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Glossopharyngeal and other Facial Neuralgias
CHAPTER 19
GLOSSOPHARYNGEAL AND OTHER FACIAL NEURALGIAS Robert A. Duarte, MD, and Charles E. Argoff, MD 1. What is the clinical presentation of glossopharyngeal neuralgia? The pain of glossopharyngeal neuralgia is similar in many ways to that seen in trigeminal neuralgia, but it has a different distribution. It presents with paroxysms of lancinating pain that involve the glossopharyngeal and vagus nerves. Pain is felt around the jaw, throat, ears, larynx, and/ or base of the tongue. The pain is typically unilateral and lasts for about 1 minute. Multiple attacks can occur throughout a day and may even awaken the patient out of a sound sleep. The usual triggers are talking and chewing. Odynophagia is a specific trigger in glossopharyngeal neuralgia that is rarely, if ever, seen in trigeminal neuralgia. When glossopharyngeal neuralgia is not caused by an underlying tumor, spontaneous remissions often occur. 2. Define odynophagia. Odynophagia is pain upon swallowing. 3. How common is glossopharyngeal neuralgia? Glossopharyngeal neuralgia is an uncommon disorder with a prevalence of only about 1/100 that of trigeminal neuralgia. Symptoms of the primary disorder usually begin when the patient is in his or her 60s. 4. A patient experiences neck pain upon swallowing and a sudden loss of consciousness. What is a likely explanation? Swallow syncope is a syndrome of unclear mechanism that occurs in patients with glossopharyngeal neuralgia. It is thought that a barrage of impulses from the glossopharyngeal nerve, through the tractus solitarius, to the dorsal motor nucleus of the vagus nerve produces bradycardia or brief asystole. It is most commonly seen in patients with tumors of the neck and, in previously operated-on patients, usually represents tumor recurrence. 5. What is the difference between idiopathic glossopharyngeal neuralgia and secondary glossopharyngeal neuralgia? The difference between idiopathic and secondary glossopharyngeal neuralgia is a clearly identified underlying cause. Clinically, idiopathic glossopharyngeal neuralgia rarely, if ever, shows objective sensory impairment on physical examination. If there is sensory loss, a causative lesion (oropharyngeal tumor, peritonsillar infection, or vascular compression) must be sought. Tumors at the base of the skull, particularly around the jugular foramen, may also cause pain radiating to the throat, so careful imaging of the oropharynx and the base of the skull must be undertaken. 6. What are the recommended treatments for idiopathic glossopharyngeal neuralgia? Anticonvulsant or Baclofen therapy is the recommended treatment for idiopathic glossopharyngeal neuralgia, and agents such as carbamazepine, gabapentin, and phenytoin are preferred. In refractory cases, intracranial sectioning of the glossopharyngeal nerve and the upper rootlets of the vagus nerve and microvascular decompression of the glossopharyngeal nerve have been performed with some success.
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CHAPTER 19 GLOSSOPHARYNGEAL AND OTHER FACIAL NEURALGIAS 117 7. A patient has severe ear pain followed by ipsilateral facial weakness. What extremely important objective sign should be investigated? In any case of facial palsy, the search for a causative lesion is imperative. When there is severe ear and face pain, the lesion to be sought is a herpetic eruption. The vesicles most commonly affect the external auditory canal and ear but may be seen on the ear, palate, or pharynx. The syndrome of facial nerve palsy with a herpetic eruption is called Ramsay Hunt syndrome, a particularly painful neuralgia caused by zoster in the geniculate ganglion. 8. What is the most common presentation of acute herpes zoster in the face? Ophthalmic zoster (a V1 distribution of the trigeminal nerve) is the most common and most troublesome. The forehead and upper lid are involved with a vesicular eruption. Pain may precede the eruption for as long as 3 to 4 days. Viral vesicles may involve the eye itself. Aside from the general symptomatic treatment for zoster, the eye must be protected from secondary infection. The complication of postherpetic neuralgia is far more common in the older person than in the young and seems to be more common when the zoster rash affects the V1 distribution. 