Occipital neuralgia

INTRODUCTION — 

Occipital neuralgia is a form of neuropathic head and/or neck pain characterized by a unilateral or bilateral paroxysmal shooting or stabbing pain in the posterior part of the scalp, involving the greater, lesser, and/or third occipital nerve distributions, sometimes accompanied by diminished sensation or dysesthesia in the affected area, and commonly associated with tenderness over the involved nerve or nerves [1,2].

This topic will review the clinical aspects and treatment of occipital neuralgia. Other cranial neuralgias and central causes of facial pain are discussed separately. (See "Overview of craniofacial pain".)

ANATOMY — 

Occipital neuralgia localizes to one of three peripheral nerves that supply sensation to the posterior scalp (figure 1) [3-6]. These nerves arise from upper cervical nerve roots (C2 and C3) and supply sensation to the posterior head and neck (figure 2).

●Greater occipital nerve – The greater occipital nerve (GON) originates from the dorsal ramus of the second cervical nerve (C2). It perforates the semispinalis capitis and trapezius muscles and travels cephalad adjacent to the occipital artery. The GON supplies sensation to the uppermost neck and posterior head from the suboccipital area to the vertex. The GON is implicated in up to 90 percent of occipital neuralgia cases [7].

●Lesser occipital nerve – The lesser occipital nerve (LON) is formed by branches of the ventral rami of C2 and C3 that join and ascend along the posterior margin of the sternocleidomastoid muscle. The LON provides sensory fibers to the area of the scalp lateral to the greater occipital nerve, behind and superior to the auricle. The LON is implicated in up to 10 percent of occipital neuralgia cases [7].

●Third occipital nerve – The third occipital nerve (TON) originates from the dorsal ramus of C3, travels along the semispinalis capitis muscle, perforates the trapezius muscle, and then travels in a paramedian and cephalad orientation. The TON supplies sensation to the upper posterior neck and lower occipital region of the scalp, medial to the GON territory. The TON alone is an uncommon source of occipital neuralgia pain.

PATHOPHYSIOLOGY — 

The pathophysiology of occipital neuralgia is not well established. It is typically attributed to the chronic entrapment and compression of the occipital nerves by adjacent structures in the neck. Potential sources of peripheral nerve compression in occipital neuralgia include:

●Cervical vertebral joint degeneration and/or osteophytes [8,9]

●Postsurgical or traumatic inflammation [6,10]

●Posterior neck and scalp muscle spasm [11-13]

●Vascular compression (eg, occipital artery [14-17] or vascular malformation [18,19])

●Neoplasm (eg, meningioma) [20]

Occipital nerves may be vulnerable to compressive injury at segments that perforate neck muscles such as the semispinalis capitis and trapezius; and rotational or flexion movements in the upper cervical region may further contribute [21]. However, only limited evidence supports the concept of irritation of the occipital nerves as the cause, chiefly from surgical studies. Some reports described the onset of occipital neuralgia following Arnold-Chiari malformation surgery or other surgeries involving the cranial base, craniocervical junction, or upper cervical spine [22,23]. In one series of 872 patients with acoustic neuroma who underwent surgical resection, occipital neuralgia subsequently occurred in 15 patients (1.9 percent) [24].

Occipital neuralgia has also been reported to result from noncompressive mechanisms of the C2-C3 nerve roots such as herpes infection [25,26], neoplastic infiltration [27-29], or iatrogenic nerve injury [30,31].

Spinal cord lesions have also been rarely reported in patients with symptoms consistent with occipital neuralgia including cervical spinal cord demyelination and spinal cord infarction [32-35]. Central lesions in the spinal cord or brainstem may lead to central or peripheral sensitization involving the occipital nerves.

EPIDEMIOLOGY — 

Occipital neuralgia is thought by many nonspecialists to be common, but it is a relatively rare condition in clinical practice among many headache specialists. An overestimation in the prevalence of the condition may be due in part to a misapplication of the diagnosis to patients with any pain in the occipital region even when diagnostic criteria are not met or even when the pain is not neuralgic.

