Transcription
[Music] Hi, I'm Professor Graeme Austin, and today I'm going to be talking to you about the curious case of the cursing countess and the fascinating history of how Tourette's syndrome got its name. I'm only going to say a little bit about the clinical features of Tourette's syndrome, as there are plenty of other good YouTube videos out there that focus on this. I really want to concentrate on the history.
[Music] Tourette's syndrome is a neurological condition that causes people to make involuntary sounds and movements called tics. Examples of physical tics include blinking, eye rolling, grimacing, shoulder shrugging, jerking of the head or limbs, jumping, twirling, and touching objects or other people. Examples of vocal tics include grunting, throat clearing, whistling, coughing, tongue clicking, animal sounds, saying random words and phrases, repeating a sound, word, or phrase, and swearing. Although swearing is the most well-known symptom of Tourette's syndrome, it is actually quite rare and it only affects around one in ten people with the disorder.
Tourette's syndrome used to be considered a rare condition, but as it has become more well-known by the general population, milder forms of the disorder have been recognized, and we now know that between one and five percent of the population have some kind of tick disorder. The symptoms are usually most severe in childhood, and as I am an adult neuropsychiatrist, I don't see many cases early in my career. However, I saw a boy who used to disguise his vocal tics by shouting out, "Remember, you're a Womble," much to the amusement of his classmates and consternation of his teachers. I'm not sure how global the Wombles were, but they were very popular in the UK in their day, and they even had some hit records, one of which was "Remember You're a Womble."
Now, to the history. Paris, 1885, to be precise. Just a few years before the Eiffel Tower was built. This was the period known as *La Belle Époque*, the literal translation of which is "the beautiful age," and it is the name given to the period in French history from the end of the Franco-Prussian War in 1871 to the last days of peace before the start of the First World War in 1914. For many, it was a golden age characterized by optimism, economic prosperity, technological innovations, and a flourishing of the arts. The 1880s was the age of Renoir, Degas, Seurat, and Toulouse-Lautrec; of the writers Guy de Maupassant, Émile Zola, and Paul Verlaine. It was also an age of crushing poverty for the masses, expanding French colonialism in Africa, the Pacific, and into China, which of course set the seeds for the Vietnam War a hundred years later. It was also an era in which anti-Semitism was common, which led to the Dreyfus Affair, in which an innocent French officer was convicted of treason and sent to the notorious prison on Devil's Island in French Guiana, the same prison, incidentally, that features in one of the greatest escape movies ever made, *Papillon*.
In 1885, Georges Gilles de la Tourette, a neurologist in training at the Hôpital de la Salpêtrière, published a collection of nine case histories of patients with a nervous disorder characterized by involuntary movements with echolalia, which simply means echoing what someone has said, and coprolalia, which literally means talking about poo. Although there have been some partial translations of Gilles de la Tourette's paper before, it wasn't until 1998 that myself and a colleague translated all nine cases into English for the first time.
Gilles de la Tourette was 28 when the paper appeared in 1885. He was the favored pupil of the Salpêtrière's director and foremost neurologist from 19th century France, Jean-Martin Charcot, whose work with hysteria was then at its height. Gilles de la Tourette was interested in movement disorders and had been urged by Charcot to sort out the chaos of chorea. Chorea is a movement disorder with unpredictable involuntary movements that are said to have a dance-like quality, and it is derived from the ancient Greek word for dance, *choreia*. This video from the Wellcome Collection was taken in 1947 and it shows Sydenham's chorea.
In 1884, Gilles de la Tourette translated and reviewed case studies of some bizarre startle reaction syndromes from around the world. These were known as the "jumping Frenchmen of Maine," "latah" from Indonesia, and "miriachit" from Siberia, described by the US Surgeon General and neurologist William Hammond. Gilles de la Tourette's 1885 paper built on this earlier work. The paper was discussed in Charcot's lectures, and Charcot himself sanctioned the use of Gilles de la Tourette's name for the syndrome, although even from within the Salpêtrière itself, there were criticisms of his work. Charcot died in 1893, and his ideas about hysteria soon began to lose favor, and Gilles de la Tourette's reputation as his strongest supporter also suffered.
From early in the 20th century, psychoanalytic thinking dominated the discussion of tics, particularly in America, and patients and their parents were told it was all to do with, you've guessed it, masturbation. In this era, Tourette's syndrome was rarely referred to by its eponym. In the 1960s, Arthur and Elaine Shapiro began successfully treating cases of Gilles de la Tourette syndrome with medication and were very vocal in their criticism of previous approaches to the disorder, commenting on the "fashion in medicine to attribute symptoms and diseases without demonstrable organic pathology to a psychological waste basket diagnosis." This was an obvious dig at psychoanalytic thinking of the time. They were so vocal in their criticisms, in fact, that they couldn't get their paper published in an American journal and they had to turn instead to the *British Journal of Psychiatry*.
