CASE REPORT
Gossopharyngeal neuralgia wvit syncope: a case report
C.H. Hie, A.E.R Arnold, J.H. Ruiter
We present the case of a 60-year-old woman with known glossopharyngeal neuralgia who was admitted to hospital because of recurrent syncopes associated with episode of painflul sensations in the caudal region of her tongue. Rhythm observation showed prolonged asystole, which was accompanied by a loss of consciousness. The asystole was preceded by an episode of pain. We concluded that the bradyarrhythmia and syncopes where associated with the glossopharyngeal neuralgia. Because ofthe life-threatening condition, we inserted a permanent
dual-dcamber pacing device. After implantation of the pacemaker, the patient had no furte syncopes, although she still complained of episodic tongue pain. (NetdbHeartJ2002;10:150-3.) Key words: cardiac pacing, glossopharyngeal neuralgia, syncope
lossopharyngeal neuralgia is an uncommon condition. It has been estimated as being a hundred times less prevalent than trigeminal neuralgia. Occasionally, syncope is associated with craniofacial pain syndrome. There are reasons to believe that the pathophysiological mechanism, although still not fully understood, involves both the vagal and the glossopharyngeal nerves, and also the carotid sinus nerve, the associated nuclei in the lower brainstem and the sympathic nervous system. Bradyarrhythmia and a sudden drop in blood pressure finally lead to syncope. There might be some grounds for surgical therapy, after failure of pharmacological therapy, to relieve the patient from the pain, C.H. Hi.
Department of Cardiology, Onze Lieve Vrouwe, Gasthuis, le Oosterparkstraat 279, 1091 HA Amsterdam. A.E.R. Anold. J.H. Rulter. Department of Cardiology, Medical Centre Alkmaar, Wilhelminalaan 12, 1815 JD Alkmaar. Address of correspondence: C.H. Hie.
150
which is presumed to provoke the syncopes in this syndrome. In our patient, a permanent dual-chamber pacemaker has proven to be effective in preventing further episodes of syncope. Cae report A 60-year-old woman presented with a known history of episodic glossopharyngeal pain for the last eight years, which was diagnosed as glossopharyngeal neuralgia and treated with clonazepam 1 mg tdd by her neurologist. She had a past history of paroxysmal atrial fibrillation and thyroid surgery because of thyrotoxicosis. She presented with a sudden loss of consciousness preceded by a sudden pain in the caudal of her tongue and the left peritonsillar region. The duration ofthe loss of consciousness was presumed to have only been a few seconds. The patient stated that she had had previous episodes of 'light-headedness', which were always preceded by or appeared simultaneously with the characteristic pain in the aforementioned regions, but these episodes were never complicated by sudden loss of consciousness. There were no symptoms of palpitations, angina pectoris or shortness of breath. There were no concomitant symptoms, suggestive of grand-mal seizures either. Her medical treatment was clonazepam 1 mg tdd. On physical examination, no abnormalities were revealed. Electrocardiography showed sinus rhythm with normal conduction and repolarisation. Chest Xray showed no abnormalities. Exercise testing revealed no conduction abnormalities or arrhythmia and no signs of coronary insufficiency. Cervical ultrasound showed no signs of a tumour ofthe glomus caroticum but both halves of the thyroid gland were enlarged with multicystic aspect. Both the serum TSH and T4 were normal. Previous EEG recording did not reveal any disturbances, but at that time the patient was without symptoms. Computed tomography of the neck and posterior fossa was also normal. Because of multiple episodes of sinus bradycardia and SA-node arrests, sometimes lasting for eight seconds, accompanied by third degree AV block (figure 1), with symptoms of light-headedness and Netherlands Heart Journal, Volume 10, Number 3, March 2002
Glossopharyngeal neuralgia with syncope: a case report
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Figure 1. Episodes ofsinus node arrest and total AVblock lkting more than eight seconds during rythm monitoring, pooked bypain in the
tongue.
