Tourette Syndrome

A Practical Guide for Teachers, Parents and Carers

Amber Carroll, Mary Robertson

• Publisher: David Fulton Publishers
• Year: 2013
• ISBN:
  9781135073343
• Edition: 1
• Topic: Education
• Subtopic: Education General

This handbook provides the knowledge and information required to equip teachers and learning support assistants with the understanding and skills needed when working with pupils with Tourette syndrome. Clinical descriptions and medical treatments are discussed and advice on diagnosis, identification and assessment in the classroom is given. Responding to the learning, emotional and behavioural difficulties pupils may experience, the authors provide multi-disciplinary strategies for application within a school.

Chapter 1

Tics and Tourette Syndrome: An Introduction

Tourette Syndrome is a model neuropsychiatric disorder that seems tantalizing in its simplicity . . . [but] is a very complex disorder, [which] is not just a disorder of tics: it is a disorder of sensations, and it has an internal life invisible to the external world.

(Swerdlow 1999)

Definitions of tics

The main symptoms of TS are tics. A tic is a sudden, rapid, recurrent, purposeless, non-rhythmic, involuntary motor movement (motor tic) or sound (vocal, phonic tic). Characteristically, tics are reduced during sleep. Tics may be simple or complex and include many examples, as can be seen in Table 1.

Table 1: Simple and complex tics

Common simple motor tics	Simple vocal tics
excessive eye blinking eye rolling squinting nose twitching mouth opening facial grimacing (pulling faces) head nodding (backwards or forwards) flicking of the hair out of the eyes (as if the hair/fringe is too long) inappropriate rapid tongue protrusion shoulder shrugging neck twisting or stretching both upper and lower limb jerking	repetitive throat clearing grunting sniffing snorting coughing Complex vocal tics inappropriate fluctuations in the pitch of the voice muttering under one’s breath saying inappropriate words making the sounds of certain animals, such as the quack of a duck or noise of a pig coprolalia (inappropraite involuntary swearing) echolalia (copying what others say)
Complex motor tics facial gestures (such as inappropriate smiling) jumping up and down licking things smelling things squatting abnormalities of gait (such as twirling and retracing steps) feeling compelled to touch things (other people, objects, or dangerous or ‘forbidden’ objects)

How Common are tics?

Childhood tics are common and have been reported to occur in approximately four per cent to 18 per cent of youngsters, with many studies finding tics in about 10 per cent of children. In nearly all studies, tics were more commonly found in boys than in girls (Robertson and Stern 2000). Tics have a variety of causes, but only some of these are causally (aetiologically) related to TS.

At least two studies examining tics in children have involved teachers in the investigations. Thus, Fallon and Schwab-Stone (1992) surveyed teachers in Eastern Connecticut in the USA, who identified 10 per cent of children between the ages of six and 12 years as having tic behaviours. Mason et al. (1998) studied 13–14-year-old pupils in a mainstream secondary school in west Essex, UK for tics and TS. Questionnaires completed by parents, teachers and pupils, as well as classroom observations and interviews, undertaken by a psychologist, were employed to identify tics; 18 per cent were diagnosed as possibly having tics.

In one study investigating the incidence of tics in a population with severe learning difficulties between the ages of seven and 86 years, in the USA, tics were reported in 16.6 per cent of individuals (Long et al. 1998). In another study undertaken in pupils with special educational needs (SEN) in a single school district in California USA, tics were found in 28 per cent (Comings et al. 1990).

Tics are found in a substantial number of children, with 10 per cent being the most common figure in studies. It appears that tics may well also be more common in people with SEN, or in people with learning difficulties, although so far relatively few studies have been carried out.

How Common is Tourette Syndrome?

Motor tics are common in children, occurring in about 10 per cent of children. Many of these children will have transient tic disorder or chronic motor tic disorder (see later), but some may well have TS.

