What is Glossopharyngeal Neuralgia?
Glossopharyngeal neuralgia (GN) is a rare pain condition that affects the nerve known as the glossopharyngeal, which is the ninth cranial nerve. To put it simply, it’s like a major headache, affecting areas that the ninth cranial nerve reaches, which includes parts like the angle of your jaw, your ear, back of your tongue and your throat. Users regularly describe the pain as sharp, sudden and severe but it starts and stops suddenly. The pain can often be triggered by things like coughing, talking or swallowing and it doesn’t stay constant but comes and goes.
This condition is listed under the ICD-10-CM-G52.1 category in the International Classification of Diseases due to its unique nature. This medical term was first coined in 1921 by a doctor named W. Harris. However, the first known symptoms were recognized by T. Weisenburg in 1910 in a 35-year-old man who had a tumor compressing the ninth cranial nerve.
Interestingly, in 1942, a group of doctors noted that in certain cases, the severe pain from glossopharyngeal neuralgia was associated with fainting spells, unusually slow heartbeat, no heartbeat, or even seizures. If these symptoms are present along with glossopharyngeal neuralgia, the condition is then called vagoglossopharyngeal neuralgia. Sometimes, glossopharyngeal neuralgia may be misdiagnosed as trigeminal neuralgia, another nerve condition, because the nature of the pain in both conditions is pretty similar.
What Causes Glossopharyngeal Neuralgia?
Essential or idiopathic glossopharyngeal neuralgia is when we don’t know why the condition occurs. There are other reasons, or secondary causes, for glossopharyngeal neuralgia:
* Vascular compression, mainly at the nerve root: this is the most common cause
* Diseases that cause the protective covering of nerve cells to deteriorate, like multiple sclerosis
* Inflammatory and autoimmune diseases, like Sjogren disease, which often affects mouth and eye moisture levels
* Infections in the mouth and near the tonsils
* Brain lesions like tumors, particularly those in the medulla or those coming from what doctors call the CP angle
* Abnormal formations in the lower part of the brain and upper part of the spine
* Eagle syndrome or stylalgia: This happens if the styloid process, a small bone just below your ear, is longer than usual, or if the stylohyoid ligament, which connects the styloid process to the throat, hardens. These changes can put pressure on the glossopharyngeal nerve.
* Mouth and throat cancers, such as carcinoma of the tongue and non-cancerous tumors like schwannomas, which grow in the tissue that covers nerves.
Risk Factors and Frequency for Glossopharyngeal Neuralgia
Glossopharyngeal neuralgia is an extremely rare condition that affects the nerves in the head. It is responsible for between 0.2 and 1.3% of all nerve pain in the head. A study done in Rochester, Minnesota, from 1945 to 1984 found that only 0.7 out of every 100,000 people were diagnosed with this condition. It’s also interesting to note that it affects both men and women equally.
In 1981, a study was conducted on 217 patients with glossopharyngeal neuralgia who had been seen at the Mayo Clinic between 1922 and 1977. Here’s what they found:
- About 57% of the patients were over 50 years old.
- About 43% were between the ages of 18 and 50.
- Interestingly, about 74% of the patients saw their symptoms disappear on their own.
- About 17% didn’t experience any relief.
- Only 12% of the patients complained of pain on both sides of their face.
Signs and Symptoms of Glossopharyngeal Neuralgia
Glossopharyngeal neuralgia, a nerve disorder causing sharp pain in the throat and back of the tongue, is diagnosed based on specific criteria from the International Classification of Headache Disorders, Third Edition (ICHD-3). These criteria include repeated sudden painful attacks on one side of the throat, lasting a few seconds to 2 minutes, with severe, sharp, stabbing or electrical shock-like sensations. These pain episodes are generally worsened by coughing, yawning, swallowing or talking. Importantly, the pain should not be explained by any other disorder.
Anyone between the ages of 21 to 75 can develop glossopharyngeal neuralgia, with no gender preference. The pain, which can occur anywhere below the ear, in the epiglottis, tonsils, base of the tongue, and/or the back of the throat, is often worsened by swallowing food or drink, regardless of temperature. The start and end of the pain is typically sudden. While the first few attacks can be extremely painful, the intensity may reduce over time. Patients might not feel any pain between attacks, but may continue to experience mild pain.
