12.3: Glossopharyngeal neuralgia

Glossopharyngeal (Vagoglossopharyngeal) Neuralgia

The IHS (Olesen, 1988) has divided glossopharyngeal neuralgia into the idiopathic type 12.3.1 and the symptomatic type 12.3.2. The condition is described as a severe transientstabbing pain experienced in ear, base of the tongue, tonsillar fossa, or beneath the angle of the jaw. The pain is therefore felt in the distribution of the auricular and pharyngeal branches of the vagus nerve as well as that of the glossopharyngeal nerve. It is commonly provoked by swallowing, talking, and coughing, and may remit relapse in the fashion of trigeminal neuralgia. The following diagnostic criteria have been listed.

A. Paroxysmal attacks of facial pain which last a few seconds to less than two minutes.

B. Pain has at least 4 of the following characteristics:

1. Unilateral location

2. Distribution within the posterior part of the tongue, tonsillar fossa, pharynx, or beneath the angle of the lower jaw, or in the ear.

3. Sudden, sharp, stabbing or burning in quality

4. Pain intensity severe

5. Precipitation from trigger areas or by swallowing, chewing, talking coughing, or yawning

C. No neurological deficit.

D. Attacks are stereotyped in the individual patient.

E. Other causes of pain ruled out by history, physical and special investigations.

Paroxysmal attacks of pain occasionally occur in the sensory domain of the glossopharyngeal nerve. They are similar to those of trigeminal neuralgia (although Dandy (1936) thought the pain was more severe) except for their position. The incidence of glossopharyngeal neuralgia to trigeminal neuralgia has been reported to be between 1:200 (Harris, 1937) and 1:70 (Spurling and Grantham, 1942). It is basically a disease of adults. Of 217 patients with glossopharyngeal neuralgia studied by Rushton et al. (1981), only 1 patient was under age 20, and his age was 18 years. Sinha (1962) reported a patient in whom the onset of pain was at 16 years of age. In about 43% of the patients reported by Rushton et al. (1981), the pain began prior to 50 years of age. In most cases, glossopharyngeal neuralgia is unilateral; however, Peet (1935) reported a young woman with bilateral glossopharyngeal neuralgia and unilateral trigeminal neuralgia. Four of the patients in the series reported by Rushton et al. had bilateral pain.

The association of trigeminal and glossopharyngeal neuralgia has been reported by Peet (1935), Brzustowicz (1955), and others (Chawla and Falconer, 1967; Laha and Jannetta, 1977). Twenty-five of the patients reported by Rushton el al. were found to have combined trigeminal and glossopharyngeal neuralgia. Knuckey and Gubbay (1979) have reported the familial occurrence of this association.

The pain in glossopharyngeal neuralgia has been described as sharp, shooting, stabbing, or ``like a needle'' and commonly lasts from a few seconds to a minute. However, Dandy (1936) noted that the pain of glossopharyngeal neuralgia may be more constant, and a substantial number of patients will experience a dull, aching or burning sensation or even a painful feeling of pressure that persists for several minutes or even several hours rather than typical tic-like pain. The pain may occur anywhere in the regions supplied with somatosensory elements of the glossopharyngeal and vagus nerves. Patients usually localize the pain to the ear or tonsil. In the series of 217 patients studied by Rushton et al. (1981), pain was localized to the ear 155 times; tonsil, 147; larynx, 69; and tongue, 43. The most common combination was tonsil and ear, noted by 68 patients (31%). Because the pain may begin in the tonsil and radiate to the ear, it may be confused with a mandibular division trigeminal neuralgia (Jefferson, 1931b), and patients with glossopharyngeal pain of this type have been subjected to division of the trigeminal sensory root as a result of misdiagnosis. In the same manner that sensory stimuli induce trigeminal pain, such stimuli as swallowing, chewing, coughing, and talking may trigger glossopharyngeal pain, as may certain foods and cold drinks (Rushton et al., 1981). In some patients, touching of the external auditory canal, the side of the neck, the skin anterior to the ear, or the area over the mastoid may trigger severe pain.

Although Dandy (1936) thought that spontaneous remissions did not occur, Rushton et al. (1981) found that patients with glossopharyngeal neuralgia commonly experience a remission of pain that may last months to years. Only 37 of 198 patients (19%) questioned by Rushton et al. reported no periods of pain relief.

