Disease Name Hypoglossal neuralgia is a paroxysmal severe pain that occurs in the area of the hypoglossal nerve division. The nature of the pain is very similar to trigeminal neuralgia, which is also divided into two categories: primary and secondary. The pain occurs on one side of the tongue, throat, tonsils, the root of the ear and the back of the jaw, sometimes the root of the ear is the main manifestation of pain. Disease Overview Hypoglossal neuralgia is an episodic severe pain limited to the distribution area of the hypoglossal nerve. The etiology and pathogenesis of the disease are not completely clear, but may be the result of a “short circuit” between the afferent impulses of the glossopharyngeal nerve and the vagus nerve caused by demyelination of the nerve. It can also be seen in tumors of the jugular foramen, skull base, nasopharynx, tonsils, etc., local arachnoiditis or aneurysm, which are called secondary glossopharyngeal neuralgia. In recent years, the development of microvascular surgery, found that some patients with hypoglossal nerve by the vertebral artery or the posterior inferior cerebellar artery compression. Clinical manifestations The disease usually starts after the age of 35, and is more common in men than in women. Sudden pain, the nature of which is similar to trigeminal neuralgia, is located in the tonsils, root of the tongue, pharynx, the deep part of the ear canal, etc., with intermittent episodes, each lasting a few seconds to 1-2 minutes, which can be triggered by swallowing, speaking, coughing, yawning, etc., and there can be a trigger point for the pain in the posterior wall of the pharynx, root of the tongue, and the fossa of the tonsils. In some cases, it can be accompanied by pharyngeal spasm, cardiac rhythm disorders, hypotensive syncope and so on. Clinically, the symptoms manifested by glossopharyngeal neuralgia can be basically categorized as follows: 1. Prevalent age: 35-50 years old. 2, the onset of the site: tonsil area, pharynx, the root of the tongue, the neck, the deep part of the ear canal, the posterior region of the mandible at. 3.Nature of pain: paroxysmal severe pain, such as cut-like, pierce-like, painful convulsions. 4.Time of pain: frequent in the morning, morning, and may have attacks during sleep, which can be differentiated from trigeminal neuralgia. 5.Foreign body sensation and infarction: there is foreign body sensation and infarction in the pharynx and larynx at the onset, which leads to frequent coughing. 6, pain stimulating factors: palpation can make pain occur, also known as “trigger point”. Commonly found in the tonsil area, the external auditory canal, the root of the tongue. Whenever swallowing, chewing, yawning, coughing can trigger pain. 7, There are intermittent periods. 8, The patient has dehydration and emaciation. It is caused by fear of pain and less eating. 9, In severe cases, there may be cardiac arrhythmia, cardiac arrest, fainting, convulsions, seizures, laryngospasm, and excessive parotid secretion. Etiology of the disease The etiology is unknown and no pathological changes are found (except for rare cases with tumors of the pontine cerebellar angle or tumors of the neck). There are more male than female cases, and the onset usually occurs after the age of 40. Similar to trigeminal neuralgia, there are intermittent episodes of brief, severe, intolerable pain, which may be spontaneous or triggered by some action (e.g., chewing, swallowing, speaking, or sneezing). The pain lasts from a few seconds to several minutes and usually begins in the tonsil area or at the base of the tongue and may radiate to the ear on the same side. The pain is strictly unilateral. In 1-2% of cases, hyperactivity of the vagus nerve may cause sinus arrest of the heart with syncope, and there may be a long interval between episodes. According to the different causes of the onset of the disease, the hypoglossal neuralgia can also be divided into primary hypoglossal neuralgia and secondary hypoglossal neuralgia two kinds. I. Primary glossopharyngeal neuralgia The etiology of primary glossopharyngeal neuralgia is still unclear, and it may be caused by nerve desheathing. The age of onset is more than 40 years old, and there are more males than females. Clinical manifestations: 1. Clinical manifestations and pain sites: different from primary trigeminal neuralgia, the pain occurs in the root of the tongue, pharynx, tonsils, root of the ear and the back of the lower jaw, sometimes with the root of the ear as the main manifestation of pain. 2. 2, the onset of the situation and the nature of the pain: with trigeminal neuralgia, the pain is usually sudden onset, sudden stop, each attack lasts for a few seconds or dozens of seconds, usually no more than two minutes. It can also be cut, pins and needles, tearing, burning, electric shock-like severe pain. 3, triggering factors: often in swallowing, talking, coughing or yawning when the pain is triggered. 