Prior to the 1980s TS (in which both motor and vocal tics must be present, although not necessarily concurrently) was thought to be very rare indeed. In the early medical and scientific literature for many years, only isolated case reports were documented. Since then the generally accepted prevalence figure for TS has been between 5 per 10,000 (Bruun 1984) and 10 per 10,000 (Costello et al. 1996); i.e. no fewer than 25,000 to 50,000 individuals in the United Kingdom. In a well known International Registry published in 1973, only 174 TS cases were documented in the USA and only 53 in the UK (Abuzzahab and Anderson 1973). For reviews of early epidemiological studies of TS see Robertson (1989; 1994; 2000). Recent investigations have, however, indicated that TS may well be much more common than was once previously thought.

The following studies all investigated pupils at schools in both the USA and the UK. Some of the studies were conducted in mainstream schools, while others were undertaken in schools for children with SEN.

Two relatively recent studies in mainstream schools (one in the UK and one in the USA) found very similar results for the frequency of TS. The UK study was undertaken in a secondary school in west Essex. All pupils aged 13 to 14 years were studied and revealed a high frequency. Questionnaires as well as classroom observations and interviews were employed to identify TS. Five out of 166 pupils (2.9 per cent) satisfied criteria for definite/probable TS. Of importance is that all individuals identified had mild TS symptoms (Mason et al. 1998).

In another school study in Monroe County, New York, USA, 3 per cent of the 35 regular school pupils had TS (Kurlan et al. 1994). These studies could be criticised in that they both employed a small number of children (166 and 35 respectively), and were essentially pilot studies. However, they do make the point that TS is probably more common in school children than was once thought.

In studies of children with SEN, however, the prevalence of TS has been demonstrated to be substantially higher in three separate studies, two in the USA and one in the UK. In the first, already mentioned study in a single Californian school district, over 3000 pupils referred for psycho-educational assessment from three schools were studied. It was estimated that 12 per cent of all pupils in special education classes had TS (Comings et al. 1990). Another study examined over 30 children from special education classes in Monroe County, New York. Of the students with SEN, 26 per cent had definite or probable tics; about one-third with tics met criteria for TS (Kurlan et al. 1994). The third study was from west Essex in the UK and included over 80 pupils. Several groups of children were studied. These included pupils from a residential school for emotional and behavioural difficulties (EBD), children from a residential school for individuals with learning difficulties, ‘problem’ children and ‘normal’ children who were all pupils at a mainstream school. Of the pupils with EBD two-thirds were judged to have tics, compared to one quarter of pupils with learning difficulties, only 6 per cent of the ‘problem’ children, and none of the ‘normal’ mainstream children; the majority were judged to have TS (Eapen et al. 1997). Full details of these studies are described in Robertson (2000).

There have been several documentations from the USA suggesting an association between TS and autism. Recently in the UK, there have been two studies suggesting that TS is found frequently in youngsters with autism spectrum disorders (autism and Asperger’s Syndrome). In a pilot study, TS was found to occur in eight per cent of 37 youngsters in the pilot study (Baron-Cohen et al. 1999a), while in a subsequent large scale study, TS was found in six per cent of 447 pupils (Baron-Cohen et al. 1999b).

All of this indicates that TS is now recognised as probably being more common than was previously thought, and that it may occur more often in children who have SEN, learning difficulties or disabilities and autistic spectrum disorders.

Where are Individuals with Tourette Syndrome found?

TS is found in all cultures and most countries. It occurs three to four times more commonly in boys than in girls. TS is found in all social classes, although some studies have suggested that individuals with TS may well underachieve socially and, for instance, not attain their parents’ social status and/or educational level. In a personal survey of many TS specialists worldwide (Robertson 1996), the only part of the world where there were no typical TS cases was Sub-Saharan Africa. There may be many reasons for this, including non-recognition and perceived relative unimportance of tics when many children are suffering from malnutrition and infectious diseases, but there may also be genetic factors, as some neurological disorders are not encountered in Sub-Saharan Africa. The symptoms of TS are similar irrespective of the country of origin.

