Trigeminal Neuralgia

What is Trigeminal Neuralgia?

Trigeminal neuralgia, also known as tic douloureux, is a long-term pain condition that happens in quick, sharp bursts that feel like electric shocks. It targets the areas connected to the fifth cranial nerve, also known as the trigeminal nerve, which supplies feeling to the forehead, cheek, and lower jaw. These painful episodes most often affect one side of the face, but can involve one or more sections of the trigeminal nerve.

What Causes Trigeminal Neuralgia?

The trigeminal nerve is the biggest nerve located in our head. It plays a vital role in how we feel sensations on our face and how we chew, or move our jaw. This nerve processes things we feel like touch, pain, and temperature from one side of our face. This information is then sent over to the opposite side of our brain, specifically to a part called the thalamus.

This nerve is divided into three parts: the ophthalmic (V1), maxillary (V2), and mandibular (v3). These divisions control sensations in these specific areas:

– Ophthalmic (V1): the eye, upper eyelid, and forehead
– Maxillary (V2): the lower eyelid, cheek, nostril, upper lip, and upper gums
– Mandibular (V3): the lower lip, lower gums, jaw, and chewing muscles.

A condition known as trigeminal neuralgia often occurs because of pressure on the trigeminal nerve near the part where it enters the brain stem. In many of these cases, about 80-90%, the pressure comes from an artery or a vein that’s too close to the nerve. Most commonly, this is due to the superior cerebellar artery that is too close in 75-80% of situations.

Sometimes, other blood vessels are the cause of the pressure, like the petrosal vein, or arteries like the anterior inferior cerebellar or vertebral arteries. The pressure could also come from tumors or other masses such as meningioma, acoustic neuroma, a type of cyst known as an epidermoid cyst, arteriovenous malformation, or saccular aneurysm.

Certain conditions like multiple sclerosis, which damages the covering of nerve cells, can increase the risk of developing trigeminal neuralgia. Multiple sclerosis is the root cause in about 2% to 4% of patients who show symptoms because it damages the region of the trigeminal nerve.

Risk Factors and Frequency for Trigeminal Neuralgia

Trigeminal neuralgia, a condition that affects the facial nerve, shows up in 4 to 13 out of every 100,000 people each year. It’s more common in women than men, with the ratio ranging from 1.5 to 1.7 women for every man affected. While most cases occur after the age of 50, it’s also seen in people in their 20s and 30s and rarely in children. The chance of getting this condition in your lifetime is estimated to be 0.16% to 0.3%.

Trigeminal neuralgia affects 4 to 13 per 100,000 people each year.
Women are more usually affected compared to men.
Most cases happen after 50 years of age, but it can also occur in people in their 20s and 30s. Cases in children are quite rare.
The chance of getting this condition in your lifetime is somewhere around 0.16% to 0.3%.
If a young person develops trigeminal neuralgia, this could indicate the presence of multiple sclerosis. Between 1% and 6.3% of people with multiple sclerosis also have trigeminal neuralgia.
People with high blood pressure are slightly more likely to get trigeminal neuralgia than those with normal blood pressure.

Signs and Symptoms of Trigeminal Neuralgia

Trigeminal neuralgia, a painful condition affecting the facial nerves, can be categorized into two types. Some patients can experience both types. The key difference lies in the nature of the pain. Type 1 pain is episodic, almost like sudden attacks, while Type 2 pain is constant, and may or may not have sudden episodes. These attacks of pain are described as electric or shock-like, lasting from a moment to several seconds.

Type 1: Mainly episodic, sudden pain
Type 2: Constant pain, with potential sudden bouts

This pain is usually one-sided and is often more common on the right side than the left. While uncommon, bilateral pain can occur, though it seldom happens simultaneously on both sides. Sleep, interestingly, is generally uninterrupted by these episodes of pain.

