Tourette Syndrome (TS) is a neurological disorder characterised by sudden rapid, involuntary movements called tics, which occur repeatedly.
Symptoms can include bouts of motor and vocal tics and the focus of these tics tends to wax and wane over time. Typically tics increase as a result of stress or tension, and may decrease when relaxed or absorbed in a task. However, symptoms are an individual phenomenon, with people exhibiting many different symptoms, perhaps over the course of their lifetime. Tics are experienced as an irresistible urge (as, for example, in a sneeze) and must eventually be expressed. Many people try and suppress their tics until they can find a secluded spot in which to release them. Even then, the feeling of relief tends to be only momentary.
Two categories of tic have been identified, namely simple and complex tics. The simple type includes eye blinking, head and limb jerking, shoulder shrugging and grimacing (motor tics); plus sniffing, grunting, throat clearing and yelping (vocal tics). Complex motor tics include jumping, smelling, touching rituals, and self-injurious behaviour. Coprolalia (vocalising offensive words and phrases), Echophenomena (repeating a sound) and Echolalia (repeating a word or phrase just heard) constitute complex vocal tics.
The range of tics, or tic-like symptoms that characterise TS is very broad and they can exist in different combinations. For a diagnosis of TS to be made, the onset of symptoms must be before the age of 18 years.
There are a number of additional behaviours thought to be associated with TS, although they are not necessary in order for a diagnosis to be established. Specifically, they include Obsessive Compulsive Disorder (OCD) and Attention Deficit Hyperactivity Disorder (ADHD). In addition, some studies suggest a link between TS and Learning Disabilities (eg reading and writing difficulties, perceptual problems), Difficulties with Impulse Control (eg socially inappropriate acts) and Sleep Disorders (eg frequent awakenings or walking/talking in one’s sleep). However, the evidence supporting such a link is not conclusive at this stage.
In terms of causation, genetic studies show that TS is inherited as a gene or genes, there being a 50% risk of the gene being transmitted with each separate pregnancy.
Gender also influences the expression of the gene, with the incidence of TS being at least 3 to 4 times greater in males than in females. The gene defect is believed to cause an abnormal metabolism of neurotransmitters within the nervous system and as yet there is no known cure for the disorder, only symptomatic relief.
However not everyone with TS experiences symptoms severe enough to require medical attention and most people with tic disorders tend to improve in adulthood.
Tourette Syndrome and OCD
The clustering of OCD and TS within families suggests a common inherited factor, with TS often being complicated by comorbid OCD. The estimated incidence of this ranges from 35% to 50%. The incidence of TS in OCD is lower (5% to 7%), although tics are reported in 20% to 30% of individuals with OCD. A distinction can be made between tic-related and non-tic-related OCD.
The most striking similarity between TS and OCD is that both are characterised by apparently senseless repetitive behaviours. Motor tics in TS are often described as ‘irresistible’, as although they can be delayed, they must ultimately be performed. Once the tic has been performed until it feels ‘just right’, a sense of relief is then experienced. This type of phenomenon also occurs with obsessions and compulsions.
OCD with comorbid tics appears to respond differently to treatment compared with non-tic-related OCD. In addition to anti-obsessional drugs (eg SSRIs such as Fluvoxamine and Paroxetine), individuals with TS seem to respond better to a combination of SSRI and neuroleptic treatment (eg Sulpiride and Pimozide), although there have been no good trials undertaken in this area at all.
Current theory suggests that immunological alterations may have occurred in individuals with OCD, either through haemolytic streptococcal or viral infection during childhood. An antigen has been shown to be stable in different populations and over time, and is present in individuals with childhood OCD, TS, chronic tic disorder and autism. It is possible that the antigen could be linked to the motor component of the various disorders. However, much further research is required in this area, and it is probable there exist multiple causes with the ability to trigger OCD symptoms according to individual vulnerability, with genetically-based theory and exposure to infection being two of the likely contributors.
The history of Tourette Syndrome
 |
| Dr. Gilles de
la Tourette |
The first reported case of Tourette Syndrome in medical literature was in 1825 when Jean Marc Gaspard Itard described the case of the Marquise de Dampierre. The Marquise was a noble woman whose symptoms included coprolalia (the utterance of swear words). She regularly shocked her high society friends by yelling out obscenities during conversations.
In 1885, Dr. George Gilles de la Tourette (1857 - 1904) a French neurologist at l'Hôpital de la Salpêtrière described nine patients with "maladie des tics", citing the Marquise de Dampierre as his primary case example. He described a condition where those affected twitched and jerked uncontrollably. The sufferers also cried out or grunted, or, in the Marquise's case, swore.
Dr. George Gilles de la Tourette (Born Georges Albert Edouard Brutus Gilles de la Tourette) was a student of Jean-Martin Charcot. Charcot was the director of l'Hôpital de la Salpêtrière and one of the most important and influential neurologists of his day.
Most of his subjects were young males. This was probably one of the reasons his "maladie des tics" was so widely accepted. When these symptoms appeared in females, they were considered to be part of the vague condition, hysteria. However, because this malady affected males as well, it was considered a separate medical condition.
At the time of Gilles de la Tourette "maladie des tics", Charcot was on a personal quest to classify groups of neurological symptoms into syndromes. "Maladie des tics" was perfect, and Charcot renamed it 'Gilles de la Tourette illness'.
Gilles de la Tourette also noticed that the illness tended to run in families. The parents or siblings of a patient with a serious case of Tourette would tend to have a mild form of the same condition.
Ironically, Gilles was shot by a deluded woman who was a patient at the famous l'Hôpital de la Salpêtrière hospital.
Tourette syndrome was initially considered to be a psychological one. The observation in the 1960s that certain drugs called neuroleptics were effective in treating TS, refocused attention from a psychological to an organic central nervous system cause.
Very much like OCD, people with Tourette Syndrome aren't psychologically impaired, obstinate, or unintelligent. Most people with the disorder lead normal productive lives, with some of them excelling in their given professions. Those thought to have suffered from TS include Samuel Johnson the lexicographer, and André Malraux, the French author, Mozart, and Manchester United and US goalkeeper Tim Howard also recently admitted to suffering from the condition.
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