A recent study by Freeman et al. (2000) has documented findings from 3,500 TS cases in 22 countries around the world including Argentina, Australia, Austria, Belgium, Brazil, Canada, Denmark, Germany, Hungary, Iceland, Israel, Italy, Japan, The Netherlands, Norway, People’s Republic of China, Poland, South Africa, Sweden, Turkey, the United Kingdom and the United States of America.

The History of Tourette Syndrome

The first clear medical description of TS was made by a French neurologist, Jean Marc Gaspard Itard, in 1825, when he reported the case of a French noblewoman, the now famous Marquise de Dampierre. She developed motor tics at the age of seven, but later, at the time of her marriage, began to use swear words, which made her socially unacceptable in her circle, and she therefore had to live the rest of her life as a recluse. Dr Gilles de la Tourette subsequently in 1885, described nine cases of the disorder which went on to earn him eponymous fame.

The first reported case of TS in the UK may possibly have been Mary Hall of Gadsden, who was reported in 1663 by William Drage (Lees et al. 1984).

The history of TS is thoroughly covered by Kushner (1999) who describes the early years of TS in France, the psychological/ psychoanalytical phase of causation, the ‘infectious era’, and finally on to modern times with the acknowledged clinical similarities and biological nature of the disorder.

TS is now well established as a medical condition and hundreds of publications about the various aspects of TS are published in the medical and scientific literature every year.

Gilles de la Tourette

Dr Georges Albert Edouard Brutus Gilles de la Tourette (1857–1904) was a French neuropsychiatrist who worked at the famous Paris hospital, the Salpetrière. His life has been thoroughly documented by Lees (1986). Dr Gilles de la Tourette described nine cases of TS and highlighted the triad of multiple tics, echolalia (repeating sounds or words of other people) and coprolalia (the involuntary uttering of obscenities or blasphemous words), and which he suggested formed a distinct entity among movement disorders. Dr Gilles de la Tourette was famous for his study of hysteria, the medico-legal aspects of hypnotism, and his interest in neuropsychiatric therapeutics. In 1886 he obtained his doctoral thesis for his use of the method of using footprints to diagnose nervous disease! He also used the notorious ‘suspension therapies’ which were successful in the treatment of tabes dorsalis (a complication of syphilis), and he invented a vibratory helmet for use in neuralgia (nerve pain) and vertigo (the sensation of dizziness). He was eventually shot by a deluded and mentally ill patient, made a full recovery, but ironically died of general paralysis of the insane, also a complication of syphilis, in 1904 in Switzerland.

The Causes of Tourette Syndrome

In 1980, Bliss, a TS sufferer, wrote: ‘I have been stalking this thing (TS) for over 35 years with a single minded determination to find something that would give me a clue, a direction, to the meaning of the problem’.

In the last two decades the important causes of TS have become well known as medical research has advanced and they include genetic influences, environmental influence and infections.

Genetics

By far the majority of TS cases are caused by genetic influences, and these may also be called primary or idiopathic TS. Although it is now generally agreed that TS is a genetically determined disorder, the precise mechanism of inheritance is as yet undetermined. The genetic background of TS has been recently reviewed by Robertson (2000). Many studies have suggested autosomal dominant inheritance (meaning that if a particular person had TS, each son/daughter would have a fifty-fifty chance of inheriting the gene). Many authorities now believe that an individual may inherit a vulnerabil...

Table of contents

• Cover
• Title Page
• Copyright
• Contents
• Acknowledgements
• Abbreviations Used in This Book
• Introduction
• 1. Tics and Tourette Syndrome: An Introduction
• 2. Clinical Characteristics of Tourette Syndrome
• 3. Co-morbid Conditions
• 4. Differential Diagnosis
• 5. Management and Treatment of Tourette Syndrome
• 6. Developing and Valuing the Individual
• 7. Forming Good Relationships
• 8. Focusing on Positive Behaviour
• 9. Ensuring Inclusion is a Process and not a State
• Appendix A: Tourette Syndrome in Public Life and in Literature
• Appendix B: Tourette Syndrome Fact Sheet
• Appendix C: Useful Addresses and Contact Numbers
• References
• Index