9. Describe the clinical features of occipital neuralgia. In occipital neuralgia, a sharp pain originates at the base of the skull and shoots up the back of the head. It may go as far forward as the coronal suture. It is generally unilateral and stabbing in nature. In idiopathic cases, there is no sensory loss, and some of the triggering mechanisms may be the same as those in other cranial neuralgias. However, occipital neuralgia may be a harbinger of more serious disease. Metastases to the occipital condyle can reproduce this syndrome, as can lesions at the C2 to C3 level of the spine. In these cases, local pressure will reproduce the pain, and there may be some accompanying sensory loss. Computerized tomography with overlapping cuts and bone windows can usually demonstrate the lesion. Occasionally, lesions around the foramen magnum can also produce this type of syndrome. Trauma is also a common cause; infection and chronic compression are less frequent causes. 10. What is superior laryngeal neuralgia? The superior laryngeal nerve, a branch of the vagus nerve, innervates the cricothyroid muscle of the larynx. This muscle stretches, tenses, and adducts the vocal cord. Superior laryngeal neuralgia usually appears as a postsurgical complication. There are paroxysms of unilateral submandibular pain, sometimes radiating to the eye, ear, or shoulder. This pain may be indistinguishable from glossopharyngeal neuralgia. It lasts from seconds to minutes and is usually provoked by swallowing, straining the voice, turning the head, coughing, sneezing, yawning, or blowing the nose. 11. What are the typical characteristics of Villaret’s syndrome? Villaret’s syndrome is characterized by unilateral paralysis of the ninth, tenth, eleventh, and twelfth cranial nerves, sometimes accompanied by a Horner’s syndrome on the same side. The syndrome may include glossopharyngeal neuralgia and is associated with lesions in the posterior retroparotid space. 12. What is Eagle’s syndrome? Eagle’s syndrome involves elongation of the styloid process of the temporal bone, which may cause impingement on the glossopharyngeal nerve and is thought to be one of the causes of secondary glossopharyngeal neuralgia. 13. Define sphenopalatine neuralgia. Sphenopalatine neuralgia has been given a number of names, including lower half headache, greater superficial neuralgia, Sluder’s neuralgia, and atypical facial pain. It is an uncommon form of facial neuralgia. The key clinical features include unilateral pain in the face (usually around the nasal region) lasting for days and associated with nasal congestion, otalgia, and tinnitus.
118 CHAPTER 19 GLOSSOPHARYNGEAL AND OTHER FACIAL NEURALGIAS Unlike trigeminal and glossopharyngeal neuralgia, sphenopalatine neuralgia is usually not associated with a trigger zone. Some authors believe that this is not a separate syndrome and may simply be a variation of cluster headache. Sphenopalatine ganglion blocks have been tried with minimal success, but this treatment remains controversial.
KEY POINTS 1. The typical triggers of glossopharyngeal neuralgia are talking and chewing. 2. Because idiopathic glossopharyngeal neuralgia rarely if ever is associated with objective sensory loss, when such sensory loss is present glossopharyngeal neuralgia secondary to a structural abnormality (vascular anomaly or tumor) or infection should be considered and evaluated for. 3. Occipital neuralgia, a sharp paroxysmal that originates at the base of the skull and shoots up the head as far as the coronal suture, is a common source of chronic neck and head pain.
BIBLIOGRAPHY 1. Rozen TD: Trigeminal neuralgia and glossopharyngeal neuralgia, Neurol Clin 22(1):185-206, 2004. 2. Sampson JH, Grossi PM, et al: Microvascular decompression for glossopharyngeal neuralgia: long-term effectiveness and complication avoidance, Neurosurgery 54(4):884-890, 2004. 3. Elias J, Kuniyoshi R, et al: Glossopharyngeal neuralgia associated with cardiac syncope, Arq Bras Cardiol 78(5):510-519, 2002. 4. Mortellaro C, Biancucci P, et al: Eagle’s syndrome: importance of a correct diagnosis and adequate surgical treatment, J Craniofac Surg 13(6):755-758, 2002. 5. Ashkenasi A, Levin M: Three common neuralgias: how to manage trigeminal, occipital and postherpetic pain, Postgrad Med 116(3):16-18, 21-24, 31-32, 2004.
