Glossopharyngeal Neuralgia,
Asystole, and Seizures
Jack N. Alpert, MD; Charles A. Armbrust, MD; Mahmood Akhavi, MD;
Eleftherios S. Stamatiou, MD; James M. Killian, MD; Michael De Shazo, MD

\s=b\

Glossopharyngeal neuralgia,

asys-

tole, and seizures occurred in a patient
with an internal carotid occlusion and
external carotid stenosis. Swallowing was
the triggering mechanism for these
events. Mechanical stimulation of the
pharynx failed to reproduce the symptoms. An ischemic injury to the glossopharyngeal nerve in the region of the
jugular foramen that resulted in an artificial synapse is the proposed etiology.

(Arch Neurol 34:233-235, 1977)

Glossopharyngeal neuralgiapain

is an
unusual form of tic douloureux.
Manifestations other than
in¬
clude cardiac arrhythmias, syncope,
seizures, and parotid hypersécrétion.1
Cardiac arrest has been reported.2"10
Usually, there is no known cause. Rare
documented etiologies include cerebellopontine angle vascular anomalies
and tumors,111'-' vertebral artery dis¬
ease," '4 extracranial tumors arising
in carotid artery, pharynx, larynx and
tonsil,1'1" arachnoiditis,17' and ossifica¬
tion of the stylohyoid ligament.17 This
is the first report of extracranial
vascular disease, internal carotid oc¬
clusion, and external carotid stenosis
as one probable cause. An artificial
synapse secondary to an ischemie
injury of the glossopharyngeal nerve
near the jugular foramen is the

suspected etiology.

REPORT OF A CASE
A 68-year-old woman was admitted to St
Luke's Hospital on Sept 15, 1974, because
of sudden loss of vision in the left eye that

Accepted for publication Dec 13, 1976.
From the Department of Neurology (Drs
Alpert, Akhavi, Killian, and De Shazo) and
Internal Medicine (Drs Armbrust and Stamatiou), St Luke's Episcopal Hospital, Texas
Medical Center, Houston.

Reprint requests to 224 Hermann Professional
Bldg, Houston, TX 77030 (Dr Alpert).

lasted 30 minutes. When vision returned
she noticed a slight blurriness in the lower
half of the visual field of the left eye. She
had a past history of hypertension and

chronic obstructive pulmonary disease.
Findings of the general physical exami¬
nation were normal except for a blood
pressure of 180/110 mm Hg. Neurological
examination showed only a mild right
ptosis and left carotid bruit.
A complete blood cell count and urinalysis were normal. The blood urea nitrogen,
fasting blood sugar, liver function tests,
electrolyte, calcium, and phosphorus levels
were also normal. A chest roentgenogram
showed emphysematous changes. The skull
roentgenogram series was normal. The
ECG demonstrated no significant abnor¬
malities.
Electroencephalography showed left
temporal theta activity. Angiography
demonstrated a left internal carotid occlu¬
sion and 90% stenosis of the left external
carotid artery (Figure). Good opacification
of left middle cerebral vessels was obtained
through the posterior circulation. Verte¬
bral and basilar studies showed no anoma¬
lous vessels, occlusive disease, or posterior
fossa mass.
The patient remained asymptomatic and
was discharged on a regimen of aspirin, 10
grains per day.
The patient was readmitted to St Luke's
Hospital on May 19,1975, because of "black
out" spells. For two days she had several
episodes of lightheadedness, generalized
weakness, and bilateral visual loss lasting
15 to 30 seconds. These episodes were not
related to position.

