Glossopharyngeal neuralgia

CASE REPORT

Glossopharyngeal Neuralgia S. Douglas Greeson, MD Christopher H. Linden, MD Hershey, Pennsylvania

An uncommon condition known as glossopharyngeal neuralgia is manifested by paroxysms of neck, throat, and ear pain which may be accompanied by cardiac dysrhythmias. A 58-year-old woman had episodes of pain associated with Mobitz II atrioventricular block and complete heart block with syncope. Temporary and then permanent cardiac pacing were required. The patient's pain was relieved by carbamazepine, obviating the need for intracranial nerve root section, Greeson SD, Linden CH: Glossopharyngeal neuralgia. Ann EFnerg

Med 10:656-658, December 1981. neuralgia, glossopharyngeal INTRODUCTION Neck pain and cardiac dysrhythmias are problems commonly seen in the emergency department. In glossopharyngeal neuralgia, however, the two may coexist in an unusual association. I The following case illustrates the importance of ascertaining this diagnosis and initiating appropriate therapy.

CASE REPORT A 58-year-old woman presented with complaints of neck pain and dizziness. Two weeks earlier she had noted the abrupt onset of pain in the area of the left mandibular angle. There was radiation to the tragus, external auditory canal, temporal region, and occasionally to the upper and lower gums. She described the pain as a constant dull ache with superimposed episodes of sharp pain lasting several seconds to a few minutes. Although partial relief was initially obtained by clenching the teeth, and administering Tylenol ®(two tablets every four hours) and then Darvocet e (two tablets every three hours), the paroxysms of pain continued to increase in frequency to approximately 30 episodes a day. On the morning of admission, spells of lightheadedness coincident with the shooting pains were noted. There was no history of trauma or visual symptoms. The patient had had rheumatic fever with Sydenham's chorea when she was a child, and her aortic valve was replaced one year prior to this admission. Medications on admission included digoxin 0.25 mg daily and warfarin 4.0 mg daily. Physical examination revealed a distraught, anxious, and occasionally tearful middle-aged woman. Blood pressure was 130/70 mm Hg. Pulse was irregular and ranged from 40 to 70 beats per minute. The patient was placed on a cardiac monitor which showed high-grade Mobitz II atrioventricular block with series of 3-4 nonconducted P-waves without ventricular escape (Figure 1). The patient was edentulous; results of examination of the ears, nose, mouth, and throat were otherwise normal. Palpation of the left side of the neck resulted in complete heart block without ventricular escape for a period of approximately eight seconds, during which the patient temporarily lost consciousness. The Mobitz II r h y t h m then resumed. Further neck palpation was deferred. There was no tenderness or nodularity of the extracranial arteries. From the Emergency Medicine Division, The Milton S. Hershey Medical Center, The Pennsylvania State University College of Medicine, Hershey Pennsylvania. Address for reprints: S. Douglas Greeson, MD, Emergency Medicine Division, The Milton S. Hershey Medical Center, The PennsylvaniaState University, Hershey, Pennsylvania 17033.

10:12 (December) 1981

Ann Emerg Med

656/71

Fig. 1. Cardiac monitor showed high-grade Mobitz H atrioventricular block with series of 3-4 nonconducted P-waves without ventricular escape.

Fig. 2, ECG confirmed Mobitz H block with rate of 34. The lungs were clear. Auscultation of the h e a r t r e v e a l e d d i s t a n t heart sounds, normal $1, artificial $2, and a 2/6 systolic ejection m u r m u r loudest at the lower left sternal border. The murmur was also heard well in the right second interspace, but it did not radiate. A right femoral bruit was noted. Neurologic testing showed normal cranial nerve, motor, sensory, c e r e b e l l a r , and reflex results. The electrocardiogram confirmed the Mobitz II block with a rate of 34 (Figure 2). A 0.5-mg bolus of atropine resulted in a sinus r h y t h m with first degree block at a rate of 72 (Figure 3). A second atropine bolus was given 10 m i n u t e s l a t e r when the block again developed. The patient was a d m i t t e d w i t h a d i a g n o s i s of h e a r t block secondary to d i g i t a l i s

72/657

toxicity and possible glossopharyngeal neuralgia. Laboratory testing revealed normal electrolytes and liver and renal screens. The Westergren sedimentation rate was slightly elevated at 76. The Owren p r o t h r o m b i n time was 12.5% (normal 70% to 100%). Digoxin was withheld and the p a t i e n t was given atropine as needed. Tylenol ~ with codeine (two tablets every three to four hours) and, later, intramuscul a r D~merol * (50 to 100 mg every four hours) were given without substantial relief of the neck pain. No trigger point for the pain was found on subsequent examinations. At 36 hours, the admission digoxin level was reported as 1.8 ng/ml (therapeutic range 1.5 to 2.0 ng/ml), but A-V block persisted. A temporary transvenous pacemaker was inserted. For