The pain is usually associated with the V2 and V3 divisions of the trigeminal nerve. That may trigger mild symptoms like eye watering, runny nose, and redness in the whites of the eyes when the V1 division is involved, though this is rare. Some patients have “trigger zones” – specific areas where a light touch can set off the pain. These zones are typically near the midline of the face, especially around the nose and mouth. Known triggers also include routine tasks such as brushing teeth, shaving, washing the face, chewing, talking, smoking, and exposure to cold air. In younger patients showing signs of trigeminal neuralgia, medical professionals may also take into account symptoms of multiple sclerosis.

During medical examinations, patients with trigeminal neuralgia typically don’t show any specific neurological deficits. Comprehensive physical exams are necessary to rule out other potential causes of facial pain. The presence of sensory loss, loss of the corneal reflex, or weakness in the facial muscles may indicate secondary trigeminal neuralgia, requiring specific attention. An in-depth oral exam is also needed to differentiate from dental causes of pain. The presence of trigger zones strongly suggest trigeminal neuralgia.

Testing for Trigeminal Neuralgia

Trigeminal neuralgia is a condition suspected through a patient’s symptoms and a physical exam. If you have been diagnosed with this condition based on these factors, your doctor will likely recommend neuroimaging, a type of medical scan that creates images of the brain and its nerves. This usually involves techniques such as magnetic resonance imaging (MRI) or magnetic resonance angiography (MRA). Between the two, MRI is usually the preferred choice.

The International Classification of Headache Disorders (ICHD-3) has established some criteria to diagnose trigeminal neuralgia. These include:

1. Repeated episodes of facial pain on one side of your face, which follows the path of the trigeminal nerve. There are two further conditions that need to be met.
2. The pain has the following features:
* It doesn’t last long, usually a fraction of a second to about 2 minutes.
* The severity of the pain is quite high.
* The pain feels like an electric shock or is sharp and shooting.
3. The pain is triggered by harmless stimuli, such as lightly touching the affected area of the face.
4. There are no better explanations or alternative diagnoses for the symptoms.

Neuroimaging studies, like MRI or MRA, help to point out the cause of secondary trigeminal neuralgia. They can also highlight possible vascular compression, a case where a blood vessel may be putting pressure on the nerve, in primary cases.

If your symptoms align with this condition, your doctor might use a specialized form of MRI known as FIESTA sequencing, with or without a substance called gadolinium contrast, to get detailed images of the blood vessels and the brain. This technique allows the doctor to clearly see the course of the trigeminal nerve and identify any pressure on it caused by blood vessels. FIESTA sequencing is particularly beneficial for patients under 40 years of age, those experiencing symptoms on both sides of the face, and those who show signs of sensory loss during the physical examination. In such cases, the risk of having secondary trigeminal neuralgia, which is caused by another underlying disease, is higher.

Treatment Options for Trigeminal Neuralgia

If you have trigeminal neuralgia, a condition that causes severe facial pain, your doctor will probably first recommend medical therapy, which means you’ll start with medication as the initial treatment. The main drugs used are called antiepileptics such as carbamazepine or oxcarbazepine. They actually control pain by obstructing some of the channels that allow nerve cells to send signals. Unfortunately, these medications don’t offer instant relief. They usually take a couple of weeks to start working.

The side effects of carbamazepine include feeling sleepy or dizzy, double vision, and feeling sick. Before patients of Asian ancestry start taking carbamazepine, it’s important to have a specific genetic test. A particular gene variant, known as the HLA-B*15:02 allele, can increase the risk of harmful skin reactions when taking carbamazepine. Oxcarbazepine is a newer drug and is often used if carbamazepine isn’t effective or causes severe side effects. It might also be a better choice if you’re taking other medications as it doesn’t interact with as many other drugs. Both medications can lead to low sodium levels in the body, a condition also known as hyponatremia.

If the first line therapy isn’t effective, your doctor may suggest other antiepileptic drugs, such as gabapentin, clonazepam, lamotrigine, phenytoin, and valproic acid. Gabapentin, in particular, has been shown to reduce facial pain by almost half in patients who didn’t find relief with surgery. However, the side effects can include feelings of dizziness, confusion, and sleepiness.

