A peculiar condition: A history of the Jumping Frenchmen Syndrome in scientific and popular accounts.

In 1878, Dr. George Beard reported to other neurologists that in Maine there existed French-Canadian woodsmen who jumped when excited. Beard observed the phenomenon firsthand and his subsequent reports attracted the attention of Georges Gilles de la Tourette in France and other neurologists worldwide for a couple of decades. During the second half of the twentieth century, interest in the jumpers revived among neurologists, as some came forward with similar observations in different parts of Canada and the United States. This article compares and contrasts the scientific reports of the jumping syndrome with those of the popular press and highlights what they revealed about the perceived status of French-Canadian descendants.

Tourette's syndrome and its borderland.

The Gilles de la Tourette syndrome (or Tourette's syndrome) has a prevalence of 1% of children with a wide range of severity and associated comorbidities. The last 20 years have seen advances in the understanding of the syndrome's complex genetics and underlying neurobiology. Investigation with imaging and neurophysiology techniques indicate it is a neurodevelopmental condition with dysfunction of basal ganglia-cortical interactions, which are now also being studied in animal models. There is also increasing evidence for treatments although it often remains difficult to manage. First-line options include neuroleptics, other drugs and specialised behavioural treatments. Deep brain stimulation is an evolving field, not yet fully established. This review focuses on the phenomenology of tics, how to assess and manage the syndrome, and uses examples of atypical cases to explore the characteristics and limits of its clinical spectrum.

[Did Mozart Suffer from Gilles de la Tourette Syndrome?] / ¿Mozart padeció síndrome de Gilles de la Tourette?

The personal and private lives of great men and women in history, like writers, painters and musicians, have been the subject of great interest for many years. A clear example of this is the vast scrutiny is cast over the famous composer, Wolfgang Amadeus Mozart. What may have started as curiosity, rapidly evolved into extensive research, as the answers about the musician's legendary talent may lie in the details of his life (his childhood, his relationships, his quirks and his mannerisms). It is usually up to historians, anthropologists or philosophers to delve into the pages of old books, trying to grasp answers and clues. However, for some time, Physicians have sought their own part in solving the puzzle. The long told hypothesis regarding Mozart's diagnosis of Gilles de la Tourette syndrome will be examined. Could all of the peculiarities and oddities of the genius be caused by a neurological disorder? Or was this musical genius just an eccentric brilliant man?.

El trastorno de Tourette a lo largo de la historia / Tourette’s disorder in history

El término trastorno de Tourette emerge en el campo de la psiquiatría con la aparición de la tercera edición del Manual Diagnóstico y Estadístico de Trastornos Mentales (DSM-III) en el año 1980. Sin embargo, el hecho de que se consensuaran sus criterios diagnósticos por el grupo de expertos responsable de la elaboración de dicha versión del DSM no implica que previamente no existieran múltiples referencias a su sintomatología. El objetivo de este trabajo es recoger aportaciones de la historia de la neurología y de la psiquiatría que permitan comprender cómo se ha ido configurando el concepto operativo de trastorno de Tourette tal como es aceptado en la actualidad por una gran parte de la comunidad científica. En la última década, merced a los estudios de asociación de todo el genoma, se han producido importantes avances en la identificación de variantes comunes y variantes raras implicadas en la etiología del trastorno de Tourette. La conclusión más relevante que se desprende de esta revisión es poner al descubierto la crisis del modelo categórico kraepeliniano del trastorno (AU)

The term Tourette’s disorder emerged in the field of psychiatry with the appearance of the third edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-III) in 1980. However, the fact that some diagnostic criteria were agreed by the expert group responsible for the development of this version of the DSM diagnostic criteria does not mean that multiple references to the symptoms of the disorder did not previously exist. The aim of this review is to collect contributions from the history of neurology and psychiatry which allow us to understand how the operating concept of Tourette’s disorder has been progressively shaped as it is currently accepted by the scientific community. In the last decade, thanks to studies of genome association, major advances have arisen in identifying common variants and rare variants involved in the etiology of Tourette’s disorder. The most important conclusion of this review is the disclosure of the crisis of the Kraepelinian categorical model of the disorder (AU)