4, board machine point: often have board machine point, part for more in the back wall of the pharynx, tonsils, tongue root, etc., a few can be in the external ear canal. 5.Other symptoms: swallowing action will often trigger the pain attack, although there is no abnormality in the interictal period, but the fear of inducing pain and dare not eat, the patient often have symptoms such as emaciation, dehydration, laryngeal spasm, arrhythmia and hypotensive syncope. 6.Neurological examination: normal. Clinically, most of the hypopharyngeal neuralgia belongs to primary hypopharyngeal neuralgia, temporary pain relief is not effective, secondary hypopharyngeal neuralgia Some cerebellar pontine angle tumors, sphenoid retinitis, vascular diseases, nasopharyngeal tumors, or caudal synostosis, etc. can provoke the hypopharyngeal nerve and cause pain in the hypopharyngeal nerve division area, which is known as secondary hypopharyngeal neuralgia. Its clinical manifestations are as follows: 1. Pain in the area of the division of the glossopharyngeal nerve. The pain episodes last for a long time or persistent, the triggering factors and board machine point is not obvious, and it is heavy at night. Symptoms of hypoglossal neuralgia damage. Jaw arch paralysis, soft palate and pharyngeal hyperalgesia or loss of sensation, impaired taste and general sensation in the posterior third of the tongue, weakened or absent pharyngeal reflexes, abnormal parotid secretion function. 2. Neighboring cerebral neuralgia. Jugular venous foramen syndrome and Horner’s symptom may occur; cerebellar pontine angle syndrome may also occur. 3.If caused by nasopharyngeal carcinoma, lumps can be found in the nasopharynx and the lymph nodes in the neck are enlarged. Secondary glossopharyngeal neuralgia is mostly secondary to intracranial tumors and foreign bodies, which are often treated with craniotomy. Diagnostic examination 1, in the history, ask about the location and nature of the pain, whether it is related to diet, and whether the pain radiates to the ear. 2, Ask the patient to eat and observe the triggering pain. Whether the trigger point is at the tonsil trap, apply cocaine solution to the affected side of the pharynx to find out whether the pain is relieved. 3, Nasopharyngeal and posterior group cerebral nervous system examination for positive signs. 4.Differential diagnosis should be differentiated from trigeminal neuralgia and cerebellar pontine horn tumor. Disease diagnosis I. History and symptoms Middle-aged men are common, often due to swallowing, talking, coughing and induced by the tonsils, pharynx or ear canal deep episodes of severe pain, each lasts a few seconds, some patients may be accompanied by bradycardia, fainting and convulsions and other symptoms during the attack. Second, physical examination findings In addition to the pain attack, the neurological examination is mostly without abnormal findings. There may be trigger points at the base of the tongue and tonsil fossa. It is often differentiated from trigeminal neuralgia, nasopharyngeal tumor invading the pharynx, and pain caused by the structures of the skull base. Treatment 1, drug therapy (1) phenytoin sodium 0.1g, 3 / d, oral; (2) vitamin B1, B12: etc.; (3) carbamazepine 0.2g, 3 / d, oral. Drug treatment in the early stage of the disease can often achieve relatively satisfactory clinical efficacy, but with the aggravation of pain, the drug is often months or years after the gradual loss of effect. 2, nerve block method for percutaneous puncture jugular venous orifice radiofrequency treatment, applicable to: (1) drug therapy is ineffective or can not tolerate the adverse effects of drugs; (2) elderly or poor general condition, can not tolerate microvascular decompression surgery; (3) combined with multiple sclerosis cases. The main problems of this treatment method are the high recurrence rate of pain (23%-54%) and the dysphagia, choking and hoarseness caused by nerve damage. Microvascular decompression surgery Microvascular decompression surgery is currently the safest and most effective surgical treatment, and its cure rate can reach 99%. Surgery is suitable for: (1) patients who have failed drug or percutaneous puncture treatment; (2) patients who are in good general condition, have no serious organic lesions, and can tolerate surgery; (3) patients who have ruled out multiple sclerosis or tumors of the bridge cerebellar angle and other lesions. Most patients’ pain disappears after surgery, and 99% of patients can be cured. Microvascular decompression was firstly proposed by Prof. Jannatta in 1967, and later Haines et al. conducted a more in-depth anatomical study on the relationship between the glossopharyngeal nerve and the microvessels, and found that 92.5% of the cases with pontine paravascular microvessel compression of the root of the glossopharyngeal nerve showed symptoms of glossopharyngeal neuralgia. The blood vessels that compress the nerve to produce pain are called “responsible vessels”, and the common responsible vessels are: (1) superior cerebellar artery (55%), the superior cerebellar artery can form a