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492
I. Fortini

52.2 Pathophysiology

Figure 52.1 illustrates the occipital nerves and highlights their complex relationship with the occipital muscles. The greater occipital nerve (GON) originates from the dorsal ramus of the C2 spinal nerve. It then passes medially to the lower border of the obliquus capitis inferior muscle and crosses the suboccipital triangle. From there, it travels along the rectus capitis posterior major muscle, innervating it, before piercing the semispinalis capitis muscle and the tendon of the trapezius. The GON continues to ascend, passing the superior nuchal line of the occipital bone and pro­ceeding toward the apex of the scalp, near the occipital artery [7, 8].
The lesser occipital nerve (LON) originates from the ventral ramus of C2 and loops inferiorly to the spinal accessory nerve. It ascends along the posterior border of the sternocleidomastoid muscle, piercing the deep cervical fascia. The nerve then passes across the posterior edge of the sternocleidomastoid insertion and enters the supercial fascia of the scalp. It divides into three branches: the auricular, mastoid, and occipital branches [7, 8].
The third occipital nerve (TON) originates from the dorsal ramus of C3, which divides into lateral and medial branches. The medial branch gives rise to both super­cial and deep branches, with the supercial branch becoming the TON.The TON
Fig. 52.1 Occipital nerves
52 Occipital Neuralgia
493
curves around the dorsolateral surfaces of C2 and C3, travelling along the semispi­nalis capitis muscle. It then turns dorsally at the C2 spinous process, piercing the semispinalis capitis, splenius capitis, and trapezius muscles. After exiting these muscles, the TON innervates a small cutaneous area just below the nuchal line. Additionally, the TON sends branches to the greater occipital nerve (GON) and lesser occipital nerve (LON) [7, 8].
Both structural and non-structural causes can lead to ON.Still, almost always it results from the compression or irritation of one of the nerves at one of several ana­tomic points, the great occipital nerve (GON) being responsible for the great major­ity of the cases. Anatomical variations, such as the course of the greater occipital nerve through the obliquus inferior capitis muscle, may lead to nerve compression. Other causes of compression of the occipital nerves include hypertrophic ligaments, brosis, and bone spurs [9].
Multiple possible compression points of the GON exist that may result in ON [10].
These points include the C2 nerve root, the obliquus capitis inferior muscle, the semispinalis capitis muscle, points where the GON penetrates the trapezius muscle, and points of intersection between the GON and the occipital artery [8, 10].
Muscle hypertrophy or spasms can also contribute to nerve compression. Men, due to increased exposure to physical activities or occupations that heighten the risk of neck injuries, may be more prone to developing ON. Additionally, postural issues, particularly those related to prolonged use of computers or mobile devices, can cause tension in the neck muscles and contribute to nerve compression [11]. Trauma to the occipital region, such as whiplash injuries, may lead to ON either through direct nerve damage or secondary changes like brosis that result in nerve compression [12].
A less common cause of ON is vascular compression of the nerves by arteries, such as the posterior inferior cerebellar artery or the occipital artery [8, 13].
Degenerative diseases of the cervical spine, such as cervical spondylosis, may be more prevalent in middle-aged and older men, increasing the risk of occipital neu­ralgia. Arthritic changes, such as C1–C2 arthrosis syndrome and atlantoaxial lateral mass osteoarthritis, can cause nerve compression [12].
Other pathologies can also be associated with ON, such as cervical cord tumors, Chiari malformation, arteriovenous malformation, and neurosyphilis, which may cause secondary nerve compression or irritation [10, 14]. Systemic diseases such as diabetes can affect nerve health, increasing susceptibility to neuropathic conditions, including ON [11].
Familial cases of ON are extremely rare but have been reported, with an autoso­mal dominant or X-linked inheritance pattern. These cases often involve neuralgia of multiple cranial nerves, suggesting potential peripheral sensory anastomoses or central convergence of afferent pathways [15, 16].
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52.3 Case Presentation