Tourette's syndrome in famous musicians / A síndrome de Tourette em músicos famosos

Tourette's syndrome (TS) is defined as a disorder characterized by multiple motor tics and at least one vocal tic that have lasted for not less than one year. It is a relatively complex neurobehavioral disorder, in which patients may present with coexistent attention deficit hyperactivity disorder, obsessive-compulsive disorder or other behavioral comorbidities. The musical genius Wolfgang Amadeus Mozart (1756-1791) and the rock star Kurt Cobain (1967-1994) may both have suffered from TS, and some contemporary musicians have had their clinical condition confirmed as TS. Our hypothetical diagnosis of TS in Mozart and Cobain is based on the presence of tics and psychiatric comorbidities. In contemporary musicians, such as Michael Wolff, Nick Van Bloss and James Durbin, TS has often only been diagnosed after a considerable delay. This delay in diagnosis and the controversies surrounding the clinical case of Mozart show how difficult a confirmatory diagnosis of this complex disease is.

A síndrome de Tourette (TS) é definida como uma desordem caracterizada por múltiplos tiques motores e pelo menos um tique vocal com duração de ao menos um ano. TS é um distúrbio neuro-comportamental relativamente complexo, em que os pacientes teriam coexistente transtorno de déficit de atenção e hiperatividade, transtorno obsessivo-compulsivo, distúrbio de comportamento ou outras co-morbidades. Talvez sejam casos de TS o do gênio musical Wolfgang Amadeus Mozart (1756-1791) e da estrela do rock, Kurt Cobain (1967-1994). Alguns músicos contemporâneos tiveram a sua condição clínica confirmada como TS. Em conclusão, os diagnósticos hipotéticos de TS nos casos de Mozart e Cobain podem ter embasamento na possibilidade de tiques e comorbidades psiquiátricas. Observou-se um atraso de diagnóstico de TS em músicos contemporâneos (Michael Wolff, Nick Van Bloss, James Durbin). Essa situação e as controvérsias sobre a clínica no caso de Mozart mostram a dificuldade para confirmação do diagnóstico dessa complexa doença.

Tourette's syndrome in famous musicians.

Tourette's syndrome (TS) is defined as a disorder characterized by multiple motor tics and at least one vocal tic that have lasted for not less than one year. It is a relatively complex neurobehavioral disorder, in which patients may present with coexistent attention deficit hyperactivity disorder, obsessive-compulsive disorder or other behavioral comorbidities. The musical genius Wolfgang Amadeus Mozart (1756-1791) and the rock star Kurt Cobain (1967-1994) may both have suffered from TS, and some contemporary musicians have had their clinical condition confirmed as TS. Our hypothetical diagnosis of TS in Mozart and Cobain is based on the presence of tics and psychiatric comorbidities. In contemporary musicians, such as Michael Wolff, Nick Van Bloss and James Durbin, TS has often only been diagnosed after a considerable delay. This delay in diagnosis and the controversies surrounding the clinical case of Mozart show how difficult a confirmatory diagnosis of this complex disease is.

Mozart at play: the limitations of attributing the etiology of genius to tourette syndrome and mental illness.

There has been a persistent attempt to explain Mozart's talent as connected to physical and mental illness. While Mozart's musical compositions and performances were often acclaimed for their "taste," the composer's personal behavior sometimes astonished those who witnessed "blödeln" or wild horseplay, practical joking, and scatological humor. Most recently, Mozart's eccentric behavior has been attributed to Gilles de la Tourette syndrome. This chapter investigates the evidence for these retrospective diagnoses and reassesses this evidence by paying particular attention to the milieu in which Mozart lived. We argue that Mozart's putative pathological behavior was a manifestation of his resilience in face of multiple adversities and was deeply rooted in his sense of play. Our hypothesis is that play, rather than neuropsychiatric disease, was essential to the operation of his genius.

Did Mozart suffer from Asperger syndrome?

The most reliable biographies of Mozart highlight elements that are compatible with current diagnostic criteria for Asperger syndrome including qualitative impairment in social interaction and stereotyped and repetitive motor mannerisms. Furthermore, numerous features are documented including difficulty in communicating his emotional state and in inferring the mental state of his interlocutors, motor clumsiness, specific skills and genius, left-handedness, special sense of humour, physical developmental abnormalities, bizarre thinking, overvalued ideas and delusions.

