Atypical
facial neuralgia, a syndrome of vascular
pain
Fay Temple
The three cases presented are the first of
their kind on reord. They have definitely
established certain factors regarding atypial
neuralgia, other than true trigeminal neuralgia.
They establish the functions of the seventh,
ninth and tenth nerves in their intracranial
component. The great intermingling of these
structures with adjacent nerves after leaving
the cranial fossa has left the determination of
their nuclear and root functions individually a
matter of conjecture. By sectioning these nerves
close to their point of origin along the lateral
aspect of the medulla before they have had the
opportunitty to give off branches to one
another, observations of their individual
function have been possible.
[...]
page 311-313
CASE 3.-The final result of section
of the posterior root of the fifth nerve, the
resection of Meckel's ganglion, stripping tue
common carotid, ablation of the superior
cervical ganglion and trunk. Section of portion
of the seventh nerve.
This case is of extreme interest in that the
patient showed the so-called "Sluder syndrome"
of pain below the eye, traced along the zygoma
back toward the ear. The sphenopalatine ganglion
was removed and the posterior root of the fifth
nerve was cut without affecting the pain. The
common carotid artery was stripped with partial
relief of pain. The superior cervical ganglion
was removed, with complete relief of pain.
During operation on the sphenopalatine ganglion,
the upper branch of the seventh nerve was
sectioned (transzygomatic approach). In view of
the anesthesia produced by destruction of the
posterior root of the fifth, this gave an ideal
opportunity for comparing the two zones, that of
the forehead with that of the cheek, the
forehead being without either the supply of the
fifth or seventh nerve, the cheek being without
the supply of the fifth nerve but still
retaining that of the seventh nerve.
The case is unique in that it offers
opportunity for study of two adjacent zones for
deep pressure sense. On careful testing it was
found that deep pressure sense was lost over the
forehead when the seventh and fifth nerve supply
had been destroyed. It was present below the
eyes when the seventh nerve supply still
remained, even though the fifth nerve supply had
been destroyed, all other forms of sensation
being absent. It would therefore seem evident
that the seventh nerve does carry deep pressure
sense as was pointed out by Davis in his
experiments on the cat, and first called
attention to by Spiller in 1906. The so-called
geniculate zone of Ramsey Hunt in and about the
ear, on the posterior aspect of the concha, in
reality shows that this zone is supplied by the
tenth, and not the seventh nerve, the
inflammatory lesions being manifested by
involvement of the ramus auricularis nervi vagi,
which often fuses with the seventh nerve before
leaving the stylomastoid foramen and is
distributed with its fibers peripherally.
This case further represents the fact that
the so-called "Sluder syndrome" may be due in
some instances to disease of Meckel's ganglion,
but in other instances it is probably due to a
disease much lower placed in either the thorax
or abdomen with referred pain by means of the
higher sympathetic arcs to the face. This will
explain the reason that other observers have not
been able to confirm Sluder's observations in
many cases, though it is well recognized that
certain cases do yield to sphenopalatine
injection or destruction.
Three of my patients who have beer under,
treatment for atypical neuralgia of the right
side for a period of from three to four years,
with some relief of symptoms due to injection of
the sphenopalatine, subsequently developed
symptoms referabIe to the gallbladder and were
operated on this condition. After removal of
gallstones in three patients, the atypical
neuralgia cleared up spontaneously. In another
case, I discovered a lesion in the apex of the
lung -the pain in all four of these cases being
referred to the face as in the true "Sluder
syndrome." This may be a coincidence, but I
suspect not.