A 65-year-old female with a medical history of well-controlled arterial hyperten­sion and hypothyroidism presented to the headache clinic with complaints of severe, acute, stabbing pain in the right occipital region. The pain, which started approxi­mately 10months ago, initially occurred four times a week. However, over the last ve months, the frequency of the episodes increased to daily occurrences, often with multiple attacks per day, occasionally extending into the nighttime. The patient denied any positional inuence on the pain and reported no associated symptoms, though tenderness over the affected region was present most of the time. The patient had been treated with a combination of non-steroidal anti-inammatory drugs (NSAIDs), venlafaxine, pregabalin, and carbamazepine, but none provided signi­cant relief. Occipital nerve blocks with lidocaine offered temporary pain relief.
Neurological examination was unremarkable, and systemic diseases other than hypertension and hypothyroidism were ruled out. Magnetic resonance imaging (MRI) revealed no abnormalities. The patient also underwent acupuncture and transcutaneous electrical nerve stimulation (TENS) sessions, but there was no improvement. Subsequently, she underwent rhizotomy of the right greater occipital nerve. No tissue abnormalities were observed during the procedure, and immediate pain relief was achieved, which persisted during follow-up visits.

52.4 Case Discussion

This case is characteristic of ON with a prolonged duration of symptoms. The posi­tive response to the anesthetic block further supports the diagnosis. In some cases, a Tinel’s sign may be present at the site of the greater occipital nerve emergence, but it was not noted in this case.
It is crucial to rule out systemic, cranial, and cervical causes of ON during diag­nosis. While non-invasive treatments such as acupuncture, TENS, and nerve blocks are commonly attempted, they do not always provide sufcient relief. In such cases, more invasive treatments, such as occipital nerve stimulation (ONS) or radio fre­quency ablation, may be necessary to achieve sustained pain relief.

52.5 Clinical Characteristics

The pain associated with this condition is typically paroxysmal, sharp, and intense, often originating from the posterior region of the head. It lasts from seconds to min­utes and may radiate toward the front of the head and face [10, 17]. Tenderness over the affected nerve area is commonly present, along with a Tinel’s sign at the point where the nerve emerges. The pain may be exacerbated by movement or pressure applied to the posterior region of the head.
52 Occipital Neuralgia
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52.6 Diagnosis