vascular loop that extends caudally to the caudal side, which is in contact with the entry of the glossopharyngeal nerve into the brainstem, and mainly compresses the superior or superior-inferior aspect of the nerve root. (2) The anterior inferior cerebellar artery (30%), which generally compresses the glossopharyngeal nerve from below, may also form a gripping compression of the glossopharyngeal nerve together with the superior cerebellar artery. (3) Basilar artery, with age and hemodynamic effects, the basilar artery may bend to both sides and compress the glossopharyngeal nerve root, usually to the side of the smaller vertebral artery. (4) Other rare responsible vessels include the posterior inferior cerebellar artery, varicose veins, transverse cerebral pontine veins, lateral veins, and basilar venous plexus. The responsible vessel can be one or multiple, either arterial or venous. Microvascular decompression is performed by placing the patient in the supine lateral head position with the healthy side on top and elevating the upper body about 20°. A transverse or longitudinal incision of about 4 cm is made behind the ear, and a posterior approach to the sigmoid sinus is used, with a bony window of about 1.5 cm × 1.5 cm, and the dura is incised in the shape of ⊥ to release some of the cerebrospinal fluid so that the cerebellum can be subsided and then entered into the pontine cerebellar angle. Surgery was performed endoscopically. The arachnoid membrane of the pontine pool was cut, and cerebral nerves VII, VIII, IX, and X were explored. The arachnoid membrane around the glossopharyngeal nerve and vagus nerve root was fully loosened, and the responsible blood vessels on the medial side of the glossopharyngeal nerve and vagus root were explored, and the glossopharyngeal nerve was cut from the pontine root area to the jugular foramen to free the vagus nerve and responsible blood vessels and decompress them. The cerebrospinal fluid is replenished with warm saline, and the dura and scalp layers are sutured to complete the procedure. Microvascular decompression is the only method of treatment that addresses the etiology of glossopharyngeal neuralgia and preserves the anatomical integrity of the glossopharyngeal nerve, so normal nerve function of the glossopharyngeal nerve is preserved. In some patients, it can also eliminate the hypertensive state caused by vascular compression of the brainstem and achieve the goal of eradicating hypertension. Because microvascular decompression has the advantages of obvious pain relief, non-destructive, few side injuries, and extremely low recurrence rate, it is now internationally recognized as the safest and most effective method for treating glossopharyngeal neuralgia. At present, microvascular decompression is the most widely used treatment for glossopharyngeal neuralgia, which is highly respected by most experts and scholars at home and abroad, because it is the only treatment for glossopharyngeal neuralgia that targets the “cause” of the disease. Compared with other therapies, its biggest advantage is that on the basis of long-term effective solution to the pain, it can retain the patient’s normal sensation, change the numbness and discomfort in the innervated area of the glossopharyngeal nerve after the previous treatment, improve the quality of life of the patients, so that the majority of patients are willing to accept the treatment. 4, Chinese medicine The motherland medicine has a long history and culture, the dialectical treatment of Chinese medicine is a cultural treasure, “Quanlong Tang” is a classic formula, based on the main formula, dialectical treatment, plus or minus medication, combined with acupuncture for better results. 5, closed therapy in the equivalent of the mandibular angle and mastoid line of the midpoint, with 10% procaine 5-10mg vertical injection in the subcutaneous 1-1.5cm, can stop the pain. RELATED INFORMATION The localization of pain, the fact that pain episodes can be provoked by swallowing movements or by touching the tonsils with a tongue depressor, and the fact that local anesthesia of the pharynx with lidocaine can temporarily eliminate the pain episodes (after local anesthesia all means of provocation are ineffective) are the features that distinguish glossopharyngiopharyngiosis from trigeminal neuralgia of the mandibular branch. Tumors of the tonsils, pharynx, and pontine cerebellar angle, as well as metastatic lesions within the anterior cervical triangle, should be excluded by brain imaging. Carbamazepine is the drug of choice. If needed, phenytoin sodium, baclofen or amitriptyline in the same dosage as used for trigeminal neuralgia treatment, or trazodone, 150-400 mg/d in 3 divided doses, may be added. If medication is ineffective, pharyngeal cocaine anesthesia may provide temporary relief, but surgery may be necessary. If the pain is limited to the pharynx, the cervical glossopharyngeal nerve may be avulsed or withdrawn; if the pain is widespread, intracranial glossopharyngeal neurotomy is necessary.