Occipital neuralgia (ON) is a painful condition affecting the posterior head in the distributions of the greater occipital nerve (GON), lesser occipital nerve (LON), third occipital nerve (TON), or a combination of the three. It is paroxysmal, 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. 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 (usually concussive or cervical), physical stress on the nerve, repetitive neck contraction, flexion or extension, and/or as a result of medical complications (such as osteochondroma, a benign bone tumour). A rare cause is a cerebrospinal fluid leak. Rarely, occipital neuralgia may be a symptom of metastasis of certain cancers 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.
Hereditary progressive mucinous histiocytosis is a very rare, benign, non-Langerhans cell histiocytosis. An autosomal dominant or X-linked hereditary disease described on the skin, it has been found almost exclusively in women. One case of the disease in a male patient has been reported. See also
Non-X histiocytosis

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