The diagnosis of occipital neuralgia (ON) is primarily clinical, based on the pres­ence of intermittent, typically unilateral, painful episodes of stabs in the occipital region. However, bilateral involvement can occur in some cases. The individual pain episodes can last seconds to minutes, and physical examination often reveals tender­ness over the affected occipital nerves [10, 17].
The diagnostic criteria for occipital neuralgia (ON) according to the International Classication of Headache Disorders, 3rd edition (ICHD-3), are as follows [18]:
A. Unilateral or bilateral pain in the distribution(s) of the greater, lesser and/or third
occipital nerves and fullling criteria B–D
B. Pain has at least two of the following three characteristics:
1. Recurring in paroxysmal attacks lasting from a few seconds to minutes
2. Severe in intensity
3. Shooting, stabbing or sharp in quality
C. Pain is associated with both of the following:
1. Dysesthesia and/or allodynia apparent during innocuous stimulation of the
scalp and/or hair
2. Either or both of the following:
(a) Tenderness over the affected nerve branches (b) Trigger points at the emergence of the greater occipital nerve 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.
Pain usually begins unilaterally and may extend into a bilateral distribution over time. Bilateral symptoms are present in one-third of cases. A positive Tinel’s sign may be present over the nerve’s distribution, especially where the GON emerges at the base of the skull over the greater occipital notch [7, 8].
According to ICHD-3 standards, diagnosis of occipital neuralgia requires conr­mation through a local anesthetic blockade of the suspected nerve. Pain relief fol­lowing the nerve block should last for at least the duration of the local anesthetic. Since single diagnostic blocks can yield false-positive rates of up to 40%, perform­ing a second block may help improve diagnostic accuracy [7, 8].
Occipital neuralgia (ON) can be easily confused with several other neurological conditions due to overlapping symptoms, particularly pain in the occipital region. While these conditions share similar symptoms, they have distinct etiologies and treatment approaches. Therefore, a thorough clinical evaluation, along with the use of imaging tests when necessary, is essential for accurate diagnosis. Conditions that may be mistaken for occipital neuralgia include:
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52.6.1 Cervicogenic Headache
In cervicogenic headache, the pain is referred to the occipital region and is caused by dysfunction in the cervical spine. While it shares similarities with occipital neu­ralgia (ON), cervicogenic headache is a secondary headache disorder typically linked to mechanical issues in the cervical spine. Unlike the sharp, excruciating pain of ON, cervicogenic headache pain tends to be more constant and less paroxysmal, lacking the intense, sudden episodes characteristic of occipital neuralgia [19].
52.6.2 Migraine
Migraine has distinct features such as photophobia, phonophobia, and nausea, but occasionally occipital pain may be a symptom, leading to confusion with ON. Additionally, migraine pain is usually throbbing, unlike the stabbing pain of ON [2].
52.6.3 Neck Pain
Pain in the neck, especially when associated with muscle spasms and stiffness, can radiate to the occipital region, mimicking ON.Neck pain generally does not present the paroxysmal, stabbing pain typical of ON [20].
52.6.4 Demyelinating Lesions
Multiple sclerosis and the neuromyelitis optica spectrum of disorders may present with neuralgiform pain in the occipital region due to lesions in the cervical spinal cord, which may be confused with ON.Still, it is often associated with other neuro­logical symptoms, such as sensory loss or weakness, which help differentiate it from isolated ON [5].
52.6.5 Cervical Myelitis
Inammation of the cervical spinal cord may present as occipital neuralgia, espe­cially when there are lesions at high levels of the cervical spine, such as C2, but is often associated with additional neurological decits, such as sensory or motor changes, that are not typical of ON [5, 21].
52 Occipital Neuralgia
A thorough differential diagnosis is essential and can be supported by anesthetic occipital nerve blocks, which help conrm occipital neuralgia (ON) if pain relief is achieved [2, 22]. In addition, there are less common symptoms of ON that may be present and can be useful in distinguishing this condition from others:
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52.6.6 Occipital Allodynia
Increased sensitivity to light touch in the occipital region, which may be indicative of occipital neuralgia.
52.6.7 Cervical Muscle Spasms
Although pain is the predominant symptom, some patients may experience spasms in the cervical muscles, which can contribute to pain and stiffness in the region.
52.6.8 Radiation ofPain toFrontal Areas
Pain from occipital neuralgia can radiate to areas innervated by the trigeminal nerve, leading to migraine-like symptoms such as photophobia and nausea, especially when the pain extends to the frontal region. These less common symptoms can complicate the diagnosis, necessitating careful clinical evaluation to differentiate occipital neuralgia from other neurological conditions [5, 23].

52.7 Treatment

Treatment of occipital neuralgia is multimodal and may include both conservative interventions and more invasive procedures, depending on the severity and the patient’s response to initial therapies. Non-steroidal anti-inammatory drugs, tricy­clic antidepressants, serotonin-norepinephrine reuptake inhibitors, and anticonvul­sants may help to alleviate symptoms.
52.7.1 Conservative andComplementary Treatments
Conservative treatments are often recommended as rst-line treatment and include pain education, self-care, physical therapy to reduce muscle tension and improve posture, and the use of transcutaneous electrical nerve stimulation (TENS) [24, 25].
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Complementary therapies that have demonstrated effectiveness in treating occip­ital neuralgia include acupuncture and transcutaneous electrical nerve stimulation (TENS). These alternative therapies can be integrated into a multimodal treatment plan, especially in patients who do not respond well to conventional interventions. However, it is important to consider that more research is needed to validate the long-term effectiveness and safety of these approaches.
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52.7.2 Acupuncture
Studies indicate that acupuncture can be effective in treating occipital neuralgia, showing a signicantly higher effectiveness rate compared to drug treatment alone. Acupuncture has also been associated with a signicant reduction in adverse events compared to medications. Side effects are generally mild and may include pain or bruising at the needle insertion site, dizziness, or fatigue [26, 27].
Acupuncture has been assessed in systematic reviews and meta-analyses, dem­onstrating signicantly higher efcacy compared to drug treatment alone. It is asso­ciated with a notable reduction in pain on the visual analogue scale and fewer adverse events [26].
52.7.3 Transcutaneous Electrical Nerve Stimulations (TENS)
Although evidence is limited, TENS is a conservative option that can be used to relieve muscle tension and improve posture, contributing to the management of pain associated with ON [24, 25].
Immobilization of the neck by the cervical collar, physiotherapy, and cryother­apy have not been shown to perform better than placebo [28].