GLOSSOPHARYNGEAL AND OTHER FACIAL NEURALGIAS Robert A. Duarte, MD, and Charles E. Argoff, MD 1. What is the clinical presentation of glossopharyngeal neuralgia? The pain of glossopharyngeal neuralgia is similar in many ways to that seen in trigeminal neuralgia, but it has a different distribution. It presents with paroxysms of lancinating pain that involve the glossopharyngeal and vagus nerves. Pain is felt around the jaw, throat, ears, larynx, and/ or base of the tongue. The pain is typically unilateral and lasts for about 1 minute. Multiple attacks can occur throughout a day and may even awaken the patient out of a sound sleep. The usual triggers are talking and chewing. Odynophagia is a specific trigger in glossopharyngeal neuralgia that is rarely, if ever, seen in trigeminal neuralgia. When glossopharyngeal neuralgia is not caused by an underlying tumor, spontaneous remissions often occur. 2. Define odynophagia. Odynophagia is pain upon swallowing. 3. How common is glossopharyngeal neuralgia? Glossopharyngeal neuralgia is an uncommon disorder with a prevalence of only about 1/100 that of trigeminal neuralgia. Symptoms of the primary disorder usually begin when the patient is in his or her 60s. 4. A patient experiences neck pain upon swallowing and a sudden loss of consciousness. What is a likely explanation? Swallow syncope is a syndrome of unclear mechanism that occurs in patients with glossopharyngeal neuralgia. It is thought that a barrage of impulses from the glossopharyngeal nerve, through the tractus solitarius, to the dorsal motor nucleus of the vagus nerve produces bradycardia or brief asystole. It is most commonly seen in patients with tumors of the neck and, in previously operated-on patients, usually represents tumor recurrence. 5. What is the difference between idiopathic glossopharyngeal neuralgia and secondary glossopharyngeal neuralgia? The difference between idiopathic and secondary glossopharyngeal neuralgia is a clearly identified underlying cause. Clinically, idiopathic glossopharyngeal neuralgia rarely, if ever, shows objective sensory impairment on physical examination. If there is sensory loss, a causative lesion (oropharyngeal tumor, peritonsillar infection, or vascular compression) must be sought. Tumors at the base of the skull, particularly around the jugular foramen, may also cause pain radiating to the throat, so careful imaging of the oropharynx and the base of the skull must be undertaken. 6. What are the recommended treatments for idiopathic glossopharyngeal neuralgia? Anticonvulsant or Baclofen therapy is the recommended treatment for idiopathic glossopharyngeal neuralgia, and agents such as carbamazepine, gabapentin, and phenytoin are preferred. In refractory cases, intracranial sectioning of the glossopharyngeal nerve and the upper rootlets of the vagus nerve and microvascular decompression of the glossopharyngeal nerve have been performed with some success.
116
CHAPTER 19 GLOSSOPHARYNGEAL AND OTHER FACIAL NEURALGIAS 117 7. A patient has severe ear pain followed by ipsilateral facial weakness. What extremely important objective sign should be investigated? In any case of facial palsy, the search for a causative lesion is imperative. When there is severe ear and face pain, the lesion to be sought is a herpetic eruption. The vesicles most commonly affect the external auditory canal and ear but may be seen on the ear, palate, or pharynx. The syndrome of facial nerve palsy with a herpetic eruption is called Ramsay Hunt syndrome, a particularly painful neuralgia caused by zoster in the geniculate ganglion. 8. What is the most common presentation of acute herpes zoster in the face? Ophthalmic zoster (a V1 distribution of the trigeminal nerve) is the most common and most troublesome. The forehead and upper lid are involved with a vesicular eruption. Pain may precede the eruption for as long as 3 to 4 days. Viral vesicles may involve the eye itself. Aside from the general symptomatic treatment for zoster, the eye must be protected from secondary infection. The complication of postherpetic neuralgia is far more common in the older person than in the young and seems to be more common when the zoster rash affects the V1 distribution. 