Do features of Mozart's letter-writing style indicate the presence of a neuropsychiatric disorder? Controversies about the Bäsle letters.

In recent decades, several scientific publications have come to the conclusion that Wolfgang Amadeus Mozart might have had a Tourette syndrome. Other papers, however, have questioned this hypothetical diagnosis. The evidence for this diagnosis was mostly based on the so-called Bäsle letters, letters that Mozart wrote to his cousin when aged around 20 years. The letters have common stylistic characteristics such as frequent mention of erotic topics and, in particular, intensive use of scatological terms. However, these characteristics cannot be interpreted as clearly indicating a Tourette syndrome but may rather be related to psychosocial and cultural aspects of that time. There is little evidence for a Tourette syndrome from other sources, such as reports of behavioural abnormalities, and the evidence is not convincing.

[Tics and Tourette syndrome in literature, cinema and television]. / Los tics y el sindrome de Tourette en la literatura, el cine y la television.

INTRODUCTION: Different neurological diseases have often been portrayed in literature, cinema and television. Tics and Tourette syndrome, for example, are commonly represented from different perspectives, which are sometimes very realistic but in some cases are used for more dramatic purposes or to make a character look ridiculous. One of the main effects of these inadequate views is to further stigmatise those who suffer these movement disorders. AIMS: To review the way tics and Tourette syndrome have been portrayed in certain literary works, films and television. DEVELOPMENT: Tics are rapid, stereotypic, involuntary, recurring, non-purposeful movements of the skeletal and pharyngeal-laryngeal muscles. In Gilles de la Tourette syndrome a number of tics are associated to involuntary vocalisations (echolalia, coprolalia). They begin in childhood and are usually associated to obsessive-compulsive behaviours. These disorders have appeared in literature in works such as Little Dorrit, Angel Guerra, La torre de los siete jorobados or Motherless Brooklyn. Film-makers have also shown an interest in tics and Tourette syndrome and they have been portrayed in films such as Young and Innocent, The Tic Code or Matchstick Men. Likewise, a number of television series also contain characters with these disorders, including Shameless, Ally McBeal, Quincy, M.E. or L.A. Law. CONCLUSIONS: Tics and Tourette syndrome have frequently been portrayed in literature, cinema and television, sometimes in a very realistic manner. In other cases, however, the way they are dealt with has only helped to create false beliefs and stereotyped images of the disorders.

TITLE: Los tics y el sindrome de Tourette en la literatura, el cine y la television.Introduccion. La literatura, el cine y la television han mostrado con frecuencia distintas enfermedades neurologicas, y entre ellas los tics y el sindrome de Tourette estan ampliamente representados con distintos enfoques, en ocasiones de forma muy realista, en otros casos para dar un mayor dramatismo o para ridiculizar a un personaje. Estas visiones inadecuadas contribuyen a estigmatizar a las personas que presentan estos trastornos del movimiento. Objetivo. Realizar una revision de la imagen que se ha ofrecido de los tics y del sindrome de Tourette en algunas obras literarias, el cine y la television. Desarrollo. Los tics son movimientos rapidos, estereotipados, involuntarios, recurrentes y no propositivos de la musculatura esqueletica y faringolaringea. El sindrome de Gilles de la Tourette asocia multiples tics a vocalizaciones involuntarias (ecolalia, coprolalia). Aparecen en la infancia y suelen asociarse comportamientos obsesivos compulsivos. Estos trastornos se han mostrado en la literatura en obras como La pequeña Dorrit, Angel Guerra, La torre de los siete jorobados o Huerfanos de Brooklyn. Tambien el cine se ha interesado por los tics y el sindrome de Tourette en peliculas como Inocencia y juventud, El codigo tic o Los impostores. Asimismo, en diversas series de television aparecen personajes con estos trastornos, como Shameless, Ally McBeal, Quincy, medico forense o La ley de Los Angeles. Conclusion. La literatura, el cine y la television han mostrado con frecuencia los tics y el sindrome de Tourette. En ocasiones de forma muy realista, pero en otros casos se han fomentado falsas creencias e imagenes estereotipadas.

