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Occipital neuralgia (ON) is a painful condition affecting the posterior head in the distributions of the
greater occipital nerve The greater occipital nerve is a nerve of the head. It is a spinal nerve, specifically the medial branch of the dorsal primary ramus of cervical spinal nerve 2. It arises from between the first and second cervical vertebrae, ascends, and then p ...
(GON),
lesser occipital nerve The lesser occipital nerve or small occipital nerve is a cutaneous spinal nerve. It arises from second cervical (spinal) nerve (along with the greater occipital nerve). It innervates the scalp in the lateral area of the head posterior to the ...
(LON), third occipital nerve (TON), or a combination of the three. It is
paroxysmal Paroxysmal attacks or paroxysms (from Greek παροξυσμός) are a sudden recurrence or intensification of symptoms, such as a spasm or seizure. These short, frequent symptoms can be observed in various clinical conditions. They are usuall ...
, lasting from seconds to minutes, and often consists of lancinating pain that directly results from the pathology of one of these nerves. It is paramount that physicians understand the differential diagnosis for this condition and specific diagnostic criteria. There are multiple treatment modalities, several of which have well-established efficacy in treating this condition. Text was copied from this source, which is available under
Creative Commons Attribution 4.0 International License


Signs and symptoms

Patients presenting with a headache originating at the posterior skull base should be evaluated for ON. This condition typically presents as a paroxysmal, lancinating or stabbing pain lasting from seconds to minutes, and therefore a continuous, aching pain likely indicates a different diagnosis. Bilateral symptoms are present in one-third of cases.


Causes

Occipital neuralgia is caused by damage to the occipital nerves, which can arise from
trauma Trauma most often refers to: * Major trauma, in physical medicine, severe physical injury caused by an external source * Psychological trauma, a type of damage to the psyche that occurs as a result of a severely distressing event *Traumatic i ...
(usually
concussive A concussion, also known as a mild traumatic brain injury (mTBI), is a head injury that temporarily affects brain functioning. Symptoms may include loss of consciousness (LOC); memory loss; headaches; difficulty with thinking, concentration, ...
or
cervical In anatomy, cervical is an adjective that has two meanings: # of or pertaining to any neck. # of or pertaining to the female cervix: i.e., the ''neck'' of the uterus. *Commonly used medical phrases involving the neck are **cervical collar **cerv ...
), physical stress on the nerve, repetitive neck contraction, flexion or extension, and/or as a result of medical complications (such as
osteochondroma Osteochondromas are the most common benign tumors of the bones. The tumors take the form of cartilage-capped bony projections or outgrowth on the surface of bones exostoses. It is characterized as a type of overgrowth that can occur in any bone w ...
, a benign bone tumour). A rare cause is a
cerebrospinal fluid leak A cerebrospinal fluid leak (CSF leak or CSFL) is a medical condition where the cerebrospinal fluid (CSF) surrounding the brain or spinal cord leaks out of one or more holes or tears in the dura mater. A cerebrospinal fluid leak can be either cr ...
. Rarely, occipital neuralgia may be a symptom of
metastasis Metastasis is a pathogenic agent's spread from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, then ...
of certain
cancer Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal b ...
s to the spine. Among other cranial neuropathies, occipital neuralgia is also known to occur in patients with multiple sclerosis.


Differential diagnosis

The conditions most easily mistaken with ON for other headache and facial pain disorders include migraine, cluster headache, tension headache, and hemicrania continua. Mechanical neck pain from an upper disc, facet, or musculoligamentous sources may refer to the occiput, but is not classically lancinating or otherwise neuropathic and should not be confused with ON. A crucial step in differentiating ON from other disorders is relief with an occipital nerve block.


Epidemiology

In one study investigating the incidence of facial pain in a Dutch population, ON comprised 8.3% of facial pain cases. The total incidence of ON was 3.2 per 100,000 people, with a mean age of diagnosis of 54.1 years.


Treatment

There are multiple treatment options for ON. The most conservative treatments, such as immobilization of the neck by the cervical collar, physiotherapy, and cryotherapy have not been shown to perform better than placebo. Non-steroidal anti-inflammatory drugs, tricyclic antidepressants, serotonin-norepinephrine reuptake inhibitors, and anticonvulsants may help to alleviate symptoms. Following diagnostic nerve blocks, therapeutic blocks may be attempted. Typically, a steroid is added to the local anesthetic with variable results. Botulinum Toxin A injection has emerged as a treatment with a conceptually lower side effect profile than many other techniques described here, with most recent trials demonstrating 50% or more improvement. It remains a common practice to utilize a landmark-only approach when performing greater and lesser occipital nerve blocks. For blockade of both nerves, medication is infiltrated along the nuchal ridge. This technique, while easy to perform and relatively safe if done correctly, may not be particularly accurate and as a result, could theoretically increase the risk of a false-positive result. To improve accuracy, ultrasound-guided techniques were developed. The original ultrasound-guided technique for injection of the GON was described by Greher and colleagues in 2010; it targets the nerve as it courses superficial to the obliquus capitis inferior muscle at the C1-C2 level. There are several advanced interventional procedures in clinical use: * Pulsed or thermal radiofrequency ablation (RFA) may be considered for longer-lasting relief after a local anesthetic blockade confirms the diagnosis. Thermal RFA aimed at destroying the nerve architecture can render long-term analgesia but also comes with the potential risks of hypesthesia, dysesthesia, anesthesia dolorosa, and painful neuroma formation. Chemical neurolysis with alcohol or phenol carries the same risks as thermal RFA. There is no such risk with pulsed RF, however, some question its efficacy as compared to other procedures. * Neuromodulation of the occipital nerve(s) involves the placement of nerve stimulator leads in a horizontal or oblique orientation at the base of the skull across where the greater occipital nerve emerges. Patients should be trialed with temporary leads first, and greater than 50% pain relief for several days is considered a successful trial after which permanent implantation may be considered. Risks include surgical site infection and lead or generator displacement or fracture after the operation. * Ultrasound-guided percutaneous cryoablation of the GON is sometimes performed. At the correct temperature, there should be stunning but not permanent damage of the nerve, but at temperatures below negative 70 degrees Celsius, nerve injury is possible. Most recently in the literature, a 2018 article by Kastler and colleagues described 7 patients who underwent cryoneurolysis in a non-blinded fashion to good effect, but the follow-up was limited to 3 months. * Surgical decompression is often considered to be the last resort. In one study of 11 patients, only two patients did not experience significant pain relief postoperatively and mean pain episodes per month decreased from 17.1 to 4.1, with mean pain intensity scores also decreasing from 7.18 to 1.73. Resection of part of the obliquus capitis inferior muscle has shown success in patients who have an exacerbation of their pain with flexion of the cervical spine. Another popular surgical technique is C2 gangliotomy, even though patients are left with several days of intermittent nausea and dizziness. As with any large nerve resection, there is a theoretical risk of developing a deafferentation syndrome, though arguably the risk is lower if the resection is pre-ganglionic.


References


External links


Occipital Neuralgia - National Institute of Neurological Disorders and Stroke
{{headache Neurological disorders