near-collapse, a permanent dual-chamber cardiac pacemaker was introduced by means of a left subclavian vein puncture. Serial invasive blood pressure recordings were performed, using the right femoral artery before and after introduction of the dual-chamber device. During an episode of glossopharyngeal pain there was a significant decrease in the heart rate which was accompanied by a fall in blood pressure (figure 2). After introduction ofthe dual-chamber pacemaker, a similar episode occurred, followed by cardiac pacing when the heart rate was less than the programmed lower rate of the pacemaker (figure 3). At follow-up six months later, no further syncopes occurred, although she continued to have episodic glossopharyngeal pain. Discussion
According to Kapoor et al. only a small percentage of the syncopes with a demonstrable aetiological moment are of neurological origin.' Syncope due to vagal involvement in the presence of glossopharyngeal neuralgia is a rare condition. In 1942, Riley described a case of glossopharyngeal neuralgia associated with syncope.2 Several cases have been reported since then in the literature. The prevalence of glossopharyngeal neuralgia among the population has been estimated to be 0.2-1.3% of cases of facial pain, which is about 100 times less than trigeminal neuralgia.3 The most common variety of
Netherlands Heart Journal, Volume 10, Number 3, March 2002
glossopharyngeal neuralgia is idiopathic. Secondary varieties may be caused by many other factors (vascular and mechanical compression, infectious processes, tumours and degenerative disorders). The rarity of syncope as concomitant symptom has been stressed by Rushton et al. They reported a group of 217 cases of glossopharyngeal neuralgia in which they found only four patients who also had syncope as concomitant symptom.4 The pathogenic mechanism ofthe syncopal attacks is not completely understood. It is likely that it must be sought in the close anatomical relationship that exists between the glossopharyngeal and the vagus nerve during their course in the medulla oblongata. Sometimes afferent nerve impulses that travel through the glossopharyngeal nerve can reach the nucleus tractus solitarii and, via collaterals, reach the dorsal nucleus of the vagus nerve. In addition it must also be mentioned that fibres of the carotid sinus nerve (Hering's nerve) join the glossopharyngeal nerve and end in the dorsal nucleus ofthe vagus nerve, creating the possibility of provoking vagal nerve stimulation by glossopharyngeal afferent stimuli. According to another concept, accessory synapses (ephapses) are formed between the glossopharyngeal and vagal fibres in the region of their ganglia or there is an abnormal communication involving the nucleus tractus solitarii and the nucleus ambiguus centrally.5 In summary the presence of an irritant stimulus and 'cross-talk' between the above circuits can result in a significant bradycardia 151
Glossopharyngeal neuralgia with syncope: a case report
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(cardioinhibitory effect) and decrease in peripheral arterial resistance (vasodepressive effect), possibly mediated by the vagus nerve and the carotid baroreceptor pathway, respectively. These two mechanisms are in the end responsible for the clinical symptoms. A drop in the systemic catecholamine levels prior to the syncope suggest a decrease of the sympathic activity, which is probably mediated by the carotid baroreceptor pathway.6'7 There are similarities with neurocardiogenic syncope, which can also be precipitated by an aspecific stimulus and finally results in a vasodepressive (mostly), cardioinhibitory or combined response. It can be said that these two conditions may share a common pathway. The sudden drop in the systemic catecholamine levels, however, as previously noted in the case of neuralgia-mediated syncope, is not compatible with the neurocardiogenic syncope. In the latter there is a consistent rise of the serum catechoamine levels just before the occurrence of syncope.8 The mechanism underlying this phenomenon is not yet clear. The management ofthe syncope mediated by glosso152
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pharyngeal neuralgia depends on several factors: how often does the syncope occur, are the bradyarrhythmias severe, can the syncope be provoked, is there a vasodepressive or cardioinhibitory response, or both, and which is predominant? If there is a strong vasodepressive response, then permanent cardiac pacing is unlikely to solve the problem. In our case, there was a combined response of cardioinhibitory and vasodepressive effects. Permanent dual-chamber cardiac pacing was successful in preventing syncopal attacks in the six months follow-up). Raefsncs 1 2
3 4
5
Kapoor W. Evaluation and outcome of patients with syncope. Medicinc 1990;69:160-75. Riley HA, Geenan WJ, Wortis H. Glossopharyngeal neuralgia initiating or associated with cardiac arrest. Trans Am Ncurol Assoc 1942;18:28-9. Chawla JC, Falconer MA. Glossopharyngeal and vagal neuralgia. BrMedJl967;3:29-31. Stevens C, Miller RH. Glossopharyngeal (vagoglossopharyngeal) neuralgia. Arch Neurol 1981;38:201-5. Barbash GL, et al. Mechanism of syncope in glossopharyngeal neuralgia. Ekctroencephalog Clin Neurophysiol 1986;63:231-5.
