Cardiac syncope induced by glossopharyngeal "neuralgia": a rare presentation

Abstracts

The first description of severe pain in the distribution of the glossopharyngeal nerve is credited to Weisenberg, in 1910¹, in a patient with cerebellopontine angle tumor. However, it was Harris, in 1926², who coined the term glossopharyngeal neuralgia to describe this rare condition characterized by paroxysms of excruciating pain located laterally at the back of the tongue, soft palate, throat, and lateral and posterior pharynx, radiating to the ear. Swallowing, coughing, yawning or chewing may trigger pain, which usually lasts from seconds to minutes. The association between glossopharyngeal neuralgia and syncope is very rare, being identified by brief episodes of bradycardia, asystole, and hypotension. Such an association, with this same pathophysiology, was first described by Riley et al³ in 1942.

A primeira descrição de dor severa no trajeto do nervo glossofaríngeo foi realizada por Weisenberg, em 1910¹, em um paciente com tumor do ângulo ponto cerebelar. Entretanto, coube a Harris, em 1926², nomear como nevralgia do nervo glossofaríngeo esse raro quadro clínico, caracterizado por paroxismos de dor intensa, unilaterais, na região posterior da língua, no palato mole, na garganta e na região lateral e posterior da faringe, irradiando para o ouvido. A dor pode ser desencadeada por deglutição, tosse, bocejo ou mastigação e normalmente dura de segundos a minutos. A associação de nevralgia do glossofaríngeo e síncope é muito rara e se deve a breves períodos de bradicardia, assistolia ou hipotensão, sendo a primeira descrição dessa associação, com essa fisiopatologia, realizada por Riley e cols., em 1942³.

Síncope do Glossofaríngeo

CASE REPORT

Cardiac syncope induced by glossopharyngeal “neuralgia”: a rare presentation

Helio Korkes; Eduardo Mesquita de Oliveira; Luigi Brollo; Denise Tessariol Hachul; José Carlos da Silva Andrade; Mario Fernando Prieto Peres; Victor Schubsky

Hospital Israelita Albert Einstein, São Paulo, SP, Brazil

^{Mailing Address}

ABSTRACT

The first description of severe pain in the distribution of the glossopharyngeal nerve is credited to Weisenberg, in 1910¹, in a patient with cerebellopontine angle tumor. However, it was Harris, in 1926², who coined the term glossopharyngeal neuralgia to describe this rare condition characterized by paroxysms of excruciating pain located laterally at the back of the tongue, soft palate, throat, and lateral and posterior pharynx, radiating to the ear. Swallowing, coughing, yawning or chewing may trigger pain, which usually lasts from seconds to minutes.

The association between glossopharyngeal neuralgia and syncope is very rare, being identified by brief episodes of bradycardia, asystole, and hypotension. Such an association, with this same pathophysiology, was first described by Riley et al³ in 1942.

Key word: Glossopharyngeal syncope.

Patient with fainting spells related to liquid intake without pain on swallowing over a period of several months. He underwent loop event monitoring, which demonstrated prolonged sinus pause with asystole during swallowing. Syncopal episodes were therefore justified by low cerebral blood flow associated with glossopharyngeal nerve stimulation, even in the absence of pain that characterizes this condition. In cases of glossopharyngeal "neuralgia" without severe pain, permanent cardiac pacing is suggested as early treatment. To our knowledge, this is the first such case reported in Brazilian literature.

Case Report

A 71-year-old white male patient complaining of sporadic syncopal episodes lasting for a few seconds upon drinking liquids. When questioned, he reported mild, ill-characterized "spasms" in the throat on these occasions.

In one of these episodes, he fell and broke a lumbar vertebra. The patient was taking antiparkinsonian drugs regularly.

• Physical examination: BP – 140 x 80 mmHg; HR – 72 bpm; Heart – RRR, S1 S2; Lungs – clear; Pulse – symmetrical. NS unremarkable.

• Supplementary examination: ECG – Left anterior fascicular block (LAFB); Gastrointestinal endoscopy - distal esophagitis and erosive gastritis; Videofluoroscopic swallowing studies – stasis of residual food in the vallecular region.

• Loop event monitoring – sinus arrest and asystole lasting 4.4 seconds, associated with reporting of presyncope during lunch (

  Fig 1)

Following the diagnosis of swallow-induced syncope with cardiac asystole, the patient underwent permanent pacemaker implantation, and since that time has remained free of syncopal episodes (thirty-five months of follow-up).

