If the patient experiences a sharp pain in the back of the neck, close to the nuchal line, and it radiates to the parietal and frontal areas, the clinician should consider the possibility of occipital neuralgia.
Occipital neuralgia (ON) is a primary headache disorder defined by the International Headache Society as “sharp, shooting, or electric shock-like pain in the distribution of the greater, lesser, or third occipital nerves”. The pain usually presents in the upper neck, back of the head, and behind the ears and can radiate to the front of the head. In addition, the occipital region of the scalp would be tender to palpation, and will have a positive Tinel’s sign, meaning that tapping in the nuchal line will elicit the pain.
Although it is currently classified as a headache disorder, many would argue that it should be considered a separate neurological condition. Due to its mixed presentation between headaches and neuralgia, it can be a diagnostic challenge and the patient might not be diagnosed and treated in a timely manner. Therefore, clinical history taking is key to establishing the diagnosis and an appropriate treatment plan. Imaging could be useful as well to confirm the diagnosis and guide clinical decision-making. Ultrasound helps to determine any swelling or entrapment of the nerves involved. Computed tomography is sometimes used if a bony compression is suspected.
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The causes of ON are not fully understood; however, several etiologies have been identified including trauma, atlas-related compression (e.g., fracture, atlantoaxial lateral mass osteoarthritis), C2 nerve root compression or arthrosis, spinal cord tumor, and neurosyphilis.
Many management modalities have been used for ON ranging from conservative approaches to minimally-invasive, and even surgical interventions. The treatment of choice is dependent on the severity of the condition as well as the patient’s response to previous methods. Non-pharmacological strategies include physical or thermal therapy, postural modification, and acupuncture. These could be combined with pharmacotherapy with anticonvulsants, selective serotonin reuptake inhibitors, and tricyclic antidepressants being the most commonly used.
If the first line of treatment was deemed ineffective, greater and lesser occipital nerve blocks using 1-2% lidocaine or 0.25-0.5% bupivacaine should be considered. Nerve blocks represent the minimally-invasive approach, yet a short duration of pain relief has been reported. For longer periods of pain management, radiofrequency ablation and occipital nerve stimulation have been employed. The combination of lidocaine and corticosteroids resulted in more persistent pain relief. Additionally, botulinum toxin (Botox) injections showed effectiveness in improving the sharp but not the dull pain associated with ON.  However, the evidence available for the long-term success of Botox for ON management is not strong enough and further well-designed clinical trials are needed.
Surgical or endoscopic-assisted occipital nerve decompression through resection of the obliquus capitis inferior is performed for pharmacologically resistant patients. If decompression is unsuccessful, other surgical options are available such as the excision of the greater occipital nerve, intradural cervical dorsal rhizotomy, or even cervical ganglionectomy. The surgical approaches are usually reserved as a final resort for persistent pain as they do not come without risks.
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