Living With Tourette's Syndrome (TS)

John Davidson appeared in a BBC documentary in 1989 about his daily battle with Tourette's syndrome. Here, he talks about how he copes with the condition, growing up with Tourette's and building self-confidence.

"John’s Not Mad" was a BBC documentary featuring John as a lonely 16-year-old boy with such severe Tourette's Syndrome(TS) that he was too scared to venture outside and face people.

Viewers saw how his uncontrollable (sometimes foul-mouthed) outbursts and violent body jerks prevented him from leading a normal life. The documentary highlighted a much-misunderstood condition, which affects 300,000 people in the UK.

“The BBC documentary changed my life tremendously due to the public awareness it created,” says John, a community worker from Galashiels, Scotland, and a TS spokesman.

“I felt that people realised I wasn't insane, and that I was a decent kid who unfortunately had this misunderstood condition. I then had the confidence to walk down the street without feeling I was being watched and laughed at. 

“Most importantly, it raised awareness of TS and has encouraged other people to get diagnosed. It's also helped me make many friends.”

John has vocal tics, including coprolalia (unintentionally using bad language). Coprolalia is one of the most well-known aspects of TS but it affects only 10% of sufferers.

TS is a condition of the nervous system, and there's no cure. Symptoms usually appear in childhood. Some symptoms disappear by the age of 18 but the condition stays throughout the person's life.

TS causes involuntary movements and involuntary noises, called tics. Vocal tics can range from coughing or throat-clearing to the involuntary utterance of whole words or phrases. During a tic, a person is fully aware of their actions but can't stop, though they may be able to suppress them temporarily. 

People with TS are of usual intelligence, but may have educational difficulties due to poor concentration. If they're not given appropriate help, they can become frustrated, withdrawn or do poorly at school.

“Growing up with TS was very difficult,” says John. “I believed I was different from everyone else because of the involuntary symptoms. 

“I often felt unloved and unwanted by family and friends. I felt that I was a hindrance to the rest of my family.”

Many people with TS lead normal lives. Others find that the condition, and people's prejudices about it, make it difficult to get jobs or form relationships.

“I was very self-conscious about my condition, especially when all I wanted was to fit in with my peers,” says John. “I was often paranoid about everything and everyone around me. At times I felt very alone.”

'Be open and honest about how you feel in different situations'

John Davidson

Coping mechanisms

Although public acceptance has made John feel much more comfortable in society than he did when he was 15, day-to-day life is still very difficult.

“My self-confidence goes up and down a lot. It tends to increase when I have good friends and lots of support around me, especially from close family,” he says.

“Having constant reassurance helps tremendously, as does getting lots of positive feedback about achievements.”

John generally has fewer tics now than when he was a teenager. But certain social situations, such as going to the supermarket, still make the tics worse.

He says that each person with TS must develop their own ways of coping, with the help of medical experts.

“At school I used to take 10 minutes time out from the class when I felt stressed and ‘ticcy’. Sitting next to a door helped me in the classroom setting as I could easily escape if I needed to,” he says.

“I often sit tapping my fingers and tapping my foot, which helps to alleviate the tics and gives me something to concentrate on. 

"In my current work situation I find it helpful to have somewhere quiet to go and gather myself.” 

John does relaxation exercises at home, attends cognitive therapy and takes medication to lessen the tics. But he knows that Tourette's will always be part of his life.

He says the best and most effective coping mechanism is understanding and patience from others around you.

“Be open and honest about how you feel in different situations. It helps others to understand what you're going through,” he says.

The causes

Tics come and go over time, varying in type, frequency, location, and severity.  The first symptoms usually occur in the head and neck area and may progress to include muscles of the trunk and extremities. Motor tics generally precede the development of vocal tics and simple tics often precede complex tics.  Most patients experience peak tic severity before the mid-teen years with improvement for the majority of patients in the late teen years and early adulthood. Approximately 10-15 percent of those affected have a progressive or disabling course that lasts into adulthood. 

Although the cause of TS is unknown, current research points to abnormalities in certain brain regions (including the basal ganglia, frontal lobes, and cortex), the circuits that interconnect these regions, and the neurotransmitters (dopamine, serotonin, and norepinephrine) responsible for communication among nerve cells. Given the often complex presentation of TS, the cause of the disorder is likely to be equally complex.