There is also another drug called baclofen, a muscle relaxant, which can help decrease the intensity and severity of pain attacks. Baclofen’s side effects may include dizziness, sleepiness, and upset stomach. A few newer drugs are being investigated but more research is needed to determine their effectiveness.

If medical therapy doesn’t bring enough relief, surgical intervention may be considered. Depending on the specific circumstances, different surgical procedures such as microvascular decompression, rhizotomy, and peripheral nerve block can be recommended. Microvascular decompression, a procedure that involves isolating the nerve from any blood vessels that might be compressing it, has been successful in most of the patients, but it’s also invasive and can come with some risks. Rhizotomy, a procedure intended to disrupt nerve function, can also provide relief, although some people experience a return of pain over time. Lastly, deep brain stimulation and botulinum toxin injections might also be considered for cases where other treatments have failed.

Trigeminal neuralgia is a medical condition that causes facial pain. However, it’s crucial for doctors to differentiate this condition from other causes of facial pain through a thorough medical history and physical examination. Conditions that can also cause similar facial pain include:

Postherpetic trigeminal neuralgia, which happens after a shingles infection
Pain related to teeth or dental issues
Short-lasting unilateral neuralgiform headache attacks (SUNA)
Short-lasting unilateral neuralgiform headache attacks with redness and tearing in one eye (SUNCT)
Trigeminal neuropathy, a condition where the trigeminal nerve is damaged
Temporomandibular joint syndrome, a condition that affects the jaw joint
Glossopharyngeal neuralgia, a condition that causes throat and ear pain

Through a careful review of symptoms and relevant tests, doctors can correctly diagnose and treat the cause of the facial pain.

What to expect with Trigeminal Neuralgia

Trigeminal neuralgia, though not fatal, can cause lifelong discomfort and can disrupt day-to-day life. This condition can be unpredictable; some patients might experience episodes of pain lasting for weeks or months, followed by periods with no pain, while others suffer from ongoing facial pain, with surges of intense pain episodes.

Medical treatments may become less effective over time, and while surgery can provide instant relief from the pain, it’s common for the pain to return. Trigeminal neuralgia, therefore, can be quite challenging to manage over the long term.

Possible Complications When Diagnosed with Trigeminal Neuralgia

Trigeminal neuralgia, a nerve-related facial pain, can become a long-term condition if not treated and can cause depression. The facial muscle spasms linked to this condition can lead to social withdrawal and loneliness. Even though anticonvulsant treatments can help, they can also cause unwanted side effects. There are risks involved in surgery and rescue treatments as well. Despite the various treatment options available, some patients might still suffer from facial numbness, loss of corneal sensation, weakened jaw or a severe facial discomfort known as anesthesia dolorosa.

Common Side Effects of Trigeminal Neuralgia:

Long-term facial pain
Depression
Social isolation because of facial muscle spasms
Adverse effects from anticonvulsant treatment
Risks involved in surgical and rescue treatments
Facial numbness
Loss of corneal (eye) sensation
Weakness in the jaw
Persistent facial discomfort known as anesthesia dolorosa

Preventing Trigeminal Neuralgia

People suffering from trigeminal neuralgia, which is a facial pain disorder, need to understand that the condition may have periods when it’s more active and other periods when it’s not. They should be aware of the different treatment approaches that are available to manage the pain, which can even include some invasive procedures.

Frequently asked questions

The prognosis for Trigeminal Neuralgia can be challenging as medical treatments may become less effective over time and surgery may provide temporary relief. Trigeminal Neuralgia can cause lifelong discomfort and disrupt day-to-day life. The condition can be unpredictable, with some patients experiencing episodes of pain followed by periods with no pain, while others suffer from ongoing facial pain with intense pain episodes.

Trigeminal neuralgia can be caused by pressure on the trigeminal nerve near the part where it enters the brain stem. This pressure is often due to an artery or a vein that is too close to the nerve. Other possible causes include tumors or other masses, such as meningioma, acoustic neuroma, epidermoid cyst, arteriovenous malformation, or saccular aneurysm. Certain conditions like multiple sclerosis, which damages the covering of nerve cells, can also increase the risk of developing trigeminal neuralgia.

