Understanding Tourette Syndrome

A guide to symptoms, management and treatment

Carlotta Zanaboni Dina, Mauro Porta

• Editore: Routledge
• Anno: 2019
• ISBN:
  9780429948213
• Edizione: 1
• Argomento: Medicine
• Categoria: Neurology

Understanding Tourette Syndrome provides accessible, concise, evidence-based guidelines on this neurodevelopmental disorder, offering parents and professionals a deeper scientific understanding of the condition and its consequences. Zanaboni Dina and Porta explore signs, symptoms and treatment of the disease, with the aim of demonstrating to all those involved in the life of a TS child solutions to manage a range of situations from diagnosis to day-to-day life.

Therapies and social intervention, including Habit Reversal Training and Deep Brain Stimulation, are described, allowing caregivers to evaluate the best course of treatment. With a focus on improving quality of life by offering practical recommendations for managing the condition at school and in the family, it places additional emphasis on sibling relationships and the importance of childhood friendship. The authors' expert subject knowledge and extensive experience of working with children and families, makes the topic accessible for any reader, and case studies demonstrate how to apply scientific understanding of the condition to a real-life situation.

This unique guide is essential reading for parents and carers, as well as practitioners in Clinical and Educational Psychology, Counselling, Mental Health, Nursing, Child Welfare, Public Healthcare and those in Education. It will also be of interest to postgraduates studying courses in Psychology, Neurology and Psychiatry.

Chapter 1

A short history of Tourette syndrome^*

Summary

In 1825, Itard first described a Tourette syndrome (TS) case: la Marquise de Dampierre having coprolalia and other behavioural diseases. In the second half of the 19th century, Gilles de la Tourette – a disciple of Charcot at the Salpêtrière Hospital – studied nine patients, giving the name to the syndrome. Between the 19th and the 20th centuries, the organic versus psychological origin of TS was debated. Nowadays, from the USA to Europe, it is considered a neurodevelopmental disease characterised not only by tics, but also by behavioural symptoms. Living with the syndrome has specific sociocultural implications.

Introduction

This chapter illustrates cultural panorama at the times of the syndrome’s birth. TS has a centuries-old history (Figure 1.1).

The syndrome’s name originates from Georges Albert Édouard Brutus Gilles de la Tourette (born 1857 Saint-Gervais-les-Trois-Clochers; died 1904 Lausanne), a French neurologist who lived in a key period for development of medical sciences, especially neurology. He was born in Saint-Gervais into a family of doctors. In 1884, he started to follow the clinical practice of the Father of Neurology, Jean-Martin Charcot at the Salpêtrière Hospital. In 1885, Gilles de la Tourette (GT) published his studies on a bizarre disease in Charcot’s “Archives de neurologie” (transl. “Archives of neurology”). He described nine cases affected by multiple tics, especially involving the face, superiority in the arts, and uncontrollable verbal bursts, including insults and profanities. The first case – the Dampierre Marquise – appeared 60 years earlier in the “Archives générales de médecine” (transl. “General archives of medicine”, 1825) by the well-known doctor, Jean Marc Gaspard Itard. He made an accurate description of the case, and GT chose it as an emblem of the pathology that he called “la maladie de tics” (transl. “the tic disease”). Charcot then named the disease “Gilles de la Tourette syndrome”, in honour of his pupil.

Figure 1.1 Tourette’s timeline.

GT = Gilles de la Tourette.

Credit: Cecilia Spalletti.

* In collaboration with Thomas Spalletti

In 1886, GT graduated; in 1887, he became clinical head and his career grew. In 1893, GT was shot by a patient who was convinced to be in a state of hypnosis; the man tried to get out the state and fired three gunshots. The young neurologist suffered from brain damage, and his studies started to decline. After some years of medico-legal consultations, he became head doctor at Paris 1990’s Expo. Then he contracted syphilis and he recovered in Lausanne, where he died at the age of 46, abandoned by his wife and whole family. GT’s clinical notes on the syndrome are still valid today.

Early descriptions

GT was not the first doctor to be interested in the disease. He put together several clinical signs of this unique syndrome, thanks to other clinicians’ case observations.