The literature on occipital neuralgia is conflicting, and the actual incidence and prevalence are unknown [36]. In a population-based study of adults aged 55 to 94 years, the lifetime prevalence of cranial neuralgias in general was 1.6 percent [37]. A population-based study in the Netherlands reported an incidence rate of 3.2 per 100,000 person-years [38]. The diagnosis was more common in older adults with a peak incidence for individuals aged 60 to 69 years of 9.7 per 100,000 person-years. The diagnosis was slightly more common among females (57 percent). However, a stronger predominance among females was reported in a 10-year prospective registry of 5515 patients from a headache outpatient clinic in which 68 patients (1.2 percent) were diagnosed with occipital neuralgia, and most (80 percent) were female [39].

Injuries such as whiplash during motor vehicle collisions may be a risk factor for occipital neuralgia [5]. Migraine and other primary headache disorders can be comorbid with occipital neuralgia [40].

CLINICAL MANIFESTATIONS — 

Occipital neuralgia is characterized by paroxysms of neuropathic head and/or neck pain.

Character of pain — The pain of occipital neuralgia is typically described as severe, stabbing, electric, shock-like, sharp, or shooting [41]. It originates in the nuchal region and immediately spreads toward the vertex (greater occipital nerve [GON] distribution) or behind the ear (lesser occipital nerve [LON] distribution). The bouts of pain may start spontaneously or be provoked by specific maneuvers such as brushing the hair, exposure to cold, or moving the neck. Some patients will avoid provoking behaviors such as wearing hats or lying supine on a pillow. The pain is usually unilateral, but bilateral cases uncommonly occur as well.

Bouts of pain typically persist for seconds to minutes before subsiding. A dull occipital discomfort may be present during periods between the painful paroxysms. In a case series of 68 patients with occipital neuralgia, baseline pain was present in 91 percent [39].

Most often, the condition appears to develop spontaneously, but some cases develop after specific events such as surgery at the craniocervical junction. (See 'Pathophysiology' above.)

Associated features — Other symptoms that may uncommonly occur along with bouts of neuropathic pain include tinnitus, nausea, or dizziness [42].

Features of other headache syndromes may also be present in patients with occipital neuralgia who have coexisting conditions such as migraine or cluster headache [40,41].

Examination findings — Percussion of the nerve often reproduces the distribution of pain (Tinel's sign). Pressure, palpation, or percussion over the occipital nerve trunks may also reveal local tenderness, trigger the background discomfort, or elicit paresthesias along the distribution of the affected nerve (figure 1) [2]. (See 'Anatomy' above.)

Cervical muscle range of motion may be restricted, and local posterior neck muscle spasms may be found in some patients.

Although somewhat contradictory to the strict definition of neuralgia, occipital neuralgia is frequently accompanied by diminished sensation or dysesthesia in the affected area [2,41]. The remainder of the neurologic examination is typically normal. An abnormal neurologic examination suggests an alternative or underlying cause of the symptoms [5,12,13]. (See 'Differential diagnosis' below.)

DIAGNOSIS — 

The diagnosis of occipital neuralgia is considered when typical clinical features are present; the diagnosis is confirmed when pain is transiently relieved by occipital nerve blockade with local anesthetic and after alternative diagnoses have been excluded (algorithm 1). (See 'Evaluation' below and 'Diagnostic criteria' below.)

We perform neuroimaging to identify structural causes that may preclude nerve blocks and to exclude alternative conditions. The clinical features of occipital neuralgia may occur in patients with other headache disorders [40,43]. (See 'Differential diagnosis' below.)

Evaluation — The evaluation of patients with features of occipital neuralgia consists of neuroimaging to evaluate for structural causes of symptoms followed by nerve block to localize symptoms to an occipital nerve (algorithm 1).

●Neuroimaging – For all patients with clinically suspected occipital neuralgia, we suggest magnetic resonance imaging (MRI) of the brain and cervical spine with contrast. Computed tomography (CT) of the brain and cervical spine with contrast may be performed as an alternative for patients unable to undergo MRI. Neuroimaging of the posterior fossa and upper cervical spine may identify structural causes of occipital neuralgia or alternative conditions that may require cause-specific treatment [44,45]. (See 'Differential diagnosis' below.)

