Trigeminal neuralgia

and Carola Felchner, science journalist

Dr. med. Fabian Sinowatz is a freelancer in the medical editorial team.

More about the experts

Carola Felchner is a freelance writer in the medical department and a certified training and nutrition advisor. She worked for various specialist magazines and online portals before becoming a freelance journalist in 2015. Before starting her internship, she studied translation and interpreting in Kempten and Munich.

More about the experts All content is checked by medical journalists.

With trigeminal neuralgia, severe facial pain occurs suddenly. They usually only last a few seconds, but can always come back. The symptoms come from the trigeminal nerve (Latin for triple nerve). It supplies the face, forehead, eyes, chin, upper and lower jaw through three branches. When damaged, the nerve reports severe facial pain to the brain. Here you can read everything you need to know about the causes, symptoms, diagnosis, therapy and prognosis of trigeminal neuralgia!

ICD codes for this disease: ICD codes are internationally recognized codes for medical diagnoses. They can be found, for example, in doctor's letters or on certificates of incapacity for work. G50

Brief overview

Definition: severe, attack-like facial pain emanating from the trigeminal nerve.
Frequency: Only about 4 out of 100,000 people in Germany suffer from trigeminal neuralgia. The disease usually only occurs after the age of 40. Due to the higher life expectancy, there are slightly more female than male patients.
Causes: often unclear (classic trigeminal neuralgia). Sometimes there is a causative illness (symptomatic trigeminal neuralgia).
Symptoms: lightning-fast, very short and extremely strong pain attacks in the face, often triggered by touch, speaking, chewing, etc.
Responsible specialist: neurologist or neurosurgeon
Therapy: medication or surgery, possibly supplemented by psychological support.
Prognosis: The pain can be controlled through therapy, but not permanently eliminated.

Trigeminal neuralgia: causes and risk factors

Depending on the cause, the International Headache Society (IHS) divides trigeminal neuralgia into two groups:

Symptomatic trigeminal neuralgia

Symptomatic trigeminal neuralgia is when radiological imaging or surgery reveals another disease to be the clear cause of the pain attacks. These possible causes include:

Diseases in which the protective sheaths of the nerve fibers (myelin sheaths) in the nervous system are destroyed (demyelinating diseases): e.g. multiple sclerosis (MS)
Brain tumors, especially so-called acoustic neuromas: These are rare, benign tumors of the auditory and equilibrium nerves. They press on the trigeminal nerve or an adjacent blood vessel so that both are pressed against each other. That triggers the pain.
stroke
Vascular malformations (angioma, aneurysm) in the area of the brain stem

Patients with symptomatic trigeminal neuralgia are on average younger than people with the classic form of the disease.

Classic trigeminal neuralgia

In classic trigeminal neuralgia, no other disease can be identified as the cause of the symptoms. This form of the disease is therefore also called "idiopathic trigeminal neuralgia" (idiopathic = without a known cause).

Experts suspect that the pain in those affected is caused by neighboring blood vessels pressing on the nerve and thus damaging the covering of the nerve (myelin sheath). Such a pathological contact between the vessel and nerve is more likely if the walls of the arteries (arteries) are thickened and rigid. This is the case with hardening of the arteries (arteriosclerosis). This can therefore increase the risk of trigeminal neuralgia.

Trigeminal neuralgia: symptoms

Trigeminal neuralgia is characterized by attack-like pain in the face that

set in suddenly and in a flash,
are extremely strong and
last a short time (fractions of a second to two minutes).

Trigeminal neuralgia pains are among the most severe pains of all. They can repeat themselves up to a hundred times a day (especially with the classic form of the disease).

Affected people often describe the pain as "shooting out of the blue" "or" bursting with electricity ". Sometimes the pain occurs without any trigger. Very often, however, there are such triggers that cause a pain attack. This can be something very common like:

Touching the skin of the face (by hand or wind)
Speak
Brush teeth
Chewing and swallowing

For fear of a pain attack, some patients eat and drink as little as possible. You can lose (dangerously) weight and develop dehydration as a result.

"There is no acute therapy"

Three questions for

Priv.-Doz. Dr. med. Charly Gaul,
Specialist in neurology
1
Who is particularly at risk of developing trigeminal neuralgia?

Priv.-Doz. Dr. med. Charly Gaul
Especially older people - the frequency increases with age. In younger patients, trigeminal neuralgia can occur especially if they have multiple sclerosis. This is then called symptomatic trigeminal neuralgia. It is therefore important to examine such patients very carefully.
2
Is there anything I can do to stop a pain attack?