A brief history of the European Society for the Study of Tourette Syndrome.

The European Society for the Study of Tourette syndrome (ESSTS) was established in Denmark in 2000 by Mary Robertson and Anne Korsgaard. The aims of the organisation are to foster research activity and raise awareness of Tourette syndrome throughout Europe. The organisation went into abeyance in 2002 but was resurrected in 2007 in Bari, Italy. Since that time ESSTS has grown and prospered. We have established elected officers and a constitution. We have successfully applied for three large scale European research grants and have members throughout the European Union. We have held yearly meetings across Europe including two training schools and we have developed successful alliances with patient support groups. ESSTS has developed and published the first European guidelines on assessment, diagnosis and treatment of Tourette syndrome.

[Gilles de la Tourette: The history of the man and his illness; a medical historical study]. / Gilles de la Tourette: de geschiedenis van de man en zijn ziekte.

BACKGROUND: Description of the life, illness and work of Georges Gilles de la Tourette (1857-1904) and the history of his eponym. AIM: To provide an historical overview of the different views about the disease of Gilles de la Tourette and the Tourette Syndrome. METHOD: Literature review. RESULTS: In 1885 Gilles de la Tourette published a paper on a rare disorder whose symptoms were sudden movements (tics) and the shouting out of obscene language (coprolalia). According to Charcot it was a specific neurological disease which he named after his pupil: la maladie des tics de Gilles de la Tourette. Gilles de la Tourette was potentially successor to Charcot as Professor of Neurology. His bad temper, the first symptoms of neurolues with megalomania, and the fact that the ideas of hysteria had undergone a drastic change, deterred him from taking up the position. After his premature death in 1904 his name was soon forgotten. In the early part of the 20th century the disease of tics was regarded as a psychiatric disorder and from 1920 those suffering from tics were referred for psychoanalysis. After 1970 the disease of Gilles de la Tourette experienced a revival. The American couple Shapiro rediscovered the disorder as a neurological disease and named it the Tourette Syndrome. Thanks to the efforts of an influential American patient organisation Gilles de la Tourette has become famous worldwide with the exception of France. In that country their compatriot is not only less well known, but the belief is still held that it is a neuropsychiatric phenomenon that requires psychotherapeutic treatment. CONCLUSION: The disease of Gilles de la Tourette or the Tourette syndrome has been regarded in different periods either as a neurological or a psychiatric disorder.

Tourette's syndrome: from demonic possession and psychoanalysis to the discovery of gene.

In this paper we make a brief historical review of the hypothesis concerning the etiology of Tourette's syndrome (TS), focusing on varying trends over time: at first, its presumed relation to witchcraft and demonic possessions, followed by the psychoanalytical theory, which attributed TS to a masturbatory equivalent. Then, progressing to modern time, to the immunological theory and finally the advent of genetics and their role in the etiology of TS.

The pathophysiology of echopraxia/echolalia: relevance to Gilles de la Tourette syndrome.

Echopraxia and echolalia are subsets of imitative behavior. They are essential developmental elements in social learning. Their persistence or reemergence after a certain age, though, can be a sign of underlying brain dysfunction. Although echophenomena have been acknowledged as a typical sign in Gilles de la Tourette syndrome (GTS) since its first description, their clinical significance and neural correlates are largely unknown. Here, we review the course of their scientific historical development and focus on their clinical phenomenology and differential diagnosis with a particular view to GTS. The neural basis of echophenomena will also be addressed. © 2012 Movement Disorder Society.

Tourette's syndrome: from demonic possession and psychoanalysis to the discovery of gene / Síndrome de Tourette: da possessão demoníaca e da psicanálise à descoberta dos genes

In this paper we make a brief historical review of the hypothesis concerning the etiology of Tourette's syndrome (TS), focusing on varying trends over time: at first, its presumed relation to witchcraft and demonic possessions, followed by the psychoanalytical theory, which attributed TS to a masturbatory equivalent. Then, progressing to modern time, to the immunological theory and finally the advent of genetics and their role in the etiology of TS.

