Vous êtes sur la page 1sur 5

LEGACY

HISTORICAL CHARACTERIZATION OF
TRIGEMINAL NEURALGIA
Paula Eboli, M.D.
Department of Neurosurgery,
University of Illinois at Chicago,
Chicago, Illinois

James L. Stone, M.D.


Department of Neurosurgery,
University of Illinois at Chicago,
Chicago, Illinois

Sabri Aydin, M.D.


Department of Neurosurgery,
University of Illinois at Chicago,
Chicago, Illinois

Konstantin V. Slavin, M.D.


Department of Neurosurgery,
University of Illinois at Chicago,
Chicago, Illinois
Reprint requests:
James L. Stone, M.D.,
912 S. Wood Street,
Chicago, IL 60612.
Email: jlstone4@aol.com
Received, June 18, 2008.
Accepted, October 9, 2008.
Copyright 2009 by the
Congress of Neurological Surgeons

TRIGEMINAL NEURALGIA IS a well known clinical entity characterized by agonizing,


paroxysmal, and lancinating facial pain, often triggered by movements of the mouth
or eating. Historical reviews of facial pain have attempted to describe this severe pain
over the past 2.5 millennia. The ancient Greek physicians Hippocrates, Aretaeus, and
Galen, described kephalalgias, but their accounts were vague and did not clearly correspond with what we now term trigeminal neuralgia. The first adequate description of
trigeminal neuralgia was given in 1671, followed by a fuller description by physician
John Locke in 1677. Andr described the convulsive-like condition in 1756, and named
it tic douloureux; in 1773, Fothergill described it as a painful affection of the face;
and in 1779, John Hunter more clearly characterized the entity as a form of nervous
disorder with reference to pain of the teeth, gums, or tongue where the disease does
not reside. One hundred fifty years later, the neurological surgeon Walter Dandy
equated neurovascular compression of the trigeminal nerve with trigeminal neuralgia.
KEY WORDS: Facial pain, Historical review, Trigeminal neuralgia
Neurosurgery 64:11831187, 2009

DOI: 10.1227/01.NEU.0000339412.44397.76

rigeminal neuralgia (TN) is a well known


clinical diagnosis, characterized by agonizing, paroxysmal, and lancinating pain (7).
The pain is perceived within 1 or more divisions
of the trigeminal nerve, is usually brief with
repetitive bursts every few seconds, and can be
triggered by activities such as chewing, speaking, brushing the teeth, swallowing, or touching
the face. A discrete trigger zone stimulated by
touch or even a breeze of air can set off a typical
paroxysm of pain. Characteristically, when
asked, the patient will point to this zone on the
face avoiding direct touch to that area. The pain
is virtually always unilateral, with rare reports of
bilateral symptoms (17, 19).

HISTORICAL ASPECTS
OF FACIAL PAIN
There have been many historical descriptions
of facial pain over the past several thousand
years. Hippocrates (circa 460377 BC) of ancient
Greece is credited with being the first physician
to reject superstitions, supernatural beliefs, or
divine forces as the cause of illness and has
often been called the Father of Medicine. He
ABBREVIATIONS: TN, trigeminal neuralgia

NEUROSURGERY

www.neurosurgery-online.com

greatly advanced the systematic study of clinical medicine, summarized the medical knowledge of previous schools, and advocated practical and ethical physician practice through the
Hippocratic Oath and other works. Hippocrates
might have encountered TN, but unfortunately
his broad characterization of headaches as consisting of infinite forms, was too vague (12).
The same is true of Aretaeus (150 AD) and
Galen (circa 129200 or 216 AD), 2 of the most
celebrated ancient Greek physicians. These
authors differentiated heterocrania (Galen)
and hemicrania (Aretaeus), terms reflecting
unilateral headache, from what they called
other head ailments or kephalalgia (16).
Aretaeus (4, 12, 21) of Cappodocia, a 2nd century AD physician who followed the method of
Hippocrates, is believed to be the first to
describe hemicranial headaches as occurring in
paroxysmal attacks, separated by pain-free
intervals, accompanied by a facial spasm, and
followed by a fainting spell. However, further
analysis of the signs and symptoms described in
his patient would likely be more compatible
with atypical TN or migraine (12).
In analyzing the history of facial pain, there
is an important error that has been copied
again and again (12). It is the belief that the
Arabic medical school possessed knowledge