Priv.-Doz. Dr. med. Charly Gaul
Because the attacks last so briefly, acute therapy is not possible. What can be done is to take anticonvulsants as a preventive measure, which are medications that are used to treat seizures. They inhibit the electrical discharges of the diseased nerves. In certain situations you can also apply local anesthetics as a preventive spray into the nostril of the affected half of the face. They prevent attacks from occurring while eating or brushing your teeth.
3
Is it justified to worry about the side effects of such drugs?

Priv.-Doz. Dr. med. Charly Gaul
If the dose of the mostly effective anticonvulsant is slowly increased, patient tolerance is usually acceptable. However, if the dose is increased rapidly or if high doses are required, side effects such as dizziness may increase. The side effects increase especially when several drugs are combined. So look for an experienced specialist - especially when interventions on the teeth are pending.
Priv.-Doz. Dr. med. Charly Gaul,
Specialist in neurology
Specialist in neurology, special pain therapy, neurological intensive care medicine. Since 2012 medical director of the Migraine and Headache Clinic Königstein im Taunus. Trigeminal neuralgia is one of the focuses that Dr. Gaul deals.

Differences between the forms of the disease

In classic trigeminal neuralgia, the second or third main branch of the trigeminal nerve (trigeminal nerve) is usually affected. The patients therefore often have unilateral pain in the area of the upper or lower jaw. There is no pain between attacks. The intense, shooting pains can trigger a reflex twitching of the facial muscles. It is also known as tic douloureux (French for "painful muscle twitching").

If all three branches of the trigeminal nerve or both halves of the face are affected, this is more indicative of symptomatic trigeminal neuralgia. In addition, patients with this form of disease are usually not pain-free between attacks. Sensory disturbances (e.g. tingling, numbness, etc.) often occur in the supply area of the trigeminal nerve.

Trigeminal neuralgia

The trigeminal nerve has three branches in the face: the forehead-eye branch, the upper jaw branch and the lower jaw branch. If the trigeminal nerve is damaged, severe facial pain occurs.

Trigeminal neuralgia can be very stressful psychologically. Therefore, many sufferers suffer from a depressive mood.

Trigeminal neuralgia: examinations and diagnosis

Not all facial pain is trigeminal neuralgia. For example, temporomandibular joint problems, dental diseases or cluster headaches can also trigger pain in the face.

It is therefore important to distinguish trigeminal neuralgia from the numerous other forms of headache and facial pain. The general practitioner can usually identify trigeminal neuralgia based on the typical course of pain. The right contact for the diagnosis and further examinations for this disease is a specialist in neurology or a specialist in neurosurgery.

The first step if trigeminal neuralgia is suspected is to take a medical history (anamnesis): The doctor asks the patient in detail about his symptoms. Possible questions are:

Where exactly are you in pain?
How long does the pain last?
How do you perceive the pain, for example as stabbing, pressing, surge-like?
In addition to the pain, do you have other complaints such as sensory disturbances in other parts of the body, visual disturbances, nausea or vomiting?
Do the pain attacks bother you emotionally?

The doctor will then perform a physical examination. For example, the doctor pays attention to whether the sensation (sensitivity) in the facial area is normal.

Further examinations must clarify whether the trigeminal neuralgia is caused by a causative disease or not. Symptomatic and classic trigeminal neuralgia are treated differently. Depending on the symptoms, the doctor can carry out one or more of the following examinations:

Magnetic resonance imaging (MRI): Using magnetic resonance or magnetic resonance imaging, the doctor can check whether a disease such as multiple sclerosis, brain tumor, stroke or vascular malformation (aneurysm) is causing the trigeminal neuralgia.

Collection and analysis of the nerve water: With a thin, fine hollow needle, the doctor can take a sample of the nerve water (cerebrospinal fluid) from the spinal canal (cerebrospinal fluid puncture). The laboratory examines whether the patient has multiple sclerosis.

Computed tomography (CT): This allows the bony structures of the skull in particular to be examined. Any pathological changes can be the cause of the pain attacks.

Angiography or magnetic resonance angiography (MRA): An X-ray examination of the blood vessels (angiography) in the skull area can identify any vascular malformations. With magnetic resonance angiography, the X-ray imaging of the vessels is carried out using magnetic resonance imaging. Imaging of the blood vessels is also useful before an operation so that the neurosurgeon can see exactly where the blood vessels run in the surgical area.

Electrophysiological examinations: These include, for example, trigeminal SEP (checking the functionality of sensitive nerve tracts, e.g. sensation of touch and pressure), checking the eyelid closing reflex, the masticatory muscle reflex (masseter reflex), etc.