Cerebrovascular imaging is typically reserved for patients with symptoms suggestive of a vascular cause such as those with Horner syndrome to identify a vertebral artery dissection or patients with constitutional symptoms to identify giant cell arteritis (GCA). Cerebrovascular imaging is also performed to confirm a vascular cause suspected by findings on brain or cervical spine imaging, such as a vascular malformation. Options include magnetic resonance (MR) angiography or CT angiography of the head and neck.

●Occipital nerve block – Anesthetic occipital nerve block (ONB) is performed in patients with clinical features suggestive of occipital neuralgia to support the diagnosis by identifying an occipital nerve branch as the localization of the symptoms [46]. Patients with occipital neuralgia report immediate partial or complete relief with anesthetic blockade of the affected nerve.

By definition and diagnostic criteria, ONB should provide at least temporary pain relief (see 'Diagnostic criteria' below). Other causes for neuralgiform pain (such as cervicogenic headache) should be explored if a properly performed anesthetic nerve block fails. However, a favorable response to ONB alone does not confirm the diagnosis of occipital neuralgia, as patients with other conditions that produce pain in the posterior head or neck may also report transient relief with nerve blockade. (See 'Differential diagnosis' below.)

ONB may produce prolonged benefits for some patients with occipital neuralgia and is used as a primary treatment for the condition. (See 'Occipital nerve block' below.)

For patients who are unable to undergo or decline diagnostic ONB, a presumptive diagnosis of occipital neuralgia is made by compatible clinical features and neuroimaging that excludes alternative structural conditions. Diagnostic reassessment may be warranted for such patients who do not respond to initial treatment options.

●The role of cervical joint anesthetic blockade – Cervical medial branch blocks may be performed to help identify cervicogenic headache as the source of pain for selected patients with a modest response to ONBs and atypical symptoms such as constant neck pain or pain that is consistently provoked by neck movement [47]. The pain in cervicogenic headache is due to dysfunction of one of the upper cervical zygapophyseal joints rather than the more peripheral occipital nerve impairment in occipital neuralgia. (See "Cervicogenic headache", section on 'Diagnostic anesthetic blockade'.)

Differential diagnosis — The differential diagnosis of occipital neuralgia includes other conditions that produce upper cervical spine and/or referred head pain. Possible sources of such pain include the cervical spinal cord as well as cervical joints, neck muscles, and vascular structures [11]. These conditions may be distinguished by specific clinical features, nonresponsiveness to diagnostic ONB, and/or imaging evaluation.

●Cervicogenic headache – Cervicogenic headache is thought to be caused by referred pain from the upper cervical joints. Patients with neck or occipital pain that is either constant (rather than intermittent) or consistently provoked by neck movement and those with an absence of impaired sensation on examination are likelier to have a cervical source of pain [48]. Diagnostic blockade directed at the nerve supply to the upper cervical joints can be used to support the diagnosis of cervicogenic headache. (See "Cervicogenic headache".)

●Cervical muscle strain – Patients with muscle tension, spasm, or strain involving posterior neck muscles may report occipital and/or neck pain including paroxysms of pain, typically following neck movement. However, unlike symptoms of occipital neuralgia, musculoskeletal pain is not neuralgic and may be relieved with rest, stretching, and local treatments such as cold compresses.

Of note, cervical muscle strain may coexist with occipital neuralgia, as chronic or severe muscle spasm may cause irritation of adjacent occipital nerves. (See 'Pathophysiology' above.)

●Other cervical spine conditions – Conditions of the cervical spine or craniocervical junction can also cause posterior head and/or neck pain, similar to that of occipital neuralgia [11]. The presence of continuous (rather than intermittent) occipital pain and the absence of impaired sensation suggest the pain is referred from structures in the cervical region [48]. These conditions may also be suspected by the presence of associated neurologic deficits and identified by imaging. These conditions include:

•Chiari malformation (see "Chiari malformations")

•Zygapophyseal arthropathy (see "Clinical manifestations of axial spondyloarthritis (ankylosing spondylitis and nonradiographic axial spondyloarthritis) in adults", section on 'Low back pain and neck pain')

•Upper cervical disc herniation (see "Clinical features and diagnosis of cervical radiculopathy")

●Migraine or tension-type headache – Some patients with migraine or tension-type headaches may have occipital or occipito-frontal predominating head pain, similar to patients with occipital neuralgia. However, these primary headache syndromes are characterized by throbbing or pressure-like nonneuralgic pain and episodes that persist for hours rather than seconds to minutes. (See "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults" and "Tension-type headache in adults: Etiology, clinical features, and diagnosis".)