Previous descriptions of the disorder belong to different eras. The most ancient dates back to the age of inquisitors: the “Malleus Maleficarum” (transl. “The Witch Hammer”, 1486), a text drawn up by two Dominican monks, Krämer and Sprenger. At that time, Pope Innocenzo VIII enacted a strict intervention against witchcraft, considering it a form of Satanism. In the aforementioned text, some cases are defined as demonic possessions, but many signs are now attributable to TS. One case is about the story of a Bohemian farmer, who went to Rome to ask his son – a priest – to free him from his demonic possession. Each time he stood in front of a church, the devil took possession of his tongue, and the man started to curse God and to swear. In those moments he couldn’t control his words. Finally, a bishop took care of him through prayer, exorcism and by feeding him only bread and water for 50 days; then the man was graced by God and he could go back home.

Demonic manifestation was the best way to interpret this pathology, characterised by violent movements, shouts and lack of control. This case has a positive ending, whereas many others could have ended under torture.

Origin of the word “tic”

“Tic” has an onomatopoeic origin in different languages: tic in French and Italian, tico in Spanish, tic/tik/tug in English, zucken/ziehen/zugen/tucken/ticket/tick in German dialects.

The term was used for the first time in veterinary medicine to describe horses’ pathologic breathing. From 1664, Solleysel, a well-known squire at Versailles, prevented buyers from purchasing horses with tics. The sound “tic” comes from the noise a horse makes when it hits the wood of the stable with its teeth. This happens when a horse is bothered by air in its stomach. Despite not being a contagious disease, horses learn it from each other (see echopraxia in Chapter 3).

La Marquise de Dampierre

La Marquise de Dampierre was a noblewoman, as described by Itard, and 60 years later by GT in his article. This was published a few months after the Marquise’s death, i.e. when she was 85 years old. She was famous not only for being a Marquise, but also for her bizarre behaviour, which caused surprise and rumours.

At the age of 7, she had onset symptoms: severe mouth, face, neck and arm tics. Shouts, insults and obscenity bursts appeared some years later. Because of these problems, the noblewoman spent her life segregated in her palace until her death. Her speech would suddenly be interrupted with shouts, swearing and unexpected words, despite her high intelligence and good manners. When she was with other people, she used to inappropriately describe others, causing embarrassment to all the bystanders. The more she was scandalised by her own explicitness and by the repetition of these vulgarities, the more these words were “forced” onto her tongue, and she couldn’t control herself.

Figure 1.2 Interpretation of Marquise’s symptoms.

GT = Gilles de la Tourette.

Credit: Cecilia Spalletti.

Besides Itard and Gilles de la Tourette, many other clinicians tried to examine the Marquise’s clinical conditions (Porta & Sironi, 2016; Figure 1.2). The point of view of each doctor follows:

1 Itard (1825) interpreted the Marquise’s clinical case under the influence of the philosopher de Condillac, who believed that learning depends on sensorial stimulation. Based on this hypothesis, Itard assumed that a sufferer could overcome their disorder through a “moral treatment”, a re-education training focused on force of will and emotions, offering senses stimulation. Health status depended on the balance of diet, weather conditions, job and lifestyle. Itard supposed that the Marquise was not able to exercise her will on behaviours as she was passively accepting her social role of wife and lady (e.g. her husband decided she wouldn’t be cured). An appropriate intervention would have comprised of increasing her self-esteem. Many doctors at that time hypothesised that the frequent female diseases were the result of women’s alienation forced upon them by their role in a patriarchal culture. This female arrest could be solved by improving women’s life conditions through the development of a higher sensibility (in some cases, it would have been necessary for the patient to live away from her pathogenic home temporarily).

2 Billod (1847) identified the Marquise’s poor will as the reason for expressing those thoughts, which would have normally been suppressed. In contrast to Itard, Billod described the will as an entity, which can’t be treated by a rehabilitation of the senses. Billod believed that a sane intellect can select ideas to express or not to express at all. In the Marquise’s case, this ability would have been altered because her will was being absorbed by other influences.

3 Roth (1850) referred to the Marquise, rejecting both Itard’s and Billod’s interpretation. He supposed that her symptoms were caused by a physiologic cause: a muscular disease, namely chorea. He considered swearing and vocal bursts as “muscular tics of the word and the larynx”.

4 Sandras (1851) defined the Marquise’s symptoms as organic, i.e. being a partial chorea.

5 Ribot (1883) employed the Degeneration Theory to explain the Marquise’s case. This theory was used to interpret many diseases (depression, intellectual disability, sterility, etc.) as consequences of bad moral and social behaviour, along with hereditary features. Alcohol abuse, an unbalanced diet and immoral actions would have disruptive effects on the nervous system, wh...