Netherlands Heart Journal, Volume 10, Number 3, March 2002
Glossopharyngeal neuralgia with syncope: a case report
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6
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ScherAM. Carotid and aortic regulation of arterial blood pressure. Circulation 1977;56:521-8. Dykman TR, et al. Glossopharyngeal neuralgia with syncope secondary to tumor - treatment and pathophysiology. Am JMed 1981;71:165-8.
Netherlands Heart Joumal, Volume 10, Number 3, March 2002
8
Sra JS, et al. Circulatory and catecholamine changes during headup tilt testing in neurocardiogenic (vasovagal) syncope. AmJCardiol 1994;73:33-7.
153
Gossopharyngeal neuralgia wvit syncope: a case report
C.H. Hie, A.E.R Arnold, J.H. Ruiter
We present the case of a 60-year-old woman with known glossopharyngeal neuralgia who was admitted to hospital because of recurrent syncopes associated with episode of painflul sensations in the caudal region of her tongue. Rhythm observation showed prolonged asystole, which was accompanied by a loss of consciousness. The asystole was preceded by an episode of pain. We concluded that the bradyarrhythmia and syncopes where associated with the glossopharyngeal neuralgia. Because ofthe life-threatening condition, we inserted a permanent
dual-dcamber pacing device. After implantation of the pacemaker, the patient had no furte syncopes, although she still complained of episodic tongue pain. (NetdbHeartJ2002;10:150-3.) Key words: cardiac pacing, glossopharyngeal neuralgia, syncope
lossopharyngeal neuralgia is an uncommon condition. It has been estimated as being a hundred times less prevalent than trigeminal neuralgia. Occasionally, syncope is associated with craniofacial pain syndrome. There are reasons to believe that the pathophysiological mechanism, although still not fully understood, involves both the vagal and the glossopharyngeal nerves, and also the carotid sinus nerve, the associated nuclei in the lower brainstem and the sympathic nervous system. Bradyarrhythmia and a sudden drop in blood pressure finally lead to syncope. There might be some grounds for surgical therapy, after failure of pharmacological therapy, to relieve the patient from the pain, C.H. Hi.
Department of Cardiology, Onze Lieve Vrouwe, Gasthuis, le Oosterparkstraat 279, 1091 HA Amsterdam. A.E.R. Anold. J.H. Rulter. Department of Cardiology, Medical Centre Alkmaar, Wilhelminalaan 12, 1815 JD Alkmaar. Address of correspondence: C.H. Hie.