Migraine and tension-type headaches may be comorbid with occipital neuralgia. (See 'Clinical manifestations' above.)

●Other craniocervical neuralgic conditions – Neuralgic conditions of other craniocervical nerves may produce electric or shock-like bouts of pain similar to occipital neuralgia but may be discriminated by distinct locations of pain and sensory abnormalities on examination. These conditions include C2 (nerve root) neuralgia or great auricular neuralgia. (See "Overview of craniofacial pain".)

●Vascular conditions – Acute vertebral artery dissection can produce posterior head and neck pain, similar to patients with occipital neuralgia. However, the pain is typically persistent and nonneuralgic, and patients may also have neurologic deficits from brainstem ischemia, including Horner syndrome, dysarthria, or visual impairment. Dissection may be identified on brain MRI and/or vascular imaging such as MR angiography of the head and neck. (See "Cerebral and cervical artery dissection: Clinical features and diagnosis".)

GCA may feature occipital-predominating headaches. However, patients with GCA frequently also present with additional features that may help to distinguish from occipital neuralgia. These include fever, weight loss, jaw claudication, visual impairment, and widespread myalgias. Inflammatory markers may be elevated on laboratory testing. GCA may be confirmed by temporal artery biopsy or ultrasound. (See "Clinical manifestations of giant cell arteritis".)

Diagnostic criteria — The diagnostic criteria for occipital neuralgia from the International Classification of Headache Disorders, 3^rd edition (ICHD-3) are as follows [2]:

●(A) Unilateral or bilateral pain in the distribution(s) of the greater, lesser, and/or third occipital nerves and fulfilling criteria B through D

●(B) Pain has at least two of the following three characteristics:

•Recurring in paroxysmal attacks lasting from a few seconds to minutes

•Severe intensity

•Shooting, stabbing, or sharp in quality

●(C) Pain is associated with both of the following:

•Dysesthesia and/or allodynia apparent during innocuous stimulation of the scalp and/or hair

•Either or both of the following:

-Tenderness over the affected nerve branches

-Trigger points at the emergence of the greater occipital nerve (GON) or in the distribution of C2

●(D) Pain is eased temporarily by local anesthetic block of the affected nerve(s)

●(E) Not better accounted for by another ICHD-3 diagnosis

The ICHD-3 classification requires distinguishing occipital neuralgia from occipital referral of pain from the atlantoaxial or upper zygapophyseal joints or from tender trigger points in neck muscles or their insertions.

TREATMENT — 

Treatment of occipital neuralgia consists chiefly of local and/or oral therapies to suppress symptoms. Patients with secondary occipital neuralgia who have structural causes identified on imaging evaluation may also warrant cause-specific treatments (algorithm 1).

Initial management — For patients with occipital neuralgia who have moderate to severe pain or debilitating symptoms, we suggest occipital nerve block (ONB) over other therapies. Pain relief, which is typically prompt, supports the diagnosis and may last several weeks or even months.

For others with mild and/or intermittent pain, those with a suboptimal response to prior ONB, and patients who prefer to avoid injections, we use alternative therapies.

Occipital nerve block — Anesthetic ONB helps support the diagnosis of occipital neuralgia in the right clinical context and provides immediate pain relief which may extend for weeks or even months [13,49]. (See 'Evaluation' above.)

Dosing and administration — ONB can be repeated when pain recurs [5,13]. Some patients report sustained relief after an initial (diagnostic) injection, while others report temporary benefit followed by a relapse of symptoms. We repeat ONB up to once every three months if using an anesthetic agent along with glucocorticoids, in agreement with a review by the American Headache Society [50]. ONB may be repeated more frequently if an anesthetic agent is used alone, but frequent injections may be inconvenient and an indication for adjunctive oral therapy.

The procedure should be performed by clinicians who are trained to perform the procedure.