VOLUME 64 | NUMBER 6 | JUNE 2009 | 1183

EBOLI ET AL.

of TN or tic douloureux. This is mainly the result of a misinterpretation of an equivocal Latin translation of the Arabic text,
The Canon of Medicine or The Law of Medicine, a 14-volume
Arabic medical encyclopedia written by Ibn Sina (Avicenna;
9801037) and completed in 1025. Ibn Sina, a Persian, was the
foremost physician and Islamic philosopher of his time. He
discussed what he called lakwat. In Latin, the title of this
chapter was Tortura facei. The unfortunate choice of the
word torturaequivalent to torture and distortionapparently produced the mistake. The Arabian expression was
indeed composed of the negation la and the substantive
kuwwet, meaning strength, power, or nerve. Therefore,
Lakwat means strengthlessness weakness or, in general,
paralysis. The entity in question was more likely facial
paralysis (12).
The first clear description of TN was provided in 1671. The
patient was a well known physician, Johannes Laurentis
Bausch of Germany (16051665) (Fig. 1), founder and first president of the Imperial Leopoldian Academy of Natural Sciences,
who suffered from severe TN
for 4 years. The pain prevented him from eating any
solid food and he was almost
unable to speak. Emaciation
gradually occurred and led to
death from a stroke in 1665.
Bauschs illness was detailed
in his eulogy published in the
Academy volume covering
the year 1671 (4, 8, 12, 21).
The well known philosopher and physician John
Locke (16321704) provided
the first full description of TN
and its treatment performed
by a physician (15). While in
Paris in 1677, Locke was calFIGURE 1. Portrait of Johannes
led to see the wife of the EngLaurentis Bausch (16051665).
lish Ambassador, the countess of Northumberland, who
was suffering an excruciating pain in the face and lower jaw (13,
14). Two teeth had been removed without relief. In letters to his
friend Mapletoft, he described her suffering in detail and outlined his treatment, which included a thorough purging of the
lady (1315).
The recognition of TN as a definite clinical entity is credited to
Nicolaus Andr in 1756 (1, 3, 21) (Fig. 2). Andr was a French surgeon who conceived the illness in terms of convulsions. He followed the methods used by Aretaeus and Caelius Aurelianus
(5th century Roman physician) to establish a differential diagnosis between true tonic convulsions, tetanus, and spasm cinique
(lip retraction as in smiling). He concluded the convulsive
movements that disturbed his patients could not be described
under spasm cinique but were more appropriately designated
tic douloureux. The term tic douloureux was used to imply facial
wincing, grimacing, and contortions that accompanied the violent

1184 | VOLUME 64 | NUMBER 6 | JUNE 2009

and unbearable pain (1, 3, 4).