Downloaded From: http://archneur.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 06/06/2015

seconds. The heart rate then
returned to about 70 beats per minute.
Compression of carotid sinus also produced
asystole for several seconds.
The patient was treated with atropine to
maintain a pulse of 80 beats per minute.
Phenytoin was given for the neuralgia
with no benefit. Carbamazepine was added
at 600 mg/day, and partial relief of pain
was obtained but episodes of asystole
persisted. A transvenous pacemaker was
inserted, but despite this, several episodes
of hypotension and bradycardia occurred.
Isoproterenol was given without benefit.
Because of the failure of medical treat¬
ment and the transvenous pacemaker to
control bradycardia and hypotension, a
seven

was implanted.
During surgery several hypotensive epi¬

permanent pacemaker

Left carotid angiogram demonstrates left
carotid occlusion and high grade left
external carotid stenosis.

There had been a two-month history of
intermittent episodes of sharp left ear and
throat pain. The pain occurred suddenly,
several times a day, and lasted a few
seconds. She was not initially aware of any
factors that stimulated the pain. She did
not complain of altered taste or saliva
secretion.
Findings of the general physical exami¬
nation were normal. Her heart rate was 76
beats per minute and regular and her blood
pressure was 140/100 mm Hg. Neurologic
examination findings were normal except
for a mild right ptosis and left carotid
bruit.
On subsequent examination several
spells were witnessed. They were mani¬
fested by sudden loss of consciousness,
head and eye deviation to the right, jerking
of all extremities, and brief postictal
confusion for about five seconds. These
always occurred when drinking fluid.
Severe left-sided throat pain was associ¬
ated with swallowing and preceded the loss
of consciousness. Vigorous stimulation of
the pharynx and tonsillar area did not
trigger the pain or seizures. Salivary secre¬
tion was not measured, but no obvious
changes were observed.
Results of the EEG during the loss of
consciousness were normal, no longer
showing left temporal theta activity. The
ECG demonstrated a baseline rhythm of 75
beats per minute. While drinking and swal¬
lowing the heart rate gradually dropped,
and finally asystole occurred for about

sodes occurred.
After pacemaker implantation the pa¬
tient became stuporous. A right Babinski
sign and an equivocal left plantar response
were present. She was dyspneic and had
rales at both bases with expiratory
wheezes.
Aminophyllin, digoxin, furosemide, and
intermittent positive pressure breathing
were administered with improvement. The
patient then had recurrence of left ear and
throat pain. An otolaryngologist found no
significant pathology. Repeat skull roent¬
genogram series with base views showed
no abnormality of the jugular foramen.
Carbamazepine was readministered, and
the pain was successfully controlled with a
dosage of 800 mg/day. After three weeks
in the intensive care unit, overt paranoid
behavior developed and chlorpromazine
was added to the regimen with return to
her previous personality state. There was
also continued neurologic improvement. At
the time of discharge the patient had no

significant neurologic abnormalities, no
pain, cardiac irregularities, or
hypotension. She has been maintained on
carbamazepine, 800 mg daily.
ear or throat

COMMENT

Diagnosis of this disorder may be
difficult. When our patient was
admitted to the hospital her primary
complaints were "blackouts," visual
loss, and lightheadedness. She had a
previously demonstrated left internal

carotid occlusion and left external
carotid stenosis. She was observed to
have clonic jerking of all extremities
associated with head and eye devia¬
tion to the right that lasted about five
seconds. These were initially thought
to be seizures with focal elements.
Simultaneous EEG recording showed

no

epileptiform activity or significant

alteration in background rhythm oth¬
er than movement artifact.
A vagovagal reflex1"-'1 or cardiospasm was suspected when swallow¬

ing produced progressive bradycardia
and asystole. Vigorous stimulation of
tonsillar and pharyngeal regions
failed to elicit pain or cardiac arrhyth¬
mia. Later, the patient described
sharp throat pain on the left side
while swallowing that preceded the

cardiac abnormalities and seizures. It
was then concluded that glossopha¬
ryngeal neuralgia precipitated asys¬
tole and seizures. The head and eye
deviation to the right may have been
due to the relatively greater ischemia
of the left cerebral hemisphere. Brain
stem pathology was excluded because
of the absence of other clinical brain
stem signs and normal vertebral and
basilar arteries on previous angiogra¬
phy. The possible connection of her
known vascular pathology, namely,
left internal carotid occlusion and left
external carotid stenosis, was then

investigated.
The principal afferent fibers of the
glossopharyngeal nerve are those aris¬
ing from the pharyngeal mucosa, the
nerve of Hering (carotid sinus nerve),
and Jacobson nerve, which supplies
the parotid gland, otic ganglion, and
mucous membrane of the middle ear.