Since then, our understanding of the neurobiology of the condition and how to treat it have increased enormously, and thankfully, patients and their families no longer have to endure embarrassing discussions about masturbation.
[Music] Gilles de la Tourette was not the first person to describe a syndrome that now bears his name. In the 18th century, Dr. Samuel Johnson, described by some as the most distinguished man of letters in English history, was noted by his friends to have almost constant tics and gesticulations which startled those who met him for the first time. He also made noises and whistling sounds, repeated sounds and words, and made irregular blowing respiratory noises, and he had a range of bizarre compulsive rituals. It is now widely accepted that he had Tourette's syndrome.
[Music] But back to France. The first case included in Gilles de la Tourette's 1885 paper was actually reported 60 years earlier by Jean-Marc Itard, chief physician at the Paris Institute for the Deaf. Itard had achieved fame earlier in his career as the physician who had tamed and educated the so-called "wild child of Aveyron," a boy who had lived feral in the woods of southern France for several years. The subject of Itard's 1825 paper was a countess, Ernestine Emily Prandra de Germant. This is not a picture of her, as we don't have one, but this is her family home. She was well-known in Paris for her eccentric behavior and coarse language. At her death at the age of 85 in 1884, the press printed examples of her obscenities, meaning well, how can I phrase it more delicately? "Excrement" and "copulating pig," perhaps. In one newspaper, it was said that during high mass at the Church of the Madeleine, it was not uncommon to hear such perfect barking that one might have believed a pack of hounds was present in the church. The countess saw many doctors over her lifetime, and there were frequent references to her unusual behavior in medical journals over the next 60 years, which Gilles de la Tourette acknowledges in his paper. However, it is unlikely that he ever saw her, other than perhaps as a passer-by in public places. She was, after all, a countess.
But what of the man himself? He was born in 1857 in a village near Poitiers into a medical family. He was a precocious, if slightly disruptive, pupil and he entered the medical school in Poitiers early, at the age of 16. He moved to Paris in 1881, the same year as this picture was painted, and presumably he enjoyed the full range of distractions that Paris had to offer. He began working in Charcot's department at the Salpêtrière Hospital in 1884. The Salpêtrière was originally built as a gunpowder factory, saltpeter being a key ingredient for this, but in 1656, Louis XIV ordered that it be converted into a hospice for the poor women of Paris, and by the time of the Revolution in 1789, it housed over 10,000 inmates, a mixture of prostitutes, prisoners, paupers, and patients. Philippe Pinel, a pioneer of humanitarian reforms in the treatment of the mentally ill, worked at the Salpêtrière, and he is often credited with "striking off the chains of patients," although the facts of who did what and when are rather more complex, but that's another story. The Salpêtrière today remains a very busy inner-city hospital and it is the largest in France. It was where Diana, Princess of Wales, was taken after her fatal car crash in 1997.
In the 1880s, Jean-Martin Charcot was at the height of his fame, and his lectures were attended by some of the most famous names in medical history, including a young Austrian neurologist called Sigmund Freud. Charcot has been called the founder of modern neurology, and there are more than a dozen medical conditions named after him. Charcot encouraged his pupils to search for historical examples of the hysterical symptoms they were examining, and this led Gilles de la Tourette to publish an analysis of Jean des Anges, a nun whose obsession with the priest Urbain Grandier led her and 20 other nuns to believe that they were possessed by him. They developed strange hysterical behavior and false pregnancies. The priest was accused of having a pact with the devil written backwards in Latin, and he was burned at the stake for witchcraft in 1634. More recently, this story has been the subject of an Aldous Huxley novel, *The Devils of Loudun*, and an acclaimed, if controversial, Ken Russell film, *The Devils*.
Gilles de la Tourette worked extremely hard, writing, experimenting, and teaching. He introduced suspension therapy to France as a treatment for tabes dorsalis, which we now know to be one of the late manifestations of syphilis. Inspired by Charcot's use of the *photo-trepidon* or vibrating chair for the treatment of Parkinson's disease, Gilles de la Tourette invented a vibratory helmet for facial neuralgia.
Throughout his career, Gilles de la Tourette published on a huge variety of topics, both medical and non-medical. He authored 30 books on over 200 medical papers. He was also a scientific correspondent and drama critic to the literary gazette *La Revue Hebdomadaire*, producing articles on a variety of topics including the so-called human calculator Jacques Inaudi, the music hall, historical figures including his hometown hero Théophile Renaudot and Joanna the Mad Queen of Castile, Joseph-Ignace Guillotin, inventor of the guillotine, criminal cases, characters in plays, hysteria in the German army, which apparently angered Chancellor Bismarck, parasites in army biscuits, and floating smallpox hospitals on the River Thames, which he visited on a brief trip to London in 1887. He married his cousin, and they went on to have four children, the last of whom died in 1979.