Andr reported 5 cases, the
first of which he saw in 1732.
Of the 5 patients reported in
detail, only the second and
third cases were true TN.
Andr believed that the cause
was a local disease process or
vicious nervous liquids that
irritated the affected nerve
and caused painful shocks.
Using this reasoning, he followed the proposal of Marchal, contemporary surgeon
to Louis XIV, and ap plied
FIGURE 2. Portrait of Nicolaus
caustic substances to the
Andr (170418th century).
infraorbital nerve at its infraorbital foramen over a period
of days until the nerve was
destroyed (4).
Approximately 17 years
after Andrs description in
French, the English physician
John Fothergill (Fig. 3) decribed the condition in his
1773 book Medical Obser vations and Inquiries. Fothergill
wrote of a painful affection
to the face, a disease that
was not a convulsive disorder
(9). He said that this condition
affected people of advanced
age, women more than men,
and that it could be related to
FIGURE 3. Portrait of John Fothertumors. He des cribed the
gill (17121780).
pain as sudden and excruciating, lasting a short time,
and returning at irregular intervals. He also stated that, to his
knowledge, this was a newly described condition.
John Hunter (17281793) (Fig. 4), the foremost British anatomist and surgeon of his time, credited as being the
Founder of Scientific Surgery, had a profound and original
understanding of functional anatomy and physiology. He
thought that any sensory stimulation carried to excess could
result in pain, and when a large nerve is compressed, the
most acute sensation (pain or numbness) will be some distance below the compression (20). Hunter did not adhere to
the then-popular concept that nerves function as hollow tubes
conveying a fluid. He knew that inflammatory pain arose
from the nerve endings of a diseased part, but that pain of the
nervous kind arose from the nerves themselves being
affected, with the parts that these nerves lead to being unaffected. He clearly described what we call forms of referred
pain today (20).
Nervous problems and pain were of much interest to Hunter.
In 1778, he wrote the Natural History of the Human Teeth with a

www.neurosurgery-online.com

HISTORICAL CHARACTERIZATION OF TRIGEMINAL NEURALGIA

pertinent chapter entitled


Nervous Pains in the Jaws
(10). In the monographs
preface, he states he had
been aware of most of these
conditions before 1755 and
had lectured on these topics
in his anatomy and surgery
course since that time:
There is one disease of
the jaw which seems in
reality to have no connection with the teeth,
but of which the teeth
are generally suspected
to be the cause . . . a
tooth is often suspected
and it is drawn out; but
FIGURE 4. Portrait of John Hunter
still the pain contin(17281793).
ues. . . . It is then supposed that the wrong tooth was extracted where-upon
that in which the pain now seems to be in 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 . . . the sensation of pain has
become more diffused, and has at last, attacked the
corresponding side of the tongue. Hence it should
appear that the pain in question does not arise from
any disease in the part, but it is entirely a nervous
affection (10, pp 6162).
John Hunters early contribution to TN seems to have been
overlooked in previous reviews (4, 8, 12, 21).
After descriptions by Andr, Fothergill, and Hunter, occasional cases of the condition were reported. In 1782, Thouret
considered it to be an affection of the nerves of the face, especially in the distribution of the infraorbital, but also implicating
the lower jaw (4). In 1802, Chaussier classified the various
forms of tic douloureux according to the 3 trigeminal divisions,
but included the VIIth nerve as well (4). Very little was added
to the above clinical descriptions, but it was Fothergills great
nephew Samuel Fothergill who more definitively labeled the
site of the lesion as the trigeminal nerve in his Concise and
Systematic Account of a Painful Affection of the Nerves of the
Face Commonly Called Tic Dolourex. Consequently, TN has
been called Fothergills disease (18).
Nevertheless, the facial grimacing accompanying tic
douloureux led many to believe that the condition involved
both the trigeminal (V) and facial (VII) nerves as a unit. It was
not until the 1820s, when Charles Bell (17741842) characterized the separate functions for these 2 nerves, that tic
douloureux or TN was truly considered localized to the trigeminal nerve (2, 6). However, as late as 1919, prominent physicians
in England and France, impressed with the convulsive facial
spasms, thought that tic douloureux was an epileptiform

NEUROSURGERY

neuralgia signifying a spasm


of muscle that was painful,
rather than a pain of spasmodic character (11, 22).
In the 1930s, while performing partial sectioning of the
trigeminal root for typical TN
by way of a posterior fossa approach, the neurological surgeon Walter Dandy (1886
1946) (Fig. 5) noted that, The
sensory root is frequently
indented, lifted up or bent at
an angle by the artery (5, p
450). This I believe is the
cause of tic douloureux (6, p
170). By this time, with accuFIGURE 5. Photograph of Walter
Dandy (18861946).
mulated surgical knowledge,
it was also appreciated that
an occasional tumor in the cerebellopontine angle could impinge
upon the trigeminal nerve and cause a similar or atypical TN.