Discussion

Glossopharyngeal neuralgia (GPN) is a rare form of pain (0.2 to 1.3% of the cases of facial pain)⁴ that occurs approximately one hundred times less frequently than trigeminal neuralgia⁵.

Regarding its etiology, although most cases are idiopathic, it may be secondary to intracranial vascular compression, cerebellopontine angle tumors, laryngeal and nasopharyngeal tumors, trauma, and carotid puncture, among other causes^6-9.

The disease is more common in patients older than 50 years of age, but there is no consensus regarding which sex is most affected^10,11. Onset is sudden and is usually characterized by severe, unilateral, paroxysmal pain along the glossopharyngeal nerve course.

The most common pain-triggering factor is swallowing, especially liquids, as in the case of this report, in addition to chewing, coughing, and speaking¹². The association between glossopharyngeal neuralgia and syncope is even rarer, and was first reported by Harris, in 1921².

In a review of 217 GPN patients conducted at the Mayo Clinic in 1981, only four were found to have associated syncope. This association, however, was confirmed in only one patient, whose first symptom was pain¹⁰.

Although there has been considerable speculation in regard to the syncope mechanism, the most accepted theory was that proposed by Gardner, in 1963¹³. According to Gardner's theory, synapses formed between glossopharyngeal and vagus nerves, in their ganglia, are activated by a sensory irritation phenomenon resulting in lower heart rate, as well as vascular peripheral resistance, possibly mediated, respectively, by the vagus nerve and carotid baroreceptors (Fig. 2).

It has been suggested that only intense neuralgic pain could activate the glossopharyngeal-vagal reflex arc causing bradycardia, hypotension and syncope, and that there is a latent period between neuralgia onset and syncope in these patients¹⁴.

Based on this theory, considered the most plausible, it is difficult to explain the syncope experienced by the patient herein reported, as it is also difficult to explain the sole report on painless glossopharyngeal neuralgia published in literature¹⁵. Nevertheless, other authors agree that this may be possible¹¹.

The use of a loop event recorder should be emphasized because this new diagnostic tool is more sensitive and practical than standard Holter ECG in these cases, since it allows the patient to be monitored for several consecutive days, thus improving the likelihood of identifying these usually sporadic and brief changes.

It is noteworthy that no reports were found in literature describing the use of this method (cardiac event recording) in the diagnosis of glossopharyngeal or swallow syncope.

As far as treatment is concerned, the medical literature supports the use of carbamazepine in the management of idiopathic neuralgia^5,10,14. Temporary pacemaker implantation to treat the reflex cardiac syncope until therapeutic levels of carbamazepine are reached was first described by Khero in 1971¹⁶.

Regarding permanent pacemaker implantation, the available literature is quite controversial¹². Neither the pharmacological approach to idiopathic neuralgia nor the surgical approach to other types requires pacemaker implantation because, by controlling the pain, they also control or abolish syncopal episodes.

Swallow syncope should be considered in the differential diagnosis of these patients. This condition is caused by vasovagal reflex and has a similar presentation. It is often accompanied by arterial hypotension, which may even precede the chronotropic effect and be precipitaded by esophageal distension or spasms (as uncharacteristically referred by the patient)¹⁷. However, in this also rare etiology of neurocardiogenic syncope patients often have esophageal abnormalities, such as diverticula and, occasionally, major changes in heart structure or rhythm¹⁸ but without pain or sensory changes upon swallowing.

In the present report, owing to the lack of pain associated with glossopharyngeal neuralgia and the severity of syncopal episodes (in one of the them the patient sustained vertebral fracture and was disabled for months), permanent pacemaker implantation was the treatment of choice, even without previous pharmacological therapy. The same approach was used in the sole report of painless glossopharyngeal nerve syncope published in literature¹⁵, and it is the formal indication for swallow syncope.

Thirty-five months after pacemaker implantation, the patient remains free of syncopal events.

References

Manuscript received September 20, 2005; revised manuscript November 29, 2005; accepted on December 8, 2005.

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Publication Dates

• Publication in this collection
  06 Mar 2007
• Date of issue
  Nov 2006

History

• Accepted
  08 Dec 2005
• Reviewed
  29 Nov 2005
• Received
  20 Sept 2005