Ann Emerg Med

another two days the pacemaker was shut off intermittently and Mobitz II block recurred periodically in association with paroxysms of neck pain. A p e r m a n e n t p a c e m a k e r was then inserted. Cervical spine and skull r a d i o g r a p h s with b a s i l a r foramina views and computerized tomographic scan of the cranium failed to reveal a cause for the neck pain. The patient was given Tegretol ~ 100 mg twice a day with gradual improvement. By the ninth day of treatment, she was asymptomatic. After one year of outpatient follow-up, she continued to take her medication and had no recurrence of pain.

DISCUSSION G l o s s o p h a r y n g e a l n e u r a l g i a is an uncommon cause of severe unil a t e r a l oropharyngeal, cervical, and

10:12 (December) 1981

ear pain. It occurs p r e d o m i n a n t l y in men over 50 years old, w i t h an average age of 62 in Chawla's series. 2 The condition m u s t be differentiated from t r i g e m i n a l tic douloureux, which is 40 times more frequent. ~ T h e p a i n of g l o s s o p h a r y n g e a l n e u r a l g i a is d e s c r i b e d "as p a r o x y s mal, lancinating, and excruciating. It is u s u a l l y perceived as o r i g i n a t i n g in the p h a r y n x and r a d i a t i n g to the ear, but m a y r a d i a t e from the e a r to the t h r o a t or neck or be confined to the ear. 3 The p a i n lasts from seconds to 5 m i n u t e s and is often p r e c i p i t a t e d by chewing, coughing, y a w n i n g , t a l k ing, and especially swallowing. 4 ~Trigger zones" are r a r e l y found in t h i s c o n d i t i o n , in c o n t r a s t to t r i g e m i n a l n e u r a l g i a . 2 E p i s o d e s typically accelerate in frequency over a few weeks, t h e n a b a t e spontaneously w i t h a n u n p r e d i c t a b l e p a t t e r n of recurrence.4 Weisenburg5 first described the syndrome in 1910 in a p a t i e n t with a cerebellopontine angle tumor. It h a s since b e e n found to occur w i t h int r a c r a n i a l v a s c u l a r anomalies, multiple sclerosis, Paget's disease, carcinomatosis, and a n a t o m i c v a r i a t i o n s of the second b r a n c h i a l arch, such as an abnormally long styloid process. 4 Sicard and Robineau, 6 in 1920, were the first to report the p a i n syndrome in the absence of identifiable pathology, a n d m o s t of t h e cases in t h e l i t e r a t u r e to date are ~idiopathic." A t t a c k s of p a i n are often associated, as in our patient, with s t r i k i n g c a r d i o v a s c u l a r m a n i f e s t a t i o n s . Syncopal episodes and convulsions seco n d a r y to c e r e b r a l h y p o p e r f u s i o n h a v e b e e n r e p o r t e d , 7 as h a v e h y potensi0n and cardiac arrest. 7 D y s r h y t h m i a s r a n g e from sinus brad y c a r d i a a n d sinus p a u s e s to wandering atrial pacemaker, varying d e g r e e s of A - V b l o c k , a n d a s y s tole. 1,3,4,7,s S y n c o p a l e p i s o d e s m a y s i m u l a t e those due to carotid sinus s e n s i t i v i t y , b u t s y m p t o m s a r e not typically reproduced by carotid sinus massage. 8 The Cardiovascular effects do not r e p r e s e n t a nonspecific v a g a l response to pain, b u t a r e t r i g g e r e d by a reflex arc from the glossopharyngeal to the vagus nerve. It has been p o s t u l a t e d t h a t intense afferent impulses m a y stimulate the dorsal motor nucleus of the v a g u s e i t h e r t h r o u g h c e n t r a l coll a t e r a l p a t h w a y s or by w a y of a n "artificial synapse," an i r r i t a t i v e lesion, along the p e r i p h e r a l course of the ninth nerve where it t r a v e l s with the carotid sinus (Hering's) nerve. 3'9