CONCLUSION
Reports of facial pain have been progressively described in
the literature from Hippocrates (circa 400 BC) to the present
time. The understanding and characterization of this condition
has evolved through the years to be known as TN, either typical or atypical. Thus, our accumulated information on tic
douloureux or TNa fascinating and challenging condition
approximates much of the period of recorded medical literature
and yet is certainly not complete. So often, medical knowledge
yields more questions for future generations than answers
yet, by nature, we continue in pursuit.

REFERENCES
1. Andr M: Practical observations on urethral diseases, and factual information on
convulsive facial contortions with principles for cure of associated gangrenous and
cancerous conditions by use of various solvents and caustics. College of the Royal
Academy [in French]. Paris, De Chir.rue S. Jacq. A lOlivier, 1756.
2. Bell C: On the nerves; giving an account of some experiments on their structure and functions, which lead to a new arrangement of the system. Philos
Trans R Soc 111:398424, 1821.
3. Brown J, Coursaget C, Preul M, Sangvai D: Mercury water and cauterizing
stones: Nicolas Andr and tic douloureux. J Neurosurg 90:977981, 1999.
4. Cole CD, Liu JK, Appelbaum RI: Historical perspectives on the diagnosis
and treatment of trigeminal neuralgia. Neurosurg Focus 18:E4, 2005.
5. Dandy WE: Concerning the cause of trigeminal neuralgia. Am J Surg
24:447455, 1934.
6. Dandy WE: Lesions of the cranial nerves. Lewis Pract Surg 12:167202, 1954.
7. Eller JL, Raslan AM, Burchiel KJ: Trigeminal neuralgia: Definition and classification. Neurosurg Focus 18:E3, 2005.
8. Fields WS, Lemak NA: Trigeminal neuralgia: Historical background, etiology
and treatment. BNI Q 3:4756, Spring 1987.
9. Fothergill J: On a painful affliction of the face. Med Observ Inquiries 5:129
142, 1773.
10. Hunter J: Nervous pain in the jaw, in Hunter J: The Natural History of the
Teeth: Explaining Their Structure, Use, Formation, Growth and Diseases. Part II.
London, J. Johnson, 1778, ed 2, pp 6163.

VOLUME 64 | NUMBER 6 | JUNE 2009 | 1185

EBOLI ET AL.

11. Hutchinson J: Epileptiform neuralgia: Its course and symptoms, in On Facial


Neuralgia and Its Treatment. New York, William Wood, 1919, pp 4548.
12. Lewy FH: The first authentic case of major trigeminal neuralgia and some
comments on the history of this disease. Ann Med Hist 10:247250, 1938.
13. Locke J: Letters to Dr. Mapletoft: Letter VII, Paris, 9th August 1677; Letters IX,
X, Paris, 4th December 1677. The European Magazine, February 1789, pp 8990,
March 1789, pp 185186.
14. Locke J: Letter to Dr. Mapletoft, in Stookey B, Ransohoff J: Trigeminal Neuralgia:
Its History and Treatment. Springfield, Charles C. Thomas, 1959, pp 810.
15. Pearce JM: John Locke and the trigeminal neuralgia of the Countess of
Northumberland, in Fragments of Neurological History. London, Imperial
College Press, 2003, pp 280283.
16. Pearce JM: Migraine. Eur Neurol 53:109110, 2005.
17. Ropper A, Brown R: Adams and Victors Principles of Neurology. New York,
McGraw-Hill Professional, 2005, ed 8, pp 161167, 11781190.
18. Rose FC: John Fothergill, in Rose FC (ed): A Short History of Neurology: The
British Contribution 16601910. Oxford, Butterworth-Heinemann, 1999, pp
8892.
19. Rowland L: Cranial and peripheral nerve lesions, in Rowland L (ed): Merritts
Neurology. Baltimore, Lippincott Williams & Wilkins, 2005, ed 11, pp 527529.
20. Stone J, Goodrich JT, Cybulski GR: John Hunters contribution to neuroscience, in Whitaker H, Smith CUM, Finger S (eds): Brain, Mind and Medicine:
Essays in Eighteenth Century Neuroscience. New York, Springer, 2007, pp 6784.
21. Stookey B, Ransohoff J: Historical background of the trigeminal nerve and
trigeminal neuralgia, in Trigeminal Neuralgia: Its History and Treatment.
Springfield, IL, Charles C Thomas Publisher, 1959, pp 332.
22. Trousseau A: Treatise on therapeutics. Clin Med Htel Dieu 2:156, 1885.