White and Sweet (1969) have proposed that the term vagoglossopharyngeal neuralgia be used instead of glossopharyngeal neuralgia since the older term diverts attention from the part often played by the vagus nerve in this condition. During short attacks of pain, the patient may experience only slowing of the pulse. In other cases, however, there may be incomplete or complete heart block (Greeson and Linden, 1981). During longer attacks, asystole may occur and long, severe attacks may be marked by protracted cardiac arrest, absence of pulse, pallor, mental confusion, syncope, or convulsions (Jacobson and Ross Russell, 1979; Taylor et al., 1981b). Roulhac and Levy (1950) described a patient in whom determination of the origin of epileptic seizures was the major problem. Detailed questioning revealed the crucial fact that sudden severe burning pain in the left side of the throat and the posterior part of the tongue preceded each seizure by a few seconds. During electrocardiographic recording, the pain was reproduced by stimulation of the left tonsil and was accompanied by bradycardia and cardiac arrest with convulsions. About 30 cases of glossopharyngeal neuralgia associated with syncopal symptoms or convulsions have been reported in the literature (Rushton et al., 1981). The usual mechanism proposed has been spillover of impulses from the glossopharyngeal nerve via the tractus solitarius to the dorsal motor nucleus of the vagus nerve, resulting in reflex bradycardia, heart block, or asystole. Other investigators have postulated that the sensitivity of the vagus nucleus itself is increased (Stowell and Gardner, 1952), and it has also been suggested that an artificial synapse develops in the more proximal portion of the glossopharyngeal nerve so that fibers in the sinus nerve of Hering are stimulated by impulses of glossopharyngeal somatic sensory or motor fibers (Kjellin et al., 1959; Alpert et al., 1977).

Rushton et al. ( 1981 ) have advocated the application of a 10% solution of cocaine or other surface anesthetic to the affected tonsil and pharynx as a test for glossopharyngeal neuralgia. They consider the test successful if the patient's pain is relieved for 1---2 hours, during which time the patient should be able to eat, drink, and tolerate probing in the trigger zone without pain.

Ekbom and Westerberg (1966) treated four patients with glossopharyngeal neuralgia with carbamazepine (Tegretol). The response of all these patients to the drug was favorable. Pain disappeared while the patient was taking the drug but returned when it was discontinued. Rushton et al. (1981) treated 18 patients with diphenylhydantoin (Dilantin) alone, only 2 of whom reported significant pain relief. Twenty patients in this series received carbamazepine alone, but, again, only four patients had sufficient relief to enable them to avoid further therapy. It would thus appear that, unlike trigeminal neuralgia, the treatment of glossopharyngeal neuralgia is primarily surgical. Alcohol injections are contraindicated because of the danger of injuring the vagus. Intracranial section of the nerve (including the rostral vagal rootlets) was introduced by Dandy (1936) since extracranial section is followed by regeneration of the nerve and return of the neuralgia. Section of the glossopharyngeal nerve has, however, been followed by sustained hypertension in some patients (Nagashima et al., 1976; Ripley et al., 1977; Alonso et al., 1981 ). Harris (1937) advocated avulsion of the nerve through extracranial operation. As in the case of trigeminal neuralgia, Jannetta (1977) considers glossopharyngeal neuralgia to be a cranial nerve hyperexcitability syndrome, often caused by vascular compression. He has described two patients who underwent suboccipital craniotomy and exploration for this disorder. Both patients were found at surgery to have cross-compression of the glossopharyngeal and vagus nerves at the brainstem entry root level by the vertebral artery (Figure 36.18). In one of the patients, decompression was performed; however, the patient had a hypertensive crisis in the recovery room, developed a large intracerebral hemorrhage, and expired. Morales et al. (1979) have described the case of a 56-year-old woman with glossopharyngeal neuralgia who underwent a retromastoid craniectomy after her pain failed to respond to Tegretol. At surgery, a tortuous left vertebral artery was found to be compressing the ninth and tenth cranial nerves. Postoperatively, the patient's pain was relieved.

In 19 of the patients reported by Rushton et al. ( 1981 ) who underwent surgical section of the intracranial glossopharyngeal nerve, a comment was made by the operating surgeon regarding compression of this nerve or the vagus nerve by an arterial loop. In five cases, the nerves that were being compressed appeared enlarged and congested.

Percutaneous radiofrequency thermocoagulation has been used effectively in a few published cases of glossopharyngeal neuralgia (Laha and Jannetta, 1977; Tew, 1977; Broggi and Siegfried, 1979; Lazorthes and Verdie, 1979; Isamat et al., 1981).

Unlike trigeminal neuralgia, glossopharyngeal neuralgia rarely appears to be associated with intracranial tumors (Weisenburg, 1910) and even more rarely with multiple sclerosis (Kahana et al., 1973).

Glossopharyngeal neuralgia has no particular ophthalmologic significance except from the standpoint of the differential diagnosis of facial pain, although the patient reported by Kjellin et al. (1959) also had hypersecretion of the ipsilateral lacrimal gland during attacks.
 

 

 

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