150
which is presumed to provoke the syncopes in this syndrome. In our patient, a permanent dual-chamber pacemaker has proven to be effective in preventing further episodes of syncope. Cae report A 60-year-old woman presented with a known history of episodic glossopharyngeal pain for the last eight years, which was diagnosed as glossopharyngeal neuralgia and treated with clonazepam 1 mg tdd by her neurologist. She had a past history of paroxysmal atrial fibrillation and thyroid surgery because of thyrotoxicosis. She presented with a sudden loss of consciousness preceded by a sudden pain in the caudal of her tongue and the left peritonsillar region. The duration ofthe loss of consciousness was presumed to have only been a few seconds. The patient stated that she had had previous episodes of 'light-headedness', which were always preceded by or appeared simultaneously with the characteristic pain in the aforementioned regions, but these episodes were never complicated by sudden loss of consciousness. There were no symptoms of palpitations, angina pectoris or shortness of breath. There were no concomitant symptoms, suggestive of grand-mal seizures either. Her medical treatment was clonazepam 1 mg tdd. On physical examination, no abnormalities were revealed. Electrocardiography showed sinus rhythm with normal conduction and repolarisation. Chest Xray showed no abnormalities. Exercise testing revealed no conduction abnormalities or arrhythmia and no signs of coronary insufficiency. Cervical ultrasound showed no signs of a tumour ofthe glomus caroticum but both halves of the thyroid gland were enlarged with multicystic aspect. Both the serum TSH and T4 were normal. Previous EEG recording did not reveal any disturbances, but at that time the patient was without symptoms. Computed tomography of the neck and posterior fossa was also normal. Because of multiple episodes of sinus bradycardia and SA-node arrests, sometimes lasting for eight seconds, accompanied by third degree AV block (figure 1), with symptoms of light-headedness and Netherlands Heart Journal, Volume 10, Number 3, March 2002
Glossopharyngeal neuralgia with syncope: a case report
I.3
I2 I
11:
.4-
a: 1.}
iI
.e
NW
i
:1 1:
Figure 1. Episodes ofsinus node arrest and total AVblock lkting more than eight seconds during rythm monitoring, pooked bypain in the
tongue.
near-collapse, a permanent dual-chamber cardiac pacemaker was introduced by means of a left subclavian vein puncture. Serial invasive blood pressure recordings were performed, using the right femoral artery before and after introduction of the dual-chamber device. During an episode of glossopharyngeal pain there was a significant decrease in the heart rate which was accompanied by a fall in blood pressure (figure 2). After introduction ofthe dual-chamber pacemaker, a similar episode occurred, followed by cardiac pacing when the heart rate was less than the programmed lower rate of the pacemaker (figure 3). At follow-up six months later, no further syncopes occurred, although she continued to have episodic glossopharyngeal pain. Discussion
According to Kapoor et al. only a small percentage of the syncopes with a demonstrable aetiological moment are of neurological origin.' Syncope due to vagal involvement in the presence of glossopharyngeal neuralgia is a rare condition. In 1942, Riley described a case of glossopharyngeal neuralgia associated with syncope.2 Several cases have been reported since then in the literature. The prevalence of glossopharyngeal neuralgia among the population has been estimated to be 0.2-1.3% of cases of facial pain, which is about 100 times less than trigeminal neuralgia.3 The most common variety of
Netherlands Heart Journal, Volume 10, Number 3, March 2002
