Causes and classification
The disease debuts predominantly in middle and older age (around 50 years), with women being affected twice as often as men. Depending on the etiology, two main forms of the pathology are distinguished:
- Idiopathic glossopharyngeal neuralgia. It occurs in the vast majority of cases. It is based on neurovascular conflict: the nerve root is compressed by a blood vessel at its exit zone from the brainstem. Most often, the culprit of compression is the posterior inferior cerebellar artery.
- Symptomatic (secondary) neuralgia. It develops against the background of other diseases that damage or compress the nerve. These include:
- Tumor processes in the region of the brainstem, cerebellopontine angle, or pharynx.
- Metastatic involvement of lymph nodes and neck tissues.
- Infectious and inflammatory diseases of the tonsils and pharynx.
- Vascular malformations.
- Anomalously elongated styloid process of the temporal bone.
- Demyelinating diseases such as multiple sclerosis.
Clinical presentation
The main symptom of the disease is a pain syndrome with specific characteristics. Patients describe the pain as excruciatingly sharp, comparable to a dagger stab or a powerful electric shock. The paroxysm is brief (usually up to 30 seconds, maximum up to 2 minutes). In the vast majority of cases, the pain is unilateral, and only in 10–25% of observations are both sides affected.
Pain propagation pattern: The pain impulse originates in the zone of the tongue base, tonsils, or pharynx. Then it rapidly irradiates to the eustachian tube, the auricle area, and the angle of the mandible. Between attacks, some patients retain moderate or mild background pain in these same zones.
Triggers and trigger zones: The attack rarely occurs spontaneously. Everyday actions act as provokers: chewing, yawning, speaking, and, above all, swallowing. Touching the so-called trigger zones (most often the tonsils or the base of the tongue) can also trigger a pain response.
Features of neurological status: There is no gross neurological deficit in the idiopathic form. However, specific symptoms may occur:
- Dysgeusia (all stimuli taste bitter) or hypersensitivity to bitterness.
- Changes in saliva volume (salivation or hyposalivation).
- Reflex spasm of the pharyngeal muscles during the act of swallowing.
A special form — vagoglossopharyngeal neuralgia. It occurs with combined compression of the glossopharyngeal and vagus nerves. In this case, the pain paroxysm is accompanied by pronounced vegetative reactions: cardiac arrhythmias up to loss of consciousness (syncope).
Diagnostic criteria
The diagnosis is established primarily on the basis of a thorough history taking and analysis of the clinical picture. To make an accurate diagnosis, the patient's condition must meet strict criteria:
- At least three documented attacks of unilateral facial pain.
- Pain sensations localized in the ear, pharynx, posterior third of the tongue, tonsils, and/or under the angle of the mandible.
- The pain has at least three of the four characteristics: paroxysmal course (from seconds to 2 minutes); extremely high intensity; shock-like/dagger-like character; clear association with swallowing, speaking, yawning, or coughing.
- Absence of other neurological deficits.
- Other possible causes of the pain syndrome have been excluded.
Disease course and differential diagnosis
The disease begins with periods of exacerbations, which are replaced by prolonged remissions, sometimes reaching two to three years. However, over time, the disease progresses: the pain-free intervals become shorter and rarer, and the pain attacks become more severe and frequent.
An important consequence of progression is rapid weight loss. Patients live in constant fear of swallowing, as it is what provokes the excruciating pain. This forces them to sharply restrict food and fluid intake.
When conducting a differential diagnosis, it is important to pay attention to the nature of the pain. Strictly paroxysmal pain is typical for the idiopathic form. If the patient complains of constant dull pain in the innervation zone, this is a strong reason to suspect a secondary (symptomatic) nature of neuralgia, for example, the presence of a neoplasm.
Briefly: answers to topical questions
What diseases must glossopharyngeal neuralgia be differentiated from?
Glossopharyngeal neuralgia is most often misdiagnosed as trigeminal neuralgia, with which primary differentiation is required.
| Sign | Glossopharyngeal neuralgia | Trigeminal neuralgia |
|---|---|---|
| Localization of pain | Base of the tongue, tonsil, fauces, point at the angle of the mandible | Innervation zone of n. trigeminus branches |
| Trigger zones | At the base of the tongue | On the face, around the lips |
| Therapeutic effect | After swabbing the base of the tongue, fauces, and tonsil with anesthetics | Upon taking anticonvulsants (carbamazepine) |
In rare cases, a combination of neuralgia of both nerves in the same patient is possible.
Which drug groups are used for the medical treatment of glossopharyngeal neuralgia?
For the medical treatment of idiopathic glossopharyngeal neuralgia, anticonvulsants and antidepressants are used according to principles similar to the therapy of trigeminal neuralgia.
- Carbamazepine is the drug of choice; the dosing regimen is similar to that for trigeminal neuralgia
- Gabapentin is used when carbamazepine is ineffective
- Amitriptyline is used when carbamazepine is ineffective
In the absence of significant relief from medical therapy, surgical treatment is indicated.
Which surgical operations are used to treat idiopathic glossopharyngeal neuralgia when conservative therapy is ineffective?
The method of choice for conservative therapy failure in idiopathic glossopharyngeal neuralgia is microvascular decompression, which eliminates nerve compression by the posterior inferior cerebellar artery.
- Microvascular decompression — cessation of pain in 80–90% of patients, effect persists for more than 10 years in >50% of patients
- Peripheral blocks of the glossopharyngeal nerve with glycerol — alternative method
- Unilateral rhizotomy (root sectioning) — alternative method
Alternative methods are used in elderly patients and individuals with concomitant diseases at high risk of complications from decompression.
To memorize the propagation path of shooting pain, use the acronym B-T-T-E: Pharynx (Throat), Tonsils, base of Tongue, Ear.
Class and exam questions
At what age do the first symptoms typically appear?
The disease most frequently debuts in middle-aged and elderly individuals, with the mean age of onset being around 50 years.
Why can a pain attack be accompanied by loss of consciousness?
This is a sign of vagoglossopharyngeal neuralgia, in which the vagus nerve is compressed along with the glossopharyngeal nerve, leading to cardiac arrhythmias and syncope.
How to distinguish tumor-related pain from standard neuralgia?
Classic (idiopathic) neuralgia is characterized by short, extremely acute paroxysms. If the pain is of a constant, dull nature, the probability of a secondary (e.g., tumor) nature of the lesion is high.
What else to study on this topic
- Postherpetic trigeminal neuropathy and allodynia
- Destructive techniques: radiofrequency thermocoagulation and glycerol rhizotomy
- Radiosurgical treatment of facial pain (Gamma Knife)
- Motor cortex neurostimulation for refractory neuralgia
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