mise à jour du
26 février 2006
Ann Otol Rhinol Laryngol
1932; 41; 1030-1062
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.
Superior laryngeal neuralgia: carotidynia or just another pain in the neck?
O'Neill B, Aronson A, Pearson B, Nauss L
The myth of carotidynia
Biousse V, Bousser M.
Carotidynia: a pain syndrome
Hill LM, Hastings G
J Fam Pract
1994;39:71 -75
Fay T. Atypical facial neuralgia. Arch Neurol Psychiat.1927;18:309-315
Fay T. Atypical facial neuralgia, a syndrome of vascular pain. Ann Otol Rhinol Laryngol 1932; 41; 1030-1062
Buetow MP, Delano MC. Carotidynia. AJR Am J Roentgenol. 2001;177(4):947
Burton BS, Syms MJ, Petermann GW, Burgess LPA. MR imaging of patients with carotidynia. AJNR 2000;21:766 -769
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