9. Describe the clinical features of occipital neuralgia. In occipital neuralgia, a sharp pain originates at the base of the skull and shoots up the back of the head. It may go as far forward as the coronal suture. It is generally unilateral and stabbing in nature. In idiopathic cases, there is no sensory loss, and some of the triggering mechanisms may be the same as those in other cranial neuralgias. However, occipital neuralgia may be a harbinger of more serious disease. Metastases to the occipital condyle can reproduce this syndrome, as can lesions at the C2 to C3 level of the spine. In these cases, local pressure will reproduce the pain, and there may be some accompanying sensory loss. Computerized tomography with overlapping cuts and bone windows can usually demonstrate the lesion. Occasionally, lesions around the foramen magnum can also produce this type of syndrome. Trauma is also a common cause; infection and chronic compression are less frequent causes. 10. What is superior laryngeal neuralgia? The superior laryngeal nerve, a branch of the vagus nerve, innervates the cricothyroid muscle of the larynx. This muscle stretches, tenses, and adducts the vocal cord. Superior laryngeal neuralgia usually appears as a postsurgical complication. There are paroxysms of unilateral submandibular pain, sometimes radiating to the eye, ear, or shoulder. This pain may be indistinguishable from glossopharyngeal neuralgia. It lasts from seconds to minutes and is usually provoked by swallowing, straining the voice, turning the head, coughing, sneezing, yawning, or blowing the nose. 11. What are the typical characteristics of Villaret’s syndrome? Villaret’s syndrome is characterized by unilateral paralysis of the ninth, tenth, eleventh, and twelfth cranial nerves, sometimes accompanied by a Horner’s syndrome on the same side. The syndrome may include glossopharyngeal neuralgia and is associated with lesions in the posterior retroparotid space. 12. What is Eagle’s syndrome? Eagle’s syndrome involves elongation of the styloid process of the temporal bone, which may cause impingement on the glossopharyngeal nerve and is thought to be one of the causes of secondary glossopharyngeal neuralgia. 13. Define sphenopalatine neuralgia. Sphenopalatine neuralgia has been given a number of names, including lower half headache, greater superficial neuralgia, Sluder’s neuralgia, and atypical facial pain. It is an uncommon form of facial neuralgia. The key clinical features include unilateral pain in the face (usually around the nasal region) lasting for days and associated with nasal congestion, otalgia, and tinnitus.
118 CHAPTER 19 GLOSSOPHARYNGEAL AND OTHER FACIAL NEURALGIAS Unlike trigeminal and glossopharyngeal neuralgia, sphenopalatine neuralgia is usually not associated with a trigger zone. Some authors believe that this is not a separate syndrome and may simply be a variation of cluster headache. Sphenopalatine ganglion blocks have been tried with minimal success, but this treatment remains controversial.
KEY POINTS 1. The typical triggers of glossopharyngeal neuralgia are talking and chewing. 2. Because idiopathic glossopharyngeal neuralgia rarely if ever is associated with objective sensory loss, when such sensory loss is present glossopharyngeal neuralgia secondary to a structural abnormality (vascular anomaly or tumor) or infection should be considered and evaluated for. 3. Occipital neuralgia, a sharp paroxysmal that originates at the base of the skull and shoots up the head as far as the coronal suture, is a common source of chronic neck and head pain.
BIBLIOGRAPHY 1. Rozen TD: Trigeminal neuralgia and glossopharyngeal neuralgia, Neurol Clin 22(1):185-206, 2004. 2. Sampson JH, Grossi PM, et al: Microvascular decompression for glossopharyngeal neuralgia: long-term effectiveness and complication avoidance, Neurosurgery 54(4):884-890, 2004. 3. Elias J, Kuniyoshi R, et al: Glossopharyngeal neuralgia associated with cardiac syncope, Arq Bras Cardiol 78(5):510-519, 2002. 4. Mortellaro C, Biancucci P, et al: Eagle’s syndrome: importance of a correct diagnosis and adequate surgical treatment, J Craniofac Surg 13(6):755-758, 2002. 5. Ashkenasi A, Levin M: Three common neuralgias: how to manage trigeminal, occipital and postherpetic pain, Postgrad Med 116(3):16-18, 21-24, 31-32, 2004.