Other examinations: Further examinations may be necessary, for example at the dentist, orthodontist or ENT specialist.

Trigeminal neuralgia: treatment

Basically, trigeminal neuralgia can be treated either with medication or surgery. There are differences between the two forms of the disease:

Classic trigeminal neuralgia is primarily treated with medication. Surgery is only performed here if the medication does not work or the side effects are too severe.
In symptomatic trigeminal neuralgia, the cause (such as a brain tumor) is often removed surgically. The procedure can reduce the pain or even turn it off for a while.

The fact that the causes of facial pain have not yet been fully clarified makes trigeminal neuralgia therapy difficult. If the right treatment is found, the pain can be relieved well, but never "turned off" completely or forever.

Medication for trigeminal neuralgia

The usual pain medication (such as ibuprofen, diclofenac, etc.) unfold their effect too late for the lightning-fast, short pain attacks. Trigeminal neuralgia therefore requires long-term preventive therapy with special pain medication.

For example, the active ingredients carbamazepine and oxcarbazepine are used. They are also used to treat epilepsy. The muscle-relaxing active ingredient baclofen often also helps. If possible, the doctor prescribes only one active ingredient alone (monotherapy) for trigeminal neuralgia. In the case of severe pain, two medications can also be useful (combination therapy).

Acute pain can be treated with the anticonvulsant phenytoin in the case of inpatient treatment in the hospital.

Surgical therapy for trigeminal neuralgia

In trigeminal neuralgia, surgery can relieve pain or even eliminate it in the long term. However, they can reappear after a few years. In addition, every operation naturally harbors risks. Before the operation, patients should therefore talk to the treating doctor in detail about the chances and risks of the procedure.

In principle, there are three surgical options for trigeminal neuralgia:

Classic surgical procedure (microvascular decompression according to Jannetta)

This method is used in healthy people with a low risk of surgery. The doctor places a Goretex or Teflon sponge between the nerve and the blood vessel through an opening in the back of the head. This is to prevent the trigeminal nerve from coming under pressure again.

About 82 percent of patients are pain-free immediately after the procedure. Ten years after the operation, the figure is 67 percent. The success rate for freedom from pain is lower for follow-up operations.

Possible side effects / risks of the operation include bleeding, injuries to the cerebellum and loss of hearing and numbness of the face on the affected side.

Percutaneous thermocoagulation (according to Sweet)

This procedure is suitable for people with a higher risk of surgery. The doctor pushes a probe through the skin to the so-called ganglion gasseri. This is a collection of nerve cell bodies of the trigeminal nerve. There, the pain fibers of the nerve in particular are destroyed - either through the application of local heat, mechanically using a pressure balloon or chemically using glycerine.

The success rate immediately after the operation is high: around 90 percent of the patients are initially pain-free. However, this success only lasts for about every second person.

A possible side effect is a sometimes painful loss of sensation on the affected half of the face.

Radiosurgical procedure

This is a comparatively gentle method of radiation therapy. The doctor irradiates the trigeminal nerve close to the brain stem once with a high dose of radiation. To do this, he uses the so-called gamma knife or cyber knife. Compared to the other two surgical methods, this procedure has fewer complications. But the chances of success are also lower:

If this procedure is performed without prior other surgeries, 63 to 75 percent of trigeminal neuralgia patients are pain-free after the procedure. If another operation has already taken place, the chances of success are significantly worse. Overall, the therapy usually only takes effect after a few weeks, i.e. significantly later than with the other methods.

The doctor treats trigeminal neuralgia in multiple sclerosis patients with medication (active ingredient misoprostol) or with percutaneous thermocoagulation and radiosurgical procedures.

Trigeminal neuralgia: course and prognosis

The course of the disease in trigeminal neuralgia is very variable. It is difficult to predict how much time will pass before the next pain attack. Sometimes there are days, weeks, months or even years between attacks.

In about 29 percent of those affected, it remains even after a single attack of trigeminal neuralgia. Often times, however, the attacks only occur occasionally at first, but accumulate over time.

Trigeminal neuralgia can have a massive impact on the everyday life of those affected - not only because of the severe pain attacks themselves, but also because of the fear of the next attack. Mental wellbeing can also suffer as a result. Therefore, some patients also develop a depressive mood. It is therefore sensible to supplement drug and / or surgical therapy for trigeminal neuralgia with psychological or psychotherapeutic treatment if necessary.

With the right treatment plan, the pain of trigeminal neuralgia can be reduced or relieved for at least a while. However, the disease cannot currently be completely cured. It is also not yet known whether and how trigeminal neuralgia can be prevented.