glossopharyngeal neuralgia is idiopathic. Secondary varieties may be caused by many other factors (vascular and mechanical compression, infectious processes, tumours and degenerative disorders). The rarity of syncope as concomitant symptom has been stressed by Rushton et al. They reported a group of 217 cases of glossopharyngeal neuralgia in which they found only four patients who also had syncope as concomitant symptom.4 The pathogenic mechanism ofthe syncopal attacks is not completely understood. It is likely that it must be sought in the close anatomical relationship that exists between the glossopharyngeal and the vagus nerve during their course in the medulla oblongata. Sometimes afferent nerve impulses that travel through the glossopharyngeal nerve can reach the nucleus tractus solitarii and, via collaterals, reach the dorsal nucleus of the vagus nerve. In addition it must also be mentioned that fibres of the carotid sinus nerve (Hering's nerve) join the glossopharyngeal nerve and end in the dorsal nucleus ofthe vagus nerve, creating the possibility of provoking vagal nerve stimulation by glossopharyngeal afferent stimuli. According to another concept, accessory synapses (ephapses) are formed between the glossopharyngeal and vagal fibres in the region of their ganglia or there is an abnormal communication involving the nucleus tractus solitarii and the nucleus ambiguus centrally.5 In summary the presence of an irritant stimulus and 'cross-talk' between the above circuits can result in a significant bradycardia 151
Glossopharyngeal neuralgia with syncope: a case report
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Fi,gure 2. A fall in the artemial blood pressure and sinus bradycardia after the onset
(cardioinhibitory effect) and decrease in peripheral arterial resistance (vasodepressive effect), possibly mediated by the vagus nerve and the carotid baroreceptor pathway, respectively. These two mechanisms are in the end responsible for the clinical symptoms. A drop in the systemic catecholamine levels prior to the syncope suggest a decrease of the sympathic activity, which is probably mediated by the carotid baroreceptor pathway.6'7 There are similarities with neurocardiogenic syncope, which can also be precipitated by an aspecific stimulus and finally results in a vasodepressive (mostly), cardioinhibitory or combined response. It can be said that these two conditions may share a common pathway. The sudden drop in the systemic catecholamine levels, however, as previously noted in the case of neuralgia-mediated syncope, is not compatible with the neurocardiogenic syncope. In the latter there is a consistent rise of the serum catechoamine levels just before the occurrence of syncope.8 The mechanism underlying this phenomenon is not yet clear. The management ofthe syncope mediated by glosso152
~ ~~~~~~
:
CA^G ...................: .:.
ofylossophaiyngealpazn.
pharyngeal neuralgia depends on several factors: how often does the syncope occur, are the bradyarrhythmias severe, can the syncope be provoked, is there a vasodepressive or cardioinhibitory response, or both, and which is predominant? If there is a strong vasodepressive response, then permanent cardiac pacing is unlikely to solve the problem. In our case, there was a combined response of cardioinhibitory and vasodepressive effects. Permanent dual-chamber cardiac pacing was successful in preventing syncopal attacks in the six months follow-up). Raefsncs 1 2
3 4
5
Kapoor W. Evaluation and outcome of patients with syncope. Medicinc 1990;69:160-75. Riley HA, Geenan WJ, Wortis H. Glossopharyngeal neuralgia initiating or associated with cardiac arrest. Trans Am Ncurol Assoc 1942;18:28-9. Chawla JC, Falconer MA. Glossopharyngeal and vagal neuralgia. BrMedJl967;3:29-31. Stevens C, Miller RH. Glossopharyngeal (vagoglossopharyngeal) neuralgia. Arch Neurol 1981;38:201-5. Barbash GL, et al. Mechanism of syncope in glossopharyngeal neuralgia. Ekctroencephalog Clin Neurophysiol 1986;63:231-5.
Netherlands Heart Journal, Volume 10, Number 3, March 2002
Glossopharyngeal neuralgia with syncope: a case report
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Fig0ure 3. Sinus bradyrardia during implantation of a DDD pacemaker)fo11owed byDDD paeing. Patient was eomplainin,, ofpain in the tongue jxtbefore onset of the bradya nvmia.
6
7
ScherAM. Carotid and aortic regulation of arterial blood pressure. Circulation 1977;56:521-8. Dykman TR, et al. Glossopharyngeal neuralgia with syncope secondary to tumor - treatment and pathophysiology. Am JMed 1981;71:165-8.
Netherlands Heart Joumal, Volume 10, Number 3, March 2002
8
Sra JS, et al. Circulatory and catecholamine changes during headup tilt testing in neurocardiogenic (vasovagal) syncope. AmJCardiol 1994;73:33-7.
153
![[Glossopharyngeal neuralgia with syncope].](/assets/img/hold.png)