Signs and symptoms of Trigeminal Neuralgia include: - Episodic or constant pain in the face - Electric or shock-like pain that lasts from a moment to several seconds - Pain mainly on one side of the face, often more common on the right side - Uncommon bilateral pain, but rarely happens simultaneously on both sides - Pain that is usually associated with the V2 and V3 divisions of the trigeminal nerve - Mild symptoms like eye watering, runny nose, and redness in the whites of the eyes when the V1 division is involved (though rare) - "Trigger zones" - specific areas where a light touch can set off the pain, typically near the midline of the face, especially around the nose and mouth - Known triggers include routine tasks such as brushing teeth, shaving, washing the face, chewing, talking, smoking, and exposure to cold air - Sleep is generally uninterrupted by episodes of pain - In younger patients, symptoms of multiple sclerosis may also be taken into account - During medical examinations, patients typically don't show any specific neurological deficits, but comprehensive physical exams are necessary to rule out other potential causes of facial pain - Presence of sensory loss, loss of the corneal reflex, or weakness in the facial muscles may indicate secondary trigeminal neuralgia, requiring specific attention - In-depth oral exam is also needed to differentiate from dental causes of pain - Presence of trigger zones strongly suggests trigeminal neuralgia.

The types of tests that are needed for Trigeminal Neuralgia include: 1. Neuroimaging studies, such as MRI or MRA, to identify the cause of secondary trigeminal neuralgia and to highlight possible vascular compression. 2. Specialized form of MRI known as FIESTA sequencing, with or without gadolinium contrast, to get detailed images of the blood vessels and the brain, particularly beneficial for certain cases. 3. Genetic test for patients of Asian ancestry before taking carbamazepine, to check for the HLA-B*15:02 allele which can increase the risk of harmful skin reactions. 4. Other antiepileptic drugs may be suggested if the initial medication therapy is not effective, such as gabapentin, clonazepam, lamotrigine, phenytoin, and valproic acid. 5. Surgical intervention may be considered if medical therapy does not bring enough relief, such as microvascular decompression, rhizotomy, peripheral nerve block, deep brain stimulation, and botulinum toxin injections.

Postherpetic trigeminal neuralgia, Pain related to teeth or dental issues, Short-lasting unilateral neuralgiform headache attacks (SUNA), Short-lasting unilateral neuralgiform headache attacks with redness and tearing in one eye (SUNCT), Trigeminal neuropathy, Temporomandibular joint syndrome, Glossopharyngeal neuralgia.

The side effects when treating Trigeminal Neuralgia include: - Feeling sleepy or dizzy - Double vision - Feeling sick - Low sodium levels in the body (hyponatremia) - Dizziness - Confusion - Sleepiness - Upset stomach - Facial numbness - Loss of corneal (eye) sensation - Weakness in the jaw - Persistent facial discomfort known as anesthesia dolorosa

A neurologist or a neurosurgeon.

Trigeminal neuralgia affects 4 to 13 per 100,000 people each year.

Trigeminal Neuralgia is typically treated with medical therapy as the initial treatment. The main drugs used are antiepileptics such as carbamazepine or oxcarbazepine, which control pain by obstructing nerve cell signals. However, these medications may take a couple of weeks to start working. If the first line therapy is not effective, other antiepileptic drugs like gabapentin, clonazepam, lamotrigine, phenytoin, and valproic acid may be suggested. Additionally, a muscle relaxant called baclofen can help decrease the intensity and severity of pain attacks. If medical therapy does not provide enough relief, surgical intervention may be considered, including procedures like microvascular decompression, rhizotomy, peripheral nerve block, deep brain stimulation, and botulinum toxin injections.

Trigeminal neuralgia, also known as tic douloureux, is a long-term pain condition that causes quick, sharp bursts of pain that feel like electric shocks. It affects the areas connected to the trigeminal nerve, which supplies feeling to the forehead, cheek, and lower jaw.