How it is diagnosed

TS is a diagnosis that doctors make after verifying that the patient has had both motor and vocal tics for at least 1 year.  The existence of other neurological or psychiatric conditions can also help doctors arrive at a diagnosis.  Common tics are not often misdiagnosed by knowledgeable clinicians.  However, atypical symptoms or atypical presentations (for example, onset of symptoms in adulthood) may require specific specialty expertise for diagnosis. There are no blood, laboratory, or imaging tests needed for diagnosis.  In rare cases, neuroimaging studies, such as magnetic resonance imaging (MRI) or computerized tomography (CT),   electroencephalogram (EEG) studies, or certain blood tests may be used to rule out other conditions that might be confused with TS when the history or clinical examination is atypical. 

It is not uncommon for patients to obtain a formal diagnosis of TS only after symptoms have been present for some time. The reasons for this are many.  For families and physicians unfamiliar with TS, mild and even moderate tic symptoms may be considered inconsequential, part of a developmental phase, or the result of another condition.  For example, parents may think that eye blinking is related to vision problems or that sniffing is related to seasonal allergies.  Many patients are self-diagnosed after they, their parents, other relatives, or friends read or hear about TS from others. 

How is TS treated

Because tic symptoms often do not cause impairment, the majority of people with TS require no medication for tic suppression. However, effective medications are available for those whose symptoms interfere with functioning. Neuroleptics (drugs that may be used to treat psychotic and non-psychotic disorders) are the most consistently useful medications for tic suppression; a number are available but some are more effective than others (for example, haloperidol and pimozide).

Unfortunately, there is no one medication that is helpful to all people with TS, nor does any medication completely eliminate symptoms.  In addition, all medications have side effects. Many neuroleptic side effects can be managed by initiating treatment slowly and reducing the dose when side effects occur. The most common side effects of neuroleptics include sedation, weight gain, and cognitive dulling.  Neurological side effects such as tremor, dystonic reactions (twisting movements or postures), parkinsonian-like symptoms, and other dyskinetic (involuntary) movements are less common and are readily managed with dose reduction.

Discontinuing neuroleptics after long-term use must be done slowly to avoid rebound increases in tics and withdrawal dyskinesias. One form of dyskinesia called tardive dyskinesia is a movement disorder distinct from TS that may result from the chronic use of neuroleptics. The risk of this side effect can be reduced by using lower doses of neuroleptics for shorter periods of time. 

Other medications may also be useful for reducing tic severity, but most have not been as extensively studied or shown to be as consistently useful as neuroleptics.  Additional medications with demonstrated efficacy include alpha-adrenergic agonists such as clonidine and guanfacine.  These medications are used primarily for hypertension but are also used in the treatment of tics. The most common side effect from these medications that precludes their use is sedation. However, given the lower side effect risk associated with these medications, they are often used as first-line agents before proceeding to treatment with neuroleptics.

Effective medications are also available to treat some of the associated neurobehavioral disorders that can occur in patients with TS.  Recent research shows that stimulant medications such as methylphenidate and dextroamphetamine can lessen ADHD symptoms in people with TS without causing tics to become more severe.  However, the product labeling for stimulants currently contraindicates the use of these drugs in children with tics/TS and those with a family history of tics. Scientists hope that future studies will include a thorough discussion of the risks and benefits of stimulants in those with TS or a family history of TS and will clarify this issue.  For obsessive-compulsive symptoms that significantly disrupt daily functioning, the serotonin reuptake inhibitors (clomipramine, fluoxetine, fluvoxamine, paroxetine, and sertraline) have been proven effective in some patients. 

Behavioral treatments such as awareness training and competing response training can also be used to reduce tics.  A recent NIH-funded, multi-center randomized control trial called Cognitive Behavioral Intervention for Tics,  or CBIT,  showed that training to voluntarily move in response to a premonitory urge can reduce tic symptoms.   Other behavioral therapies, such as biofeedback or supportive therapy, have not been shown to reduce tic symptoms.  However, supportive therapy can help a person with TS better cope with the disorder and deal with the secondary social and emotional problems that sometimes occur. 

