Trigeminal neuralgia and other cranial neuralgias

Alan Stiles MD (Dr. Stiles of Thomas Jefferson University Hospital has no relevant financial relationships to disclose.)
Stephen D Silberstein MD, editor. (Dr. Silberstein, Director of the Jefferson Headache Center at Thomas Jefferson University, receives honorariums from Allergan, Avanir Pharmaceuticals, Curelator, Depomed, Dr. Reddy's Laboratories, eNeura,  INSYS Therapeutics, Lilly USA, Supernus Pharmacerticals,  Thernica and Trigemina for consulting. He is also the  principal investigator for a clinical trials conducted by Alder Biopharmaceuticals, Amgen, electroCore Medical, Lily USA and Teva.)
Originally released May 8, 2001; last updated May 21, 2005; expires May 21, 2008
Notice: This article has expired and is therefore not available for CME credit.

This article includes discussion of trigeminal neuralgia and other cranial neuralgias, atypical odontalgia, cranial neuralgias and facial pain of unknown cause, facial pain of unknown origin, idiopathic facial pain, tic douloureux, trigeminal nerve disorder, trigeminal neuropathic pain, trigeminal neuropathy, atypical facial pain, geniculate neuralgia, glossopharyngeal neuralgia, herpetic and postherpetic neuralgia of the trigeminal nerve, occipital neuralgia, persistent idiopathic facial pain, raeder syndrome, trigeminal neuropathy, trigeminal neuralgia, trigeminal dysesthesia, and trigeminal dysesthesia-sympathetically maintained. the foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Historical note and terminology

Trigeminal neuralgia is the classic neuropathic pain, first described by Aretaeus in the first century AD. Many physicians have penned descriptions of this affliction; the first to comprehensively describe it was John Locke in 1677. He describes a patient as having:

…such violent and exquisite torment, that it forced her to such cries and shrieks as you would expect from one upon the rack, to which I believe hers was an equal torment, which extended itself all over the right side of her face and mouth. When the fit came there was, to My Lady's own expression of it, as it were a flash of fire all of a sudden shot into all of those parts, and at every one of those twitches which made her shriek out, her mouth was constantly drawn to the right side towards the right ear by repeated convulsive motions, which were constantly accompanied by her cries… These violent fits terminated on a sudden and then My Lady seemed to be perfectly well… (Locke 1789)

John Hunter (1728-1793), an English physiologist and surgeon, describes trigeminal neuralgia in his Treatise on the Natural History of the Human Teeth in 1778 as:

This pain is seated in some one part of the Jaws. As simple pain demonstrates nothing, a Tooth is often suspected, and is perhaps drawn out; but still the pain continues, with this difference however, that it now seems to be in the root of the next Tooth: it is then supposed either by the patient or the operator, that the wrong Tooth was extracted; wherefore, that in which the pain now seems to be, is drawn, but with as little benefit. I have known cases of this kind, where all the Teeth of the affected side of the Jaw, have been drawn out, and the pain has continued in the Jaw; in others, it has had a different effect, the sensation of pain has become more diffused, and has at last, attacked the corresponding side of the tongue. In the first case, I have known it recommended to cut down upon the Jaw, and even to perforate and cauterize it, but all without effect.

Hence it should appear, that the pain, in question, does not arise from any disease in the part, but entirely a nervous affection.”

In 1912 Osler captured the essence of the clinical description of trigeminal neuralgia as it was understood in his time:

In advanced cases the paroxysms follow one another rapidly and without assignable cause, and in the intervals the patient may never be quite free from pain. They are inaugurated by almost any form of external stimulus, by a draught of air, by movement of the facial muscles or of the tongue in speaking, by touching the skin, particularly over those points from which the pain seems to take its origin, by the act of swallowing, especially when the pain involves the mucous membrane field of distribution of the nerve. It is not a self-limited disease. In some instances the neuralgia reaches such a frightful intensity that it renders the patient's life insupportable. In former years suicide was not an uncommon consequence (Osler 1912).

For years, if one excluded trigeminal neuralgia and glossopharyngeal neuralgia, no other diagnosis was made. Osler makes brief mention of a form of cervico-occipital neuralgia, a subject that is controversial to this date (Osler 1912). Osler is silent on other categories of facial pain. Clark and Taylor describe the case of a woman with geniculate neuralgia, with pain just in front of the left ear, which was relieved by surgical section of the seventh and eighth cranial nerves, leaving her with a complete peripheral facial palsy.

Patients who did not fit the criteria for trigeminal, glossopharyngeal, or geniculate neuralgia had their pain labeled “atypical” or “idiopathic” pains of the face. Lacking etiology and mechanisms, physicians considered these pains to be of psychogenic origin. The term “atypical facial pain” has been used since 1924 for pain in the facial region that does not fit the criteria for trigeminal neuralgia (Pfaffenrath et al 1993). Stomatodynia, atypical odontalgia, phantom tooth pain, myogenic pain, traumatic neuralgia, trigeminal nerve disorder, trigeminal neuropathy, trigeminal neuropathic pain, and oral and facial dysesthesias are all included under the rubric “idiopathic or atypical facial pain” (Woda and Pionchon 1999). The variety of terms and lack of etiology yields no uniform definition and skeptical acceptance of diagnostic criteria. Basic science pain research is expanding our knowledge of both peripheral and central pain physiology and leading clinicians to integrate this knowledge into their clinical decision making. Pain research, new pharmacological options, and increased clinical interest in and treatment of pain conditions have led to a plethora of literature describing the conditions that produce facial pain and has revealed facial pain to be less psychogenic and more pathophysiologic (Graff-Radford and Solberg 1993).

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