COMMENTS

his review of the history of trigeminal neuralgia (TN) reminds us of


the benefit of careful, unbiased observation and reporting of medical phenomena and the unique role that a neurosurgeon may have as
a result of his/her ability to see not only the clinical presentation of a
disease but also the operative anatomy in a living patient.
Ronald Brisman
New York, New York

various forms of pain and came so clearly to conclusion that TN was its
own unique entity. The extraction of teeth to cure this disorder clearly
did not work; the description was provided more than 230 years ago,
yet this error in treatment still persists. The authors have provided us
with a wonderful and most insightful article on this historical disorder.
James T. Goodrich
Bronx, New York

he authors recount and attempt to reconstruct the historical aspects


of TN and our current understanding of this disorder. They make
the interesting observation that the Persian scholar Ibn Sina may be
wrongly credited with a description of this disorder, owing in large
part to mistranslation, from the original Arabic text, of a word that
was thought to signify pain, but which more likely means paralysis.
They recount observations from the British and French schools in the
17th century as the landmark contributions to the modern notions
underlying TN. The contributions of John Hunter, an 18th century
anatomist and surgeon, to the understanding of the pathophysiology of
TN are particularly interesting, since previous reviews have not
acknowledged his work.
Curiously, the observations of Ismail Al-Jurjani are not mentioned in
this article. This Persian physician, a protg of Ibn Sina, likely did
describe TN in the following passage of his book, Zakhireh-i-Khwarazmshah (Thesaurus of the Shah of Khwarazm): There is a type of pain which
affects the teeth on one side and the whole of the jaw on the side which
is painful (1). Although it is, of course, impossible to know with certainty that he was referring to TN, Jurjani was prescient in his explanation: The cause of spasm and anxiety is the proximity of the artery to
the nerve (1).
This article makes for a concise and interesting narrative of our understanding of TN throughout history. It is humbling to realize that the
modern concepts of pathophysiology of this disorder are not vastly different from observations and speculations that are nearly 1000 years old.
Oren Sagher
Ann Arbor, Michigan

N is clearly a miserable disorder to have, and it has only been


within the past 30 years that we have had any form of reasonable
method, either surgical or medical, to treat it. What a delight it was to
read of this disorders most interesting historical background. Starting
with the ancients, Eboli et al. have reviewed early writings, looking for
documentation of TN. Although I would have thought that principals
like Hippocrates and Galen were likely to have written on the subject,
it is not clear that they were aware of this disorder.
The authors have provided a notable service to the historical community in correcting errors in the literature dealing with Avicenna and
his supposed description of TN; the authors point out that he was
likely describing facial paralysis and not facial pain. The first clear
description appears to be that by a physician who suffered from the
disorder, Johannes Laurentis Bausch, who was so debilitated by the disorder that he died from a stroke.
In treating patients with TN, it is very common to see them after
multiple tooth extractions and root canals, all with no relief of the pain.
Therefore, it was interesting to read John Lockes 1677 report of seeing
a noblewoman treated in Paris with extractions. Locke provided a clear
description of this painful disorder. Unfortunately for this lady, the
treatment was purginga treatment that clearly would not have any
benefit, except for a placebo effect.
The authors present a number of historical vignettes on TN. One I
particularly enjoyed concerned John Hunter. It was just amazing to
me to see the clarity of thought in Hunters mind as he sorted out the