It therefore seems evident that pathologic
changes in the chest, or abdomen may refer to
the higher arcs evidences of irritation which
are relayed forward into the face. Destruction
of the sphenopalatine ganglion therefore in
these cases will not relieve pain, as the pain
is similar to that seen in amputation neuromas,
where, even after the member has been removed,
the pain is often referred into the extremities
which have long since been absent. A case of a
patient operated on this week seems definitely
to establish the fact that the superior cervical
ganglion acts as a higher relay arc station in
the transmitting of this pain to the face. The
patient operated on by Dr. Elsberg with section
of the posterior root of the fifth nerve for
pain of the atypical type, was later operated on
by Dr. Stookey, with section of the jugular and
carotid branches just above the superior
cervical ganglion. The patient still had pain in
the face and was extremely tender to pressure
over the cervical sympathetic nerve on the left
side. Removal of the superior cervical ganglion
and its chain has produced complete relief of
pain in the face on that side.
As in other cases in this series, pain is
frequently relieved from reference to the face
by removal of the superior cervical ganglion
chain, but pressure over the sympathetics below
the site of operation produces tenderness, and
there is pain sometimes referred to the arm and
shoulders, showing that the pathologic change
lies below, but does not find a means of
expression in the higher arcs after ablation of
the superior cervical ganglion. This case is
therefore an example of the fact that in some
cases atypical facial neuralgia may be due to
involvement of the sphenopalatine ganglion, but
in other cases, it is probably due to pathologic
changes lying deep in the abdomen or, chest, and
therefore a search for this source of
involvement does not lie in the head or neck; it
is futile to operate on these patients in the
sphenopalatine region. A means of differential
diagnosis in this type of case is offered. It
will be found, however, on carefully testing the
patient, that this pain is most intense when the
sympathetic chain is involved by pressure. It
therefore becomes a diagnostic sign which I have
termed "carotidynia"; I have found it
present in all cases of involvement of the
sympathetic system, from lesions other than that
of true "Sluder syndrome." If patients with
cases of this character are not operated on for
relief from pain in Meckel's ganglion, but are
considered from the standpoint of deep
pathologic changes of the chest or abdomen, it
will offer, less confusion as to observations
and results. This evidence of
"carotidynia", has not been found present
in true trigemitial neuralgia. It is only
slightly present in the cases of true "Sluder
syndrome" (lower half headache of true
sphenopalatine origin). It is always present in
those cases of atypical neuralgia, in which the
pathologic process is thought to lie in the
chest or abdomen, with pain referred to the
higher arcs through the superior cervical
ganglion.
During the acute stage of sinus disease, I
have seen the "carotidynia" marked on the
side of the atypical neuralgia, and pressure
over the superior cervical ganglion referred the
pain directly into the face, below the eye, deep
in the region of the zygoma. Irritation through
the higher sphenopalatine ganglion may therefore
produce hyperirritability of the superior
cervical ganglion, indicating that this
structure is a relay center for arcs both from
above and below.
The important feature in the two cases cited
is that the pain fibers must enter the cervical
cord to reach consciousness, and that these do
enter below the superior cervical ganglion
whether referred from Meckel's above or the
thoracic trunk below. That the superior cervical
ganglion does play an important role in their
transmission (cutting the fibers above the
ganglion does not relieve the pain) is due
either to pathologic change below or to
involvement of the ganglion itself (two
ganglions now being studied). Wherever the
cause, if tractable and unyielding to local
measures, the relief of pain can be obtained
only by section of the fibers that carry the
sensation to the brain. These fibers enter the
cervical cord. Section of the connections of the
sympathetic chain with the cord will relieve the
pain by interruption of the pain arc to
conscious levels; it does not cure the
underlying cause any more than section of the
posterior root of the fifth nerve "cures" true
trigeminal neuralgia. It simply destroys the
pathways for the reception of pain to
consciousness.
The real cause of trigeminal neuralgia is
not known any more than the cause of "atypical
facial neuralgia." The relief of pain in both
cases lies in destruction of the pain arc.
Ramisectomy therefore may supplant resection of
the cervical chain and should be considered in
order to avoid, if possible, the resultant
myosis and enophthalmos that accompanies
cervical sympathetic ablation.