Technique — ONB is typically directed at the greater occipital nerve (GON) but may also target the lesser occipital nerve (LON) or third occipital nerve (TON), based on the location of pain and tender points on palpation. Symptoms and examination findings restricted to the lateral border of the occipital region may favor LON injection while those restricted to paramedian location may favor TON injection.

●Identifying injection sites – Injection sites may be identified either by surface landmarks or with imaging guidance according to local protocol.

Landmark-based ONB is usually performed by targeting tender points approximating affected branches of the C2 nerve, either the greater GON or LON (figure 1). Injection sites are identified with surface localization using the external occipital protuberance as a reference landmark. The GON is approximated at two centimeters inferior and laterally; the LON is approximated at five centimeters laterally [51]. Other landmark-based techniques to identify injection sites include using one-third the distance from the external occipital protuberance to the mastoid process for the GON, and two-thirds the distance for the LON [49,52,53].

Imaging-guided ONB is usually performed with ultrasound guidance to identify relevant nerve and vascular structures [23]. The injection site may also be identified using CT guidance [54]. Limited data suggest imaging-guided ONB may be more effective than landmark-based ONB. In a small clinical trial of 32 patients with occipital neuralgia who underwent ONB or sham procedure, patients assigned to ONB by ultrasound guidance had a greater reduction in baseline pain at 30-minute and four-week follow-up than those assigned to ONB by landmark guidance [55]. Ultrasound guidance may permit injections closer to and more proximally along the nerve leading to more effective anesthetic and/or antiinflammatory effect.

●Medication choice – The selection of anesthetic agent varies by duration of intended action and local availability (table 1). ONB with an anesthetic agent may be performed either with or without additional glucocorticoid, according to local protocol.

The benefit of adding glucocorticoids to an anesthetic agent for ONB is uncertain. High-quality trial data are unavailable. Indirect evidence suggests additional glucocorticoids may result in longer benefits than ONB with anesthetic agent alone [56]. In one prospective study of 44 patients with occipital neuralgia, lidocaine or bupivacaine and 4 mg dexamethasone targeting either the GON or both GON and LON, the response rate was 95 percent at 24 hours and sustained at six-month follow-up [51]. Adjunctive medications were used by fewer than 20 percent of patients, and the mean interval to repeat injection was 270 days. In another single-center registry including 53 patients receiving an injection of lidocaine without a corticosteroid, the response rate of optimal pain relief was 73 percent, with more than 95 percent of patients reporting pain relief lasting two months [39].

●Procedure – Once the injection site is identified, the needle is advanced to the periosteum and then is retracted a few millimeters. After aspiration to confirm the needle has not been advanced intravascularly, the agent is then instilled in one area or divided in a fan-like series via the single injection site [57]. Immediate partial or complete symptom relief constitutes a favorable response to support the diagnosis of occipital neuralgia.

Procedural details and additional information on techniques for ONBs are discussed separately. (See "Scalp block and cervical plexus block techniques".)

Adverse effects — ONB is generally safe with few complications, as long as the agent is not inadvertently injected intravascularly [58]. Rarely, a patient may become cushingoid secondary to serial glucocorticoid-containing blocks [46,59]. In one retrospective review of 89 patients receiving a total of 315 nerve blocks, 9 percent reported adverse effects, most commonly transient dizziness or elevated blood pressure. While adverse effects were more common with bilateral injections, adverse effects were similar whether lidocaine concentration was 1, 2, or 5 percent [60].

Alternative or adjunctive treatment options — Alternative therapies may be used for patients with transient or partial relief with ONB. In addition, some patients may prefer oral agents over injections. However, oral therapies expose patients to the risk of systemic adverse effects, and evidence supporting the use of alternative therapies is limited to small case series and reports.

●Oral medications for most patients – Oral medications may be used in conjunction with or as an alternative to ONBs. Gabapentinoids or other oral agents commonly used to treat other neuralgic conditions and headache disorders may provide pain relief alone or in concert with ONB. However, most of these agents have not been studied systematically for occipital neuralgia. The selection of an agent should be individualized based on comorbid conditions and prior response to therapies. Options include [5,43,48,61]:

•Gabapentin

•Pregabalin

•Carbamazepine

•Baclofen

•Amitriptyline

The dosing and adverse effects of agents used for neuropathic pain are discussed separately. (See "Trigeminal neuralgia", section on 'Treatment'.)