1186 | VOLUME 64 | NUMBER 6 | JUNE 2009

1. Ameli NO: Avicenna and trigeminal neuralgia. J Neurol Sci 2:105107, 1965.

n this brief but entertaining review, Eboli et al. trace the history of
TN through the ages, as the signs and symptoms that characterize
the disease emerged from increasingly detailed descriptions. One
lesser-known contribution to this narrative is that of the famous
Scottish surgeon John Hunter, who described pain in the jaw that
was not related to the teeth. Although Hunters cursory description of
the disorder falls far short of Fothergills, he did state in his 1778
treatise that Neuralgia of the fifth pair of nerves may arise from
teeth that are perfectly sound (1), thus making the connection
between this mysterious pain syndrome and the fifth cranial nerve.
Illustrative of the times, Hunters treatment recommendations were at
least far more benign than carbamazepine, radiofrequency lesioning,
or microvascular decompression: Sea bathing has been in some cases
of singular service (1).
Jaimie M. Henderson
Stanford, California

1. Hunter J: The Natural History of the Teeth: Explaining Their Structure, Use,
Formation, Growth and Diseases. London, J. Johnson, 1778.

www.neurosurgery-online.com

HISTORICAL CHARACTERIZATION OF TRIGEMINAL NEURALGIA

he authors provide a concise and informative review of the long


history of this very important neurological malady. The fact that
this condition is diagnosed by the clinical history allowed TN to be recognized in ancient times, but it is interesting to note that it was not well
described as a separate clinical syndrome until a few hundred years
ago. It is also very appropriate to note Professor Walter Dandys seminal observation of the vascular compression etiology of TN. It is helpful for modern neurosurgeons and neurologists to know that surgery
for TN was prominent at the origins of modern neurosurgery, even at
a time when the mortality of cranial operations was quite high. This is
a testament to the misery provoked by this condition, as well as the
success of trigeminal nerve transection/ablation (and subsequent vascular decompression operations).
Robert R. Goodman
New York, New York

his article deals with TN, one of the neurological diseases that,
although benign, have challenged the quality of life of patients
since ancient times. It suggests a new classification of the different
types and pathology of facial pain, in which TN is included.
This concept is very important in a time of evidence-based medicine.
Indeed, the comparison of different therapeutic methods and techniques
is possible only if the clinical picture of a disease is recognized and classified, avoiding the so-called apples and oranges comparison.

The historical report of the descriptions, by various authors, of this


disease is quite interesting. It gives an idea of the difficulty, during the
Enlightenment period, of accepting the concept that pain could arise
from a structure that is different from the one in which the pain is
referred (e.g., the teeth instead of the nerve in TN). Nevertheless, the
proposed classification does not consider the possibility of a double
pathologyagain, this is an event contrary of our positivistic and simplistic approach to disease. Our experience with typical TN and multiple sclerosis (1) is witness to that concept: more than 50% of patients
were cured with a technique that the magnetic resonance imaging
rationale suggested should be avoided.
The authors have reported on the historical contributions of outstanding surgeons of the past. They were deprived of modern imaging
studies but guided by clinical expertise, i.e., the rigorous analysis of
symptoms and anatomopathological knowledge.
Giovanni Broggi
Milan, Italy

1. Broggi G, Ferroli P, Franzini A, Servello D, Dones I: Microvascular decompression for trigeminal neuralgia: Comments on a series of 250 cases, including 10
patients with multiple sclerosis. J Neurol Neurosurg Psychiatry 68:5964,
2000.

Mouse brain cryosection. Axons were labeled with Vybrant DiI cell-labeling solution (Cat. no. V22885). Neuron cell bodies were
stained with NeuroTrace 500/525 green fluorescent Nissl stain (Cat. no. N21480). Nuclei were stained with DAPI (Cat. no. D1306,
D3571, D21490). Copyright 2009 Life Technologies Corporation. See Khalessi et al., pp 10151028.

NEUROSURGERY

VOLUME 64 | NUMBER 6 | JUNE 2009 | 1187

Vous aimerez peut-être aussi