In 1893, shortly after the deaths of Charcot and his own son, Gilles de la Tourette was shot in the head by an ex-patient of the Salpêtrière who claimed to have been hypnotized to do so against her will. Some of her claims bore an uncanny resonance to the wild accusations of Sister Jean des Anges about Urbain Grandier. The newspapers reveled in the case, as it seemed to mirror the public and very acrimonious dispute Gilles de la Tourette had with another neurologist about whether an individual could be hypnotized to perform a crime against their will. Fortunately, it was a superficial wound, and the bullet was easily removed. This is a letter to a friend he wrote on the day it happened. He ends it with, "Quel drôle de quoi!" What a strange story!
However, Gilles de la Tourette's health never fully recovered, as he was beginning to show signs of the illness that was to claim his life in just another 11 years: syphilis. Syphilis is a terrible disease. It is now relatively uncommon in the developed world, but there are still seven million new cases per year worldwide. It begins with a primary chancre at the site of first contact. I can remember a lecturer at medical school excitedly describing a chancre of the big toe, which I thought slightly odd at the time, but I don't suppose it would create too much surprise these days. Later, a widespread rash appears. It then goes into a latent phase, which can last for many years. Then the tertiary stage appears. I have shown here a model of a patient with severe gummatous syphilis, as some images of these patients are just too graphic to show. When syphilis affects the brain and nervous system, it is known as neurosyphilis, and it can cause a huge variety of neurological symptoms, such as tabes dorsalis, and psychiatric symptoms. Tell me, how is your memory? 100 memories? Manifestations that it is still known as "the great imitator." At the start of my career, we still routinely tested for syphilis in all new psychiatric patients. The end stage was known as General Paralysis of the Insane, or GPI, and as this video of the admission register for an asylum in England in 1906 shows, it was extremely common. Although syphilis was suspected as the cause of GPI and tabes dorsalis as early as 1857, this issue was still disputed until the spirochete bacteria responsible were found in the brain of a patient in 1917 by Hideyo Noguchi, whose work is still celebrated on the Japanese 1000 yen banknote.
But back to Gilles de la Tourette. He continued to publish extensively, though, including a collection of Charcot's lessons and notes on the treatment of hysteria in a massive three-volume work, and he was appointed a professor of forensic medicine. For the Universal Exhibition of 1900, he had the honor of being appointed chief medical officer, and despite his deteriorating health, he proved an excellent organizer, and he was awarded the Legion d'Honneur. Sadly, after this, his health went into sharp decline. He had periods of melancholia, or depression, he spoke of committing suicide, and he had episodes of manic excitability and delusions of grandeur. He had to resign from his post at the Salpêtrière and he was persuaded by Charcot's son to go to Switzerland to convalesce, away from the prying eyes of the French newspapers. He was told that there was a celebrity patient waiting for him at a hospital in Sierre, but there was no famous patient. It was the start of his own involuntary hospitalization. His mental state and behavior progressively deteriorated. He was unsteady on his feet, he became more and more grandiose and psychotic, his speech was incoherent, and he often suffered from seizures. This rather sad, grainy footage is actually of Friedrich Nietzsche from the same period, but it gives an idea of what the advanced stages of neurosyphilis look like. We don't have any images of Gilles de la Tourette in his final illness, and we don't know what treatments he was offered. Perhaps it was suspension therapy. But he died in May 1904, at the age of 47, with his family, who had stayed with him in Lausanne throughout his illness, by his side. His obituaries were brief and less than effusive, and thereafter he was largely forgotten. There was no mention of him or the syndrome in British textbooks of psychiatry from 1950 and 1969, and only a brief mention in the 1971 French textbook. Fewer than 50 case descriptions of his tick disorder appeared in the medical literature until interest was reawakened in the 1960s.
[Music] Now, however, there is much more interest in the syndrome and its historical background, and more interest in the man, Georges Gilles de la Tourette, whose name will forever be attached to it. That's it for now. I hope you've enjoyed it. If so, please like it and consider subscribing to my channel. As always, I've listed the academic references, and if you'd like a complete list of all of the references and picture credits, please contact me. There are some excellent books on the history of the syndrome, including Howard I. Kushner's *A Cursing Brain*, published in 1999, but for a fantastically detailed account of Georges Gilles de la Tourette, the man himself, it is hard to imagine Olivier Polizzinski's 2019 biography being surpassed.
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