The effectiveness of oral therapies for occipital neuralgia is variable. Most patients report a meaningful response in the author’s experience, but many patients may also require periodic ONBs for optimal pain relief.

Analgesic medications such as nonsteroidal antiinflammatory drugs have been reported to be ineffective or produce only transient symptom relief [62,63].

●Alternative botulinum toxin injections – Botulinum toxin injections may be beneficial for some patients with occipital neuralgia unresponsive to ONB and oral agents [64,65]. In one small series, ONBs with onabotulinumtoxinA appeared to provide a longer duration of analgesia than diagnostic local anesthetics [64]. In the other series, botulinum toxin injected into regions traversed by the greater and lesser occipital nerves was associated with improvement in shooting pain but no improvement in baseline dull and aching pain [65]. Ultrasound may have a role in finding specific occipital nerve entrapment locations to target with botulinum toxin injections [23].

●Patients with cervical muscle strain – For selected patients with occipital neuralgia due to acute traumatic cervical strain/sprain injury, initial therapy consists of applying heat locally to alleviate muscle spasm or cold locally one to three times daily to alleviate pain [5]. Nonsteroidal antiinflammatory drugs or acetaminophen may also provide adjunctive pain relief for patients with acute muscle strain. Other measures for these patients include immobilization with a neck collar during sleep or rest may provide relief from painful paroxysms [5,66]. (See "Management of nonradicular neck pain in adults".)

Options for refractory pain — Patients with an inadequate response to ONB or other initial therapies should be evaluated in experienced centers for consideration of interventional and surgical treatments. Options for refractory occipital neuralgia include pulsed radiofrequency (PRF) or occipital nerve decompression or stimulation. PRF may be used in some centers as a nondestructive neuromodulatory option for refractory cases. Decompression is typically used for patients with imaging evidence of a suspected structural source of compression. The selection of treatment varies by local availability and expertise.

●Pulsed radiofrequency – PRF treatment directed at the symptomatic occipital nerve branch may provide pain relief for patients with refractory occipital neuralgia [67]. In one trial of 81 patients with occipital neuralgia, patients assigned to PRF and ONB with an anesthetic agent had a better response rate at six weeks than those assigned to ONB with an anesthetic agent plus glucocorticoids (61 versus 36 percent; odds ratio [OR] 2.79, 95% CI 1.13-6.9), but this benefit declined (34 versus 14 percent) by three months and was nonsignificant at six months [68]. In a retrospective series of 102 patients treated with PRF, the response (≥50 percent pain relief) rate was sustained for at least three months in 51 percent [69]. Longer-term benefits with this technique are uncertain.

●Occipital nerve decompression – Occipital nerve surgical decompression may benefit patients with medically refractory occipital neuralgia and a suspected compressive cause [70-72]. In a small series of 11 patients with refractory occipital neuralgia who underwent decompression at the level of the semispinalis capitis and trapezial tunnel, nine patients reported significant or complete pain relief over a mean follow-up period of approximately 12 months [73]. In another surgical series, pain resolved in 6 of 11 patients at six months following decompression [74]. However, comparative data on surgical decompression are limited and should be reserved for patients with refractory pain who can be treated in a center with expertise in peripheral nerve neurosurgery.

●Occipital nerve stimulation – Nerve stimulation has been used in some patients with severe occipital neuralgia unresponsive to ONBs and oral therapies, but data are limited regarding this approach [27,75,76]. Additional data are warranted to better define the role of this technique for patients with occipital neuralgia.

Treatment of secondary occipital neuralgia — Patients with structural etiologies of occipital neuralgia may warrant cause-specific treatments as part of the management of symptoms. These may include:

●Vascular malformations

●Neoplasms (see "Management of known or presumed benign (WHO grade 1) meningioma" and "Peripheral nerve tumors", section on 'Schwannoma')

●Neuroinvasive infection (eg, herpes) (see "Treatment and prevention of herpes simplex virus type 1 in immunocompetent adolescents and adults")

●Transverse myelitis (see "Transverse myelitis: Treatment and prognosis")

In some cases, occipital neuralgia symptoms may resolve following treatment of underlying causes [26,28,77].

