Historical Perspectives on the Diagnosis and Treatment of Trigeminal Neuralgia

Chad D. Cole, M.SC.; James K. Liu, M.D.; Ronald I. Apfelbaum, M.D.

Disclosures

Neurosurg Focus. 2005;18(5) 

In This Article

Abstract and Overview

Since the earliest recorded history of medicine, physicians have been challenged by the difficulty in relieving the great pain experienced by individuals suffering from trigeminal neuralgia (TN). The nature of the pain and the events that incite it have been well described, but effective treatments with acceptable levels of side effects remained elusive until the latter part of the 20th century. As a result, many theories about the origins of TN have been proposed, along with numerous treatment modalities. The pathophysiological causes of TN remain incompletely understood, but the medical and surgical treatment techniques currently used offer effective ways to relieve this extremely painful condition. In this historical review the authors discuss the initial descriptions of tic douloureux, Fothergill disease, and TN, along with various therapeutic interventions and their refinements.

Trigeminal Neuralgia as a Clinical Entity. Trigeminal neuralgia, also known as tic douloureux or Fothergill disease, is a clinical syndrome distinguished by brief paroxysms of unilateral, lancinating facial pain that is characteristically triggered by cutaneous stimuli, such as a breeze on the face, chewing, talking, or brushing the teeth.[3,4,38] Many of those who are affected experience multiple attacks daily, and, although they are free of pain between attacks, they live in constant fear of recurrences.[38] The mysterious nature of this disease confused the early development of treatment, because the origin remained elusive. Nevertheless, a review of the history of TN indicates that innovative observations made by discerning clinicians resulted in methods that were at least partially successful in alleviating the pain of this disease, despite the limited understanding of its exact pathogenesis.

Aretaeus of Cappodocia provided one of the earliest descriptions of migraine headache in the second century, and his description has also been credited as the first account of TN.[50,51,59] As Stookey and Ransohoff[59] relate, in his chapter "Cephalaea," Aretaeus made no clear distinction between symptoms that represented a migraine headache and those that represented TN. Approximately 900 years later (AD 1037), as recounted by Ameli,[1] Avicenna described two facial syndromes; one was characterized by "a disease in which the face is pulled unnaturally, its normal shape is distorted and the natural ability of both lips meeting is inhibited." This clearly was a description of facial palsy, but the second case included pain that had a throbbing, tearing, and stabbing nature, characteristics consistent with TN.

The first clear description of TN that was not confused with another disease entity was made in 1688 by Drs. Johannes Michael Fehr and Elias Schmidt, secretaries of the Imperial Leopoldian Academy of Natural Sciences.[59] In their eulogy of Johannes Laurentius Bausch, they vividly described the painful symptoms that had plagued the distinguished founder of their Academy for the 4 years prior to his death. Bausch was said to have experienced a sharp, shooting pain in his right maxilla that came on suddenly like a bolt of lightening, varying in intensity, leaving him unable to speak or eat solid food.[59] In the fall of 1665, the pain disappeared, only to return early that same winter. The pain that Bausch subsequently experienced was so intense that it forced him to remain bedridden. He eventually died of malnutrition because his dietary intake was limited by the excruciating pain.[19]

John Locke, a physician and well-known philosopher, provided the first full description of TN by a medical practitioner, along with an account of its treatment. In 1677, while in Paris, Locke was called in to evaluate the wife of the English ambassador, the Countess of Northumberland, who was suffering from excruciating pain in the face and lower jaw.[37,52] Locke deliberated whether to prescribe "opening medicine" (laxative therapy), because of the wintry weather at the time. In spite of the cold and the inconvenience he would cause his patient, he eventually overcame his reluctance and thoroughly purged the Countess. Her pain improved several weeks later.[59]

Nearly a century after that, two prominent clinical accounts were reported, one by Nicolas André (Fig. 1A) and one by John Fothergill (Fig. 1B) that further characterized this disease entity. In 1756, André reported two cases of TN, which he termed tic douloureux.[2,14,52] He conceptualized the disease in terms of convulsions, and he believed that true tonic convulsions, tetanus, and spasm clinique belonged in a single disease spectrum. The term tic douloureux was used to imply contortions and grimaces accompanied by violent and unbearable pain. André believed that the cause was "vicious nervous liquids" that distressed the nerve and caused painful shocks. Using this reasoning, he followed the proposal of Maréchal (a contemporary surgeon) by applying caustic substances to the infraorbital nerve at the infraorbital foramen over a period of days until the nerve was destroyed.[43,59]

A: Portrait of Nicolas André. (Reprinted from Brown JA, et al: Mercury water and cauterizing stones: Nicolas André and tic douloureux. J Neurosurg 90: 977–981, 1999.) B: Portrait of John Fothergill. (Reprinted with permission from Stookey B, Ransohoff J: Trigeminal Neuralgia: Its History and Treatment. Springfield, IL: Charles C Thomas, 1959.)

In 1773, John Fothergill, an English physician, presented 14 cases of a painful affliction of the face.[20] His description of TN has been considered an accurate and clear account.[59] In Fothergill's description he made the following comments:

The affection seems to be peculiar to persons advancing in years, and to women more than to men.... The pain comes suddenly and is excruciating; it lasts but a short time, perhaps a quarter or half a minute, and then goes off; it returns at irregular intervals, sometimes in half an hour, sometimes there are two or three repetitions in a few minutes.... Eating will bring it on some persons. Talking, or the least motion of the muscles of the face affects others; the gentlest touch of a hand or a handkerchief will sometimes bring on the pain, whilst a strong pressure on the part has no effect. [20]
Fothergill asserted that TN was not within the spectrum of a convulsive disorder. Instead, he postulated that this disease might be the manifestation of some type of cancer; he found a hard tumor of the breast in two of the 14 cases he presented. Given his meticulous description of the clinical symptoms, many thereafter referred to this condition as "Fothergill's disease." [43,59]

Thus, although a clear description of this distinctive condition had been made by the end of the 18th century, it was not until the 1820s, when Charles Bell established the separate functions of the trigeminal and facial nerves, that tic douloureux and Fothergill disease would be truly differentiated from other conditions.[67] Bell's contribution enabled tic douloureux to be localized to the trigeminal nerve, which ultimately led to the evolution of the name of the disease to trigeminal neuralgia.

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