients have experienced pain relief lasting up to a few months post-injection. This is not a permanent solution, however, as chronic use of local anesthetics causes neurotoxicity.
Botulinum toxin has been utilized effectively for headache and migraine pain relief; it has also been found to relieve pain caused by GON and trigeminal neuralgia. Current theory suggests that Botulinum A can inhibit neuropathic pain modulators such as substance P and glutamate; it also may reduce central sensitization of the injected nerve.
Pulsed radiofrequency therapy reduces aberrant nerve signaling to control pain. This method utilizes electric fields to induce tissue changes that inhibit excessive nerve signaling. These signals can reduce neuropathic pain over time in some patients.
Ablative therapy can potentially reduce pain signaling by destroying the pathologic afferent pathways. Thermoablative and cryoablative techniques have been used to treat patients and provide relief; a risk of these procedures is development of hypesthesias and dysesthesias from peripheral sensitization.
Neurolysis of the GON has also been used therapeutically in certain cases; ideal patients have already experienced relief from GON nerve blocks, have suffered from occipital region trauma, or experienced GON tenderness. Cervicogenic headache must be ruled out before neurolysis can be used for treatment.
Occipital nerve stimulation involves the implantation of a device designed to electrically stimulate cervical nerves2. This device can be controlled by the patient and can be removed to fully reverse the procedure. Chronic nerve stimulation has been shown to reduce neuropathic pain in patients and can be considered as a safer alternative to invasive surgical decompression.
Surgical nerve decompression is used in the setting of severe refractory pain. These procedures have the potential to provide significant symptomatic relief. The approach to resection varies, with some procedures pursuing obliquus capitis inferior resection and others performing gangliectomies of the C2 or C3 nerves. Decompression is not frequently performed due to the risk of worsened post-operative pain, neuromas, and causalgia.
Surgical options are recommended when conservative therapy, injections, and medical management have failed; this is primarily due to the risks of infection and post-operative complications, including worsening of preexisting pain. Decision making for any course of treatment must be shared between the patient and provider, in order to create an optimal plan that effectively weighs all risks and benefits.
There are limited studies focused on optimal treatment methods for this condition. While there is no curative treatment, symptomatic relief can be achieved with the various therapeutic options available.
Occipital neuralgia is a neuropathic pain disorder resulting in severe paroxysmal pain of the C2/C3 regions of the posterior head and neck that last from seconds to minutes. Diagnosis requires exclusion of other headache and pain disorders, and is confirmed with a nerve block to the GON, LON, or TON. Treatment options range from conservative management to neuroablation, nerve stimulator implantation, and surgical decompression. Stepwise management of symptoms is commonly employed; all decision making should weigh the risks and benefits of medical and invasive therapeutics. Ophthalmologists should be aware of ONa as patients can present with ipsilateral eye pain with a normal ocular exam.
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