People with this disorder have a remarkably consistent pain pattern. Pain episodes can occur minutes apart and then not recur for several days. They experience pain mostly during the day, but it can also disturb their sleep. Chewing and swallowing can be so painful that it can lead to weight loss. Attacks can fade away on their own, but a residual uncomfortable feeling may last several weeks to months.
A physical examination often reveals no abnormalities or changes to light touch and pinprick sensitivity in the areas where the patient experiences pain. However, some patients may feel abnormal sensations or extreme sensitivity in these areas. If a patient lacks the cough or gag reflex, it may be necessary to conduct a thorough investigation into the causes of the pain. In rare instances, this disorder can occur alongside trigeminal neuralgia, another nerve disorder causing facial pain.
Testing for Glossopharyngeal Neuralgia
Diagnosing glossopharyngeal neuralgia, a condition that causes intense pain in the throat and back of the tongue, depends mostly on the symptoms you report and a physical examination. Doctors will perform a thorough examination of your throat and neck. Additionally, they’ll also usually ask for some laboratory tests. These include a complete blood count, basic metabolic panel (which measures different chemicals in your body), erythrocyte sedimentation rate (that detects inflammation), and tests for anti-nuclear antibodies (to rule out any autoimmune diseases). This is done to ensure there’s no underlying infection, inflammation, cancer, or temporal arteritis (a condition that affects blood vessels).
If you’re having frequent severe symptoms or complications like fainting, the doctor might ask for a heart evaluation including an echocardiogram (an ultrasound of the heart) and Holter monitoring (wearable device that records your heart’s activity).
Sometimes, the intense pain can be due to something pressing on the nerve at the base of the skull. For these situations, doctors use imaging tests.
A Computed Tomography (CT) scan might not directly show the nerve, but it’ll help spot any unusual growth or structure like a long and hardened styloid process (a small bone just below the ear).
Similarly, an X-ray of your neck (from the side view) can reveal if the styloid process is excessively long and heavily calcified (hardened due to the build-up of calcium).
Magnetic Resonance Imaging (MRI) is another imaging method that’s particularly helpful in viewing the glossopharyngeal nerve. An MRI with contrast dye can help highlight the nerve, blood vessels, and nearby structures. The major cause of nerve compression is usually the posterior inferior cerebellar artery (PICA), followed by the vertebral artery and anterior inferior cerebellar artery. The MRI can also reveal any unusual spots in the brain, tumors, or malformations.
Lastly, a Magnetic resonance angiogram (MRA) may be conducted to check if any loops of blood vessels might be pressing on the nerve.
Treatment Options for Glossopharyngeal Neuralgia
Glossopharyngeal neuralgia, a condition that causes intense throat and ear pain, can often be managed effectively with medication. Medications such as carbamazepine or oxcarbazepine are the most common treatment options.
There are also other types of medications that can be used to help manage the nerve pain caused by this condition, such as:
– Gabapentin
– Duloxetine
– Valproic acid
– Clonazepam
– Lamotrigine
– Baclofen
– Phenytoin
– Pregabalin
– Topiramate
These medicines are started at low doses and depending on their effectiveness and the patient’s tolerance, the dose may slowly increase. This condition tends to have periods of waxing and waning symptoms, which means there’ll be times when the pain comes and goes. So, the medication can be reduced to a maintenance dose when the pain is less severe. Sometimes, using a combination of two or more different types of medications can help achieve better pain relief with fewer side effects. In severe cases, opioids can be used for a short period.
Aside from medication, additional methods can help manage the pain such as cold and hot compresses, physical therapy, and psychological counseling. The effectiveness varies but they can bring about some relief.
Interventional pain management techniques might be considered as well. This can involve blocking the glossopharyngeal nerve, which can help both in diagnosing the cause of the pain and treating it. The first step is a test block with local anesthesia. If this test is successful in reducing the pain, a more permanent block can be performed. Various methods are used for these more permanent blocks which include using chemicals like alcohol or glycerol to deactivate the nerve. Radiofrequency ablation, where heat is used to deactivate the nerve, could also be an option. There can, however, be complications with these procedures such as changes in voice or irregular heart rate.