52.8 Minimally Invasive Treatment

52.8.1 Nerve Blocks
Injections of local anesthetics, such as lidocaine or bupivacaine, with or without corticosteroids, are often used for short-term pain relief and may have diagnostic value [11, 12, 17].
Ultrasound guidance was introduced to increase the effectiveness of the proce­dure. Greher etal. (2010) described the original ultrasound-guided technique for injection of the GON.It targets the nerve as it courses supercial to the obliquus capitis inferior muscle at the C1–C2 level [29].
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52.8.2 Botulinum Toxin A
Botulinum toxin A injection has emerged as a treatment with a relatively lower side effect prole compared to many other approaches. Recent trials have shown that it can result in a 50% or greater improvement in symptoms [10, 28].
52.8.3 Radio Frequency
Pulsed radio frequency (PRF) ablation of the occipital nerves is effective in provid­ing long-term pain relief in refractory patients. Studies indicate that PRF may pro­vide greater pain relief compared to steroid injections [24, 30].
52.8.4 Occipital Nerve Stimulation
Occipital nerve stimulation (ONS) is a valuable option for treating refractory cases of occipital neuralgia and can provide signicant pain relief in some patients. This minimally invasive procedure has shown promising results, though further studies are needed to conrm its long-term effectiveness [24, 31].
Salmasi etal. (2020) followed three patients for an average of eight months, with an average pain reduction of approximately 50% [31].
Occipital nerve stimulation (ONS) is both safe and effective, with response rates ranging from 63 to 100% in patients with occipital neuralgia. Furthermore, a high percentage of patients continue to respond positively to ONS over the long term [32].

52.9 Surgical Treatments

In very refractory cases, surgical procedures such as occipital nerve decompression or neurectomy may be considered, but there are associated risks [11, 33, 34].
Occipital neuralgia can be difcult to manage, and treatment options should be carefully considered based on the patient’s unique clinical presentation and their response to previous interventions. Treatment decisions must be personalized, weighing factors such as efcacy, potential risks, and the patient’s preferences. A systematic review investigated 12 articles on the surgical management of occipital neuralgia and did not nd sufcient evidence to recommend neurolysis versus neu­rotomy [35].
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52.10 Conclusions

Occipital neuralgia is an uncommon but signicant cause of intense, paroxysmal, sharp headaches that typically last from seconds to minutes. These headaches are localized in the areas innervated by the greater occipital nerve (GON), lesser occipi­tal nerve (LON), or third occipital nerve (TON). The primary etiologies include anatomical, traumatic, iatrogenic, and vascular causes. Diagnosis is based on a thor­ough patient history, clinical examination, and a positive response to an anesthetic block of the occipital nerves. Other conditions may mimic occipital neuralgia and can also respond to nerve blocks, so these must be ruled out through careful clinical assessment and, if necessary, imaging studies. Conservative treatments, such as pharmacological therapy combined with physical therapy, are often effective. Minimally invasive interventions, such as occipital nerve blocks, can be both diag­nostic and therapeutic. In cases that are refractory to these treatments, additional options like radio frequency (RF) ablation or neuromodulation may be required for long-term relief. Surgical decompression, neurotomy, and neurolysis are considered last-resort options for particularly challenging cases.

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