Is it inherited

Evidence from twin and family studies suggests that TS is an inherited disorder. Although early family studies suggested an autosomal dominant mode of inheritance (an autosomal dominant disorder is one in which only one copy of the defective gene, inherited from one parent, is necessary to produce the disorder), more recent studies suggest that the pattern of inheritance is much more complex.  Although there may be a few genes with substantial effects, it is also possible that many genes with smaller effects and environmental factors may play a role in the development of TS.

Genetic studies also suggest that some forms of ADHD and OCD are genetically related to TS, but there is less evidence for a genetic relationship between TS and other neurobehavioral problems that commonly co-occur with TS. It is important for families to understand that genetic predisposition may not necessarily result in full-blown TS; instead, it may express itself as a milder tic disorder or as obsessive-compulsive behaviors. It is also possible that the gene-carrying offspring will not develop any TS symptoms.

The gender of the person also plays an important role in TS gene expression. At-risk males are more likely to have tics and at-risk females are more likely to have obsessive-compulsive symptoms.

Genetic counseling of individuals with TS should include a full review of all potentially hereditary conditions in the family.

What is the prognosis 

Although there is no cure for TS, the condition in many individuals improves in the late teens and early 20s. As a result, some may actually become symptom-free or no longer need medication for tic suppression. Although the disorder is generally lifelong and chronic, it is not a degenerative condition. Individuals with TS have a normal life expectancy. TS does not impair intelligence. Although tic symptoms tend to decrease with age, it is possible that neurobehavioral disorders such as ADHD, OCD, depression, generalized anxiety, panic attacks, and mood swings can persist and cause impairment in adult life.

Research

Within the Federal government, the National Institute of Neurological Disorders and Stroke (NINDS), a part of the National Institutes of Health (NIH), is responsible for supporting and conducting research on the brain and nervous system.  The NINDS and other NIH components, such as the National Institute of Mental Health, the Eunice Kennedy Shriver National Institute of Child Health and Human Development, the National Institute on Drug Abuse, and the National Institute on Deafness and Other Communication Disorders, support research of relevance to TS, either at NIH laboratories or through grants to major research institutions across the country.  Another component of the Department of Health and Human Services, the Centers for Disease Control and Prevention, funds professional education programs as well as TS research.

Knowledge about TS comes from studies across a number of medical and scientific disciplines, including genetics, neuroimaging, neuropathology, clinical trials (medication and non-medication), epidemiology, neurophysiology, neuroimmunology, and descriptive/diagnostic clinical science.

Genetic studies. Currently, NIH-funded investigators are conducting a variety of large-scale genetic studies. Rapid advances in the technology of gene discovery will allow for genome-wide screening approaches in TS, and finding a gene or genes for TS would be a major step toward understanding genetic risk factors. In addition, understanding the genetics of TS genes may strengthen clinical diagnosis, improve genetic counseling, lead to the clarification of pathophysiology, and provide clues for more effective therapies.

Neuroimaging studies.  Advances in imaging technology and an increase in trained investigators have led to an increasing use of novel and powerful techniques to identify brain regions, circuitry, and neurochemical factors important in TS and related conditions.

Neuropathology.  There has been an increase in the number and quality of donated postmortem brains from TS patients available for research purposes.  This increase, coupled with advances in neuropathological techniques, has led to initial findings with implications for neuroimaging studies and animal models of TS.

Clinical trials.  A number of clinical trials in TS have recently been completed or are currently underway.  These include studies of stimulant treatment of ADHD in TS and behavioral treatments for reducing tic severity in children and adults. Smaller trials of novel approaches to treatment such as dopamine agonists and glutamatergic medications also show promise.

Epidemiology and clinical science.  Careful epidemiological studies now estimate the prevalence of TS to be substantially higher than previously thought with a wider range of clinical severity. Furthermore, clinical studies are providing new findings regarding TS and co-existing conditions.  These include subtyping studies of TS and OCD, an examination of the link between ADHD and learning problems in children with TS, a new appreciation of sensory tics, and the role of co-existing disorders in rage attacks. One of the most important and controversial areas of TS science involves the relationship between TS and autoimmune brain injury associated with group A beta-hemolytic streptococcal infections or other infectious processes. There are a number of epidemiological and clinical investigations currently underway in this intriguing area.