In certain situations, if the pain persists despite these treatments, surgery may be an option. There are various surgical procedures that can be used based on the cause of the pain. If a blood vessel is pressing on the nerve, a procedure called microvascular decompression could be used. Or parts of the nerve could be removed to alleviate the pain. Other less invasive surgical techniques are also available, but the data about their safety and effectiveness is limited.
What else can Glossopharyngeal Neuralgia be?
When doctors are trying to diagnose glossopharyngeal neuralgia, they can often misdiagnose it as something else because the symptoms can be similar. Here are some of the conditions that can be confused with glossopharyngeal neuralgia:
- Trigeminal neuralgia: This condition and glossopharyngeal neuralgia have similar pain symptoms. Both are related to the cranial nerves and have similar underlying causes and treatment plans. Sometimes, both conditions can occur at the same time. However, glossopharyngeal neuralgia is much less common, so it is often overlooked. The location of the pain and what triggers the pain is different for each condition. Glossopharyngeal neuralgia causes pain in the throat and tonsil area, and activities like swallowing and chewing can make it worse. Trigeminal neuralgia causes facial pain in the areas where the trigeminal nerve is located, and activities like touching the face, washing the face, or brushing the teeth can make it worse.
- Jacobson’s neuralgia: If the only symptom of glossopharyngeal neuralgia is a loss of feeling in the ear, it can sometimes be confused with intermedius or Jacobson neuralgia.
- Temporal arteritis and temporomandibular joint dysfunction: These conditions can cause pain in the same area as glossopharyngeal neuralgia, but they are entirely different. The pain characteristics are also different.
Because of these common sources of confusion, doctors need to take a detailed medical history and conduct a thorough physical examination to make the right diagnosis.
What to expect with Glossopharyngeal Neuralgia
Glossopharyngeal neuralgia, a condition characterized by severe throat and ear pain, has a prognosis (or likely outcome) that can differ greatly based on the individual’s symptoms. Many people only have a single episode of sudden, severe pain, known as a paroxysmal attack. The chance of this condition recurring in the following year is as low as 3.6%. Furthermore, only one in four patients may need surgery, while the others can usually manage their symptoms with medication. Fewer than one in four patients will have pain on both sides.
However, certain symptoms may indicate a poorer prognosis, such as pain on both sides, numerous episodes of severe, unbearable pain, and constant pain.
Possible Complications When Diagnosed with Glossopharyngeal Neuralgia
When the glossopharyngeal nerve (a nerve in your throat) is irritated, it sends signals to different parts of your brain. These signals can cause a strong response from the vagus nerve, another important nerve that helps regulate heart rate and blood pressure. This response can result in uncontrollable slowing of the heart rate (bradycardia), lower blood pressure (hypotension), and abnormal heart rhythms (cardiac dysrhythmias). This can lead to less blood flow to the brain (cerebral hypoperfusion), seizures, fainting (syncope) and certain movements such as convulsions, limb shaking, smacking your lips together, and eyes rolling upwards, all of which are indications of a lack of oxygen to your brain (cerebral hypoxia).
Heart-related issues often occur during the pain episodes or right after the pain stops. Treating the pain caused by glossopharyngeal neuralgia with medications and/or surgeries can help manage these conditions. Some people may just have the heart-related symptoms without the painful episodes. This variant is referred to as non-neurologic glossopharyngeal neuralgia. These individuals could be treated with a procedure that involves relieving pressure on the glossopharyngeal nerve or completely removing it.
Possible Symptoms:
- Uncontrollable slowing of the heart rate (Bradycardia)
- Lower blood pressure (Hypotension)
- Abnormal heart rhythms (Cardiac dysrhythmias)
- Less blood flow to the brain (Cerebral hypoperfusion)
- Seizures
- Fainting (Syncope)
- Convulsions
- Limb shaking
- Smacking lips together
- Eyes rolling upwards
Preventing Glossopharyngeal Neuralgia
People suffering from glossopharyngeal neuralgia, a condition characterized by severe throat and ear pain, should have a good understanding of their situation and realistic expectations about their treatment plan. They should continuously communicate with their healthcare providers about whether their treatments are working or not so that any necessary adjustments can be made.