PROGNOSIS — 

The prognosis of occipital neuralgia is variable. Some patients may have transient symptoms when the inciting cause is temporary such as those with symptoms due to cervical muscle spasm or strain. However, the role of invasive procedures in the treatment armamentarium suggests others have chronic symptoms with bouts of neuralgic pain that recur intermittently and require multiple treatment modalities to achieve symptom relief.

SOCIETY GUIDELINE LINKS — 

Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Neuropathic pain".)

SUMMARY AND RECOMMENDATIONS

●Definition and anatomy – Occipital neuralgia is a form of neuropathic head and/or neck pain characterized by a unilateral or bilateral paroxysmal shooting or stabbing pain in the posterior part of the scalp, involving the greater, lesser, and/or third occipital nerve distribution (figure 2 and figure 1). (See 'Anatomy' above.)

●Clinical features – Occipital neuralgia produces episodic bouts of severe, stabbing, electric, shock-like, sharp, or shooting pain in the nuchal region that spreads toward the vertex. The bouts of pain may start spontaneously or be provoked by specific maneuvers such as brushing the hair, exposure to cold, or moving the neck. Examination may reveal local tenderness, trigger the background discomfort, or elicit paresthesias along the distribution of the affected nerve. (See 'Clinical manifestations' above.)

●Diagnosis – The diagnosis of occipital neuralgia is considered when typical clinical features are present; the diagnosis is confirmed when pain is transiently relieved by a local occipital anesthetic block after alternative diagnoses have been excluded (algorithm 1). (See 'Diagnosis' above.)

●Evaluation – The evaluation of patients with features of occipital neuralgia consists of neuroimaging to evaluate for structural causes of symptoms followed by nerve block to localize symptoms to an occipital nerve (algorithm 1). (See 'Evaluation' above.)

•For all patients with occipital neuralgia, we suggest MRI of the brain and cervical spine with contrast. CT of the brain and cervical spine with contrast may be performed as an alternative for patients unable to undergo MRI.

•Anesthetic occipital nerve block (ONB) is performed in patients with clinical features suggestive of occipital neuralgia to support the diagnosis by identifying an occipital nerve branch as the location of the symptoms. Patients with occipital neuralgia report immediate partial or complete relief with anesthetic blockade of the affected nerve.

●Differential diagnosis – The differential diagnosis of occipital neuralgia includes other conditions that produce upper cervical spine and/or referred head pain. These include cervicogenic headache, cervical muscle strain, other spinal conditions such as cervical disc herniation, migraine or tension-type headache, other neuralgic conditions such as C2 neuralgia, or vascular conditions such as vertebral artery dissection. (See 'Differential diagnosis' above.)

●Treatment – Treatment of occipital neuralgia consists chiefly of local and/or oral therapies to suppress symptoms. Patients with secondary occipital neuralgia who have structural causes identified on imaging evaluation may also warrant cause-specific treatments (algorithm 1).

•Occipital nerve block – For patients with occipital neuralgia who have moderate to severe pain or debilitating symptoms, we suggest ONB over other therapies (Grade 2B). Pain relief, which is typically prompt, supports the diagnosis and may last several weeks or even months. Indirect evidence suggests that glucocorticoids along with the local anesthetic provides more sustained relief than the local anesthetic alone.

Repeat ONB can be administered up to every three months for recurrent pain.

•Alternative or adjunctive options – For patients with mild and/or intermittent pain, those with a suboptimal response to prior ONB, and patients who prefer to avoid injections, we use alternative or adjunctive therapies that have been used for other neuralgic or headache conditions such as gabapentin, pregabalin, carbamazepine, baclofen, amitriptyline. The selection of a specific agent should be individualized. (See 'Alternative or adjunctive treatment options' above.)

•Refractory pain – Patients with an inadequate response to ONB or other initial therapies should be evaluated in experienced centers for consideration of interventional and surgical treatments. Options for refractory occipital neuralgia include pulsed radiofrequency (PRF) or occipital nerve decompression or stimulation. The selection of treatment varies by local availability and expertise. (See 'Options for refractory pain' above.)