10:12 (December) 1981

Fig. 3. Sinus rhythm with first degree block at a rate of 72 followed administration of atropine. I m p u l s e s conducted t h r o u g h a n inmonitoring. The cardiovascular manj u r e d or ischemic region have been ifestations m a y necessitate pacemakshown to excite n e i g h b o r i n g inactive er insertion. fibers 1° and m i g h t establish the reflex arc. The a u r i c u l a r b r a n c h of the The authors gratefully acknowledge the assistance and time of Donna J. Rentzel vagus m a y itself contribute some of in the preparation and completion of this the afferent impulses. Atropine paper. u s u a l l y produces complete t e m p o r a r y r e v e r s a l of t h e b r a d y a r r h y t h m i a s , s b u t s y m p a t h o m i m e t i c agents such as REFERENCES i s o p r o t e r e n o l m a y not. 11 Cocainiza1. Ray BS, Stewart HJ: Glossopharynt i o n of t h e p o s t e r i o r p h a r y n x h a s geal neuralgia: A cause of cardiac arrest. t r a n s i e n t l y decreased p a i n in some Am Heart'J 35:458~ 1948. cases, b u t does n o t a t t e n u a t e t h e 2. Chawta JC, Falconer MS: Glossophavagal effects. ~2 ryngeal and vagal neuralgia. Br Med J A t e m p o r a r y t r a n s v e n o u s pace3:529, 1967. m a k e r m a y be needed, as in our case, 3. Svien HJ: Partial glossopharyngeal pending permanent pacemaker neuralgia associated with syncope. J p l a c e m e n t or surgical i n t e r r u p t i o n of Neurosurg 14:452, 1957. t h e reflex arc. 11 The fact t h a t t h e 4. Jamshidi A, Masroor MA: Glossophacondition occurs in a population ryngeal neuralgia with cardiac syncope: a l r e a d y prone to u n d e r l y i n g cardiac Treatment with a p e r m a n e n t cardiac p a t h o l o g y c a n c o m p l i c a t e t h e dipacemaker and carbamazepine. Arch Inagnostic picture. Our patient had tern Med 136:843, 1976. g l o s s o p h a r y n g e a l n e u r a l g i a in t h e 5. Weisenburg TH: Cerebellopontine s e t t i n g of v a l v u l a r , p e r h a p s r h e u tumor diagnosed for six years as tic matic, h e a r t disease, and possibly cadouloureux: The symptoms of irritation of the ninth and twelfth cranial nerves. rotid sinus h y p e r s e n s i t i v i t y as well. JAMA 54:1600, 1910. P h a r m a c o l o g i c m a n a g e m e n t of t h e p a i n with D i l a n t i n ~ and Tegre6. Sicard R, Robineau M, cited by Harris W: P e r s i s t e n t pain in lesions of the tol ~ has been t r i e d with l i m i t e d sucperipheral and central nervous system. cess; this m o d a l i t y m a y be useful in Br Med J 2:896, 1921. e l d e r l y or d e b i l i t a t e d p a t i e n t s who 7. Garretson HD, Elvidge AR: Glossophaa r e poor surgical risks. 4,s However, ryngeal neuralgia with asystole and seii n t r a c r a n i a l s e c t i o n of t h e n i n t h zures. Arch Neurol 8:26, 1963. nerve and the upper two v a g a l root8. Kong Y: Glossopharyngeal neuralgia lets r e m a i n s the definitive t r e a t m e n t associated with bradycardia, syncope, and for protracted and i n t r a c t a b l e pain. 2 seizures. Circulation 30:109, 1964. The procedure also abolishes the car9. Karnosh LJ, Gardner WJ, Stowell A: diovascular manifestations. It is perGlossopharyngeal neuralgia: Physiologic m a n e n t l y effective, a n d no significonsiderations of the role of the ninth and c a n t c o m p l i c a t i o n s h a v e b e e n retenth cranial nerves. Transactions of ported.l,s, 11 American Neurological Association 73: 205, 1947. CONCLUSION 10. Kjellin K: Glossopharyngeal neuralGlossopharyngeal neuralgia, gia associated with cardiac arrest and a l t h o u g h rare, should be considered hypersecretion from the ipsilateral paroti n t h e d i f f e r e n t i a l d i a g n o s i s of id gland. Neurology 9:527, 1959. paroxysmal neck, throat, and ear 11. Khero BA, Mullins CB: Cardiac synpain. It is c l i n i c a l l y i m p o r t a n t not cope due to glossopharyngeal neuralgia: only as a cause of prolonged and seTreatment with a transvenous pacemakvere discomfort, b u t also because of er. Arch Intern Med 128:806, 1971. its association with a v a r i e t y of car12. Riley HA, German WJ: Glossophadiac d y s r h y t h m i a s , some p o t e n t i a l l y ryngeal neuralgia initiating or associated fatal. P a t i e n t s in whom the diagnosis with cardiac arrest. Transactions of American Neurological Association 68:28, 1942. is s u s p e c t e d m a y r e q u i r e c a r d i a c

Ann Emerg Med

658173