The nerve of Hering joins the main
trunk of the glossopharyngeal nerve
near the jugular foramen, and Jacobson nerve joins the petrosal ganglion
in the lower part of the jugular
foramen. Vascular supply to this
region is from the ascending pharyn¬
geal branches of the external carotid

artery.

It is known that impulses can be
discharged from injured regions of
nerve.-'- After ischemia local irritation

of the nerve develops and repetitive
discharges may occur in bursts. Fiber
interaction is a normal process at
injured regions, and artificial syn¬
apses can be set up with motor-tosensory transmission in these areas.-3
Small pain fibers of the C-group are
practically lacking in accommodation
and easily excited by fiber interac¬
tion.21
Therefore, we propose a mechanism
of an ischemie injury to the ninth

Downloaded From: http://archneur.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 06/06/2015

cranial nerve resulting in an artificial
synapse, motor-to-sensory, in the re¬
gion of the jugular foramen to explain

glossopharyngeal neuralgia, asystole,
and seizures in our patient. Impaired
perfusion to this area occurred be¬
cause of a left internal carotid occlu¬

sion and left external carotid stenosis.
The artificial synapse would be above
the junction of the nerve of Hering
and Jacobson nerve close to the
jugular foramen. Manual stimulation
of tonsillar and pharyngeal regions
did not trigger pain, which supports
the theory of a more proximal defect
as the site of the pathology.
To summarize, swallowing attempts
resulted in impulses descending
through motor branches of the glosso¬
pharyngeal nerve. The artificial syn¬
apse in the region of the jugular
foramen resulted in diverting a
significant number of these impulses

directly to the sensory fibers and back

phenytoin or carbamazepine has also
been successful.1"·25·2" In our patient

solitarius and terminated in the dorsal
motor nucleus of vagus. The bombard¬
ment of impulses received at the
dorsal motor nucleus of vagus was
perceived as arising from the carotid
sinus through the nerve of Hering,
although, in fact, they were motor
impulses diverted from the periphery
via the artificial synapse. A maximal
vagai response was then generated as
a result of these misdirected impulses.
Consequently, bradycardia, and asys¬
tole occurred.
Treatment in the past has been
most successful with intracranial sec¬
tion of glossopharyngeal and upper
two rootlets of vagus nerves, while the
cardiac abnormalities are controlled
either with atropine or a transvenous
pacemaker.2 ,2"24 Treatment with only

controlling heart rate or hypotension.
Consequently, a permanent pacemak¬
er was implanted and carbamazepine

to the brain stem. There, transmission
continued in the fasciculus tractus

neither method

was

effective

in

was added. There is a recent

report of
the same treatment method.27 Subse¬
quently, her glossopharyngeal neural¬
gia has been completely controlled
with carbamazepine, 800 mg daily for
15 months. There have been no
further cardiac irregularities or hypotensive episodes.

Nonproprietary Names and
Trademarks of Drugs
Carbamazepine—Tegretol.
Chlorpromazine—Thorazine, Chlor-PZ,
Cromedazine, Promachel.

IsoproterencA—Norisodrine.

References
1. Kjellin K, Muller R, Widen L: Glossopharyngeal neuralgia associated with cardiac arrest and
hypersecretion from the ipsilateral parotid gland.
Neurology 9:527-532, 1959.