Os autores fazem uma breve revisão histórica enfatizando alguns importantes fatores relacionados com a síndrome de Tourette (ST) através dos tempos. Primeiramente, a relação da síndrome com possessões demoníacas, bruxaria; depois, a hipótese psicanalítica, que considerava os tiques como um equivalente masturbatório. Na sequência, discute-se a hipótese imunológica; depois, revisa-se a hipótese imunológica; finalmente, o papel dos genes na etiologia da ST.

[Neurobiology, clinical characteristics and therapy in Tourette's syndrome]. / Neurobiologische Grundlagen, Klinik und Therapie des Tourette-Syndroms.

Tourette's syndrome (TS) is characterised by motor and vocal tics. The clinical manifestation during childhood and the frequent remission during early adulthood point to a dysregulation in the maturation of neuronal pathways. As a neurobiological correlate, the cortico-striato-thalamo-cortical circuit is the main focus of research. Two main features of a movement, the speed and direction of a movement, are controlled by the basal ganglia. Both features are dysregulated in TS.  Besides the motor symptoms, patients with TS suffer from comorbidities such as attention deficit hyperactivity syndrome, depression and obsessive compulsive disorders. Tics are modified in frequency and intensity by actions that require a high level of concentration such as reading or by distress, e.g., teasing by peers. The results of structural and functional imaging data support the hypothesis of altered signal transmission in the basal ganglia and a dysfunction in the limbic system. They also point to a complex interaction between cortical motor areas, the anterior cingulum, prefrontal regions and the basal ganglia.

Neurobiology of tourette syndrome: current status and need for further investigation.

Tourette syndrome (TS) is a common, chronic neuropsychiatric disorder characterized by the presence of fluctuating motor and phonic tics. The typical age of onset is ∼5-7 years, and the majority of children improve by their late teens or early adulthood. Affected individuals are at increased risk for the development of various comorbid conditions, such as obsessive-compulsive disorder, attention deficit hyperactivity disorder, school problems, depression, and anxiety. There is no cure for tics, and symptomatic therapy includes behavioral and pharmacological approaches. Evidence supports TS being an inherited disorder; however, the precise genetic abnormality remains unknown. Pathologic involvement of cortico-striatal-thalamo-cortical (CSTC) pathways is supported by neurophysiological, brain imaging, and postmortem studies, but results are often confounded by small numbers, age differences, severity of symptoms, comorbidity, use of pharmacotherapy, and other factors. The primary site of abnormality remains controversial. Although numerous neurotransmitters participate in the transmission of messages through CSTC circuits, a dopaminergic dysfunction is considered a leading candidate. Several animal models have been used to study behaviors similar to tics as well as to pursue potential pathophysiological deficits. TS is a complex disorder with features overlapping a variety of scientific fields. Despite description of this syndrome in the late 19th century, there remain numerous unanswered neurobiological questions.

Could a neurological disease be a part of Mozart's pathography?

As expected, since we recently celebrated the 250th anniversary of birth of Wolfgang Amadeus Mozart, there has been again a renewal of interest in his short but intensive life, as well as in the true reason of his untimely dead. Mozart lived and died in time when the medical knowledge was based mostly on subjective observations, without the established basics of standardized medical terminology and methodology. This leaves a great space for hypothesizing about his health problems, as well as about the cause of his death. The medical academic community attributed to Mozart approximately 150 different medical diagnoses. There is much speculation on the possible causes of Mozart's death: uremia, infection, rheumatic fever, trichinellosis, etc. Recently some authors have raised the question about a possible concomitant neurological disease. According to available records, Mozart has shown some elements of cyclotimic disorder, epilepsy and Gilles de la Tourette syndrome. Furthermore, the finding of a temporal fracture on (allegedly) Mozart's skull, gives a way to speculations about the possibility of a chronic subdural hematoma and its compressive effect on the temporal lobe. Despite numerous theories on Mozart's pathography that also include a concomitant neurological disorder, the medical and history records about Mozart's health status indicate that he probably had suffered from an infective illness, followed most likely by the reactivation of rheumatic fever, which was followed by strong immunologic reaction in the last days of his life. Taking all the above into consideration, it is reasonably to conclude that Mozart's neurological disturbances were caused by the intensity of the infective disease, and not primarily by a neurological disease.