Glossopharyngeal neuralgia (GPN) is rare neurological condition characterized by sudden, repeated episodes of severe pain in the throat, tongue, ear, and tonsils. These are areas innervated by the glossopharyngeal and vagus nerves. The episodes can last from a few seconds to a few minutes. Episodes of pain may be triggered by swallowing, chewing, talking, coughing or yawning.
- What causes glossopharyngeal neuralgia?
- How common is glossopharyngeal neuralgia?
- How is glossopharyngeal neuralgia treated?
- Who is a candidate for glossopharyngeal neuralgia surgery?
What causes glossopharyngeal neuralgia?
Most cases of GPN are idiopathic, of unknown cause with no abnormalities detected by imaging. GPN can be caused when the glossopharyngeal nerve is compressed by an artery or a vein, tumors, or other lesions. Once diagnosed, there are both medicines and surgical procedures to reduce or relieve the debilitating pain caused by this disease. Patients with GPN are given high priority in scheduling their evaluation.
How common is glossopharyngeal neuralgia?
GPN is a very rare condition, occurring in an estimated two to seven people per million. GPN often affects patients in their 50s, but can affect people of any age.
How is glossopharyngeal neuralgia treated?
Traditionally, medicines for GPN includes anti-epileptic medications like carbamazepine, gabapentin, phenytoin, oxcarbazepine, or pregabalin. However, for many patients, medical therapy may not be fully effective. Over time, microvascular decompression (MVD), a surgical procedure, has become an effective treatment for persistent GPN. A majority of patients that undergo MVD achieve long-term pain relief.
Microvascular decompression (MVD) is the most common surgical procedure for the treatment of glossopharyngeal neuralgia. This is a procedure where a small incision is made behind the ear, a small hole is drilled in the skull, and, under microscopic visualization, the glossopharyngeal nerve is exposed. In some cases, there is a blood vessel (typically an artery, but sometimes a vein) compressing the glossopharyngeal nerve. By moving this blood vessel away from the nerve and interposing a padding made of Teflon felt, the pain can be relieved. However, if there is no obvious nerve compression by blood vessels, the surgical procedure may involve transection of the glossopharyngeal nerve and sensory branches of the vagus nerve, which also offers pain relief.
MVD has a long-term success rate of approximately 80%. The procedure requires an average hospital stay of two to three days.
Who is a candidate for glossopharyngeal neuralgia surgery?
Surgical evaluation for glossopharyngeal neuralgia includes confirming the diagnosis of glossopharyngeal neuralgia, reviewing a brain magnetic resonance imaging (MRI) scan to exclude other treatable causes of facial pain, and evaluating the severity of the pain, the general medical condition of the patient, and the patient's preference for treatment goals versus risk aversion.
Glossopharyngeal neuralgia surgery is reserved for people who still experience debilitating pain despite medical treatment.
At UCSF, patients can be evaluated by experienced neurologists and neurosurgeons who specialize in the evaluation and treatment of glossopharyngeal neuralgia:
- To schedule an evaluation to confirm a diagnosis of glossopharyngeal neuralgia and discuss treatment options, contact the Neurology Clinic at (415) 353-2273.
- For patients that have a confirmed diagnosis of glossopharyngeal neuralgia, but still experience debilitating pain despite medications, contact the Neurosurgery Clinic for referrals to discuss possible surgical treatment. Contact patient navigator Erica Terry at (415) 353-2241, and fax referral and medical records to (415) 353-2889.
What are the potential side effects of MVD?
Surgical risks may include swallowing difficulties, infection, bleeding, spinal fluid leakage, and risks of anesthesia. Rare neurological injury can include damage to hearing, vascular injury (stroke), and, very rarely, death.
Will I have pain when I wake up?
Patients will have some incisional pain and headache postoperatively, but the nurses will give you medication to help you control this pain.
How long will I need to stay at the hospital?
Patients usually spend two nights in the hospital.
When may I resume normal activities?
Soreness at the incision site and headaches are common following surgery and typically subside within the first 1-2 weeks following your surgery. You will need to avoid heavy lifting or straining in the first six weeks following your surgery. Walking is encouraged as much as possible. When you are tired or have a headache, stop and rest.
You will increase your activity gradually so that you are back to your normal routine 4-6 weeks after surgery.
Will surgery be completely curative?
No one can promise that any surgery for glossopharyngeal neuralgia will be successful for all patients, and there is always the chance that pain will recur at a later date; however, surgery is the best chance at relieving the underlying problem behind glossopharyngeal neuralgia pain.