2. Khero BA, Mullins CB: Cardiac syncope due
to glossopharyngeal neuralgia. Arch Intern Med
128:806-808, 1971.
3. Stowell A, Gardner WJ: Glossopharyngeal
neuralgia. J Nerv Ment Dis 116:302-309, 1952.
4. Richburg PL, Kern CE: Glossopharyngeal
neuralgia with syncope and convulsions. JAMA
152:703-704, 1953.
5. Svien HJ, Hill NC, Daly DD: Partial glossopharyngeal neuralgia associated with syncope.
J Neurosurg 14:452-457, 1957.
6. Thomson JL: Glossopharyngeal neuralgia
accompanied by unconsciousness. J Neurosurg
11:511-514, 1954.
7. Roulhac GE, Levy I: Glossopharyngeal
neuralgia associated with cardiac arrest and
convulsions. Arch Neurol Psychiatry 63:133-139,
1950.
8. Garretson HD, Elvidge AR: Glossopharyngeal neuralgia with asystole and seizures. Arch
Neurol 8:26-31, 1963.
9. Ray BS, Stewart HJ: Glossopharyngeal
neuralgia: A cause of cardiac arrest. Am Heart J
35:458-462, 1948.
10. Kong Y, Heyman A, Entman M, et al:

Glossopharyngeal neuralgia associated with
bradycardia, syncope and seizures. Circulation
30:109-113, 1964.

11. Weisenburg TH: Cerebello-Pontile tumor
diagnosed for six years as tic douloureux. JAMA

54:1600-1604, 1910.
12. Lillie HI, Craig WMcK: Anomalous vascu-

cerebellopontine angle, severe
neuralgic pain in the ear and profound nervous
disturbance: Operation and recovery. Arch Otolaryngol 23:642-645, 1936.
13. Pope FM: Thrombosis of vertebral artery
pressing on glossopharyngeal nerve: Unilateral
lar lesion in

loss of taste at back of tongue. Br Med J 2:1148\x=req-\
1149, 1889.
14. Brihaye J, Perier O, Smulders J, et al:

Glossopharyngeal neuralgia caused by compres-

sion of nerve by atheromatous vertebral artery.
J Neurosurg 13:299-302, 1956.
15. Deparis M: Glossopharyngeal neuralgia in
Vinken PJ (ed): Handbook of Clinical Neurology.
New York, John Wiley & Sons Inc, 1968, vol 5, pp
350-361.
16. Dandy WE: Glossopharyngeal neuralgia
(tic douloureux). Arch Surg 15:198-214, 1927.
17. Graf CJ: Glossopharyngeal neuralgia and
ossification of the stylohyoid ligament. J Neuro-

surg 16:448-453, 1959.
18. Kopald HH, Roth HP, Fleshler B, et al:

Vagovagal syncope. N Engl J Med 271:1238-1241,

1964.
19. Sapru RP: An unusual cause of syncope.
Proc R Soc Med 61:955-956, 1968.
20. James AH: Cardiac syncope after swallowing. Lancet 1:771-772, 1958.
21. Iglauer S, Schwartz BA: Heart-block periodically induced by the swallowing of food in a
patient with cardiospasm (vagovagal syncope).
Ann Otol Rhinol Laryngol 45:875-880, 1936.
22. Kugelberg E, "Injury activity" and "trigger zones" in human nerves. Brain 69:310-324,
1946.
23. Granit R, Leksell L, Skoglund CR: Fibre
interaction in injured or compressed region of
nerve. Brain 67:125-140, 1944.
24. Adson AW: The surgical treatment of glossopharyngeal neuralgia. Arch Neurol Psychiatry
12:487-506, 1924.
25. Ekbom KA, Westerberg CE: Carbamazepine in glossopharyngeal neuralgia. Arch Neurol
14:595-596, 1966.
26. Kiluk KI, Knighton RS, Newman JD: The
treatment of trigeminal neuralgia and other
facial pain with carbamazepine. Mich Med
67:1066-1069, 1968.
27. Jamshidi A, Masroor M: Glossopharyngeal
neuralgia with cardiac syncope. Arch Intern Med
136:843-845, 1976.

Downloaded From: http://archneur.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 06/06/2015