Dystonia Association South Africa

(Incorporating Essential Tremor, Tourette's Syndrome

 and other Movement Disorders)

Reg No 004-729 NPO

P O Box 4351, Randburg, 2125 or 3rd Floor Standard Bank Building, Oak Avenue, Randburg 2194

Tel: +27 11 326 2112 Fax: +27 11 326 3041

e-mail dystonia@dystonia.org.za , Web www.dystonia.org.za
Office hours - 08.30 to 14.00 Monday to Friday
Urgent enquiries 082 357 6586
 

YEAR 2006/2007

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Tourette's Syndrome

Tourette's Syndrome (TS) is an inherited neurological disorder characterized by sudden, involuntary, repetitive muscle movements (motor tics) and vocalizations (vocal tics). The disorder is also known as Gilles de la Tourette syndrome for the neurologist who described it in 1885.

The nature and complexity of the tics are usually variable over time with natural waxing and waning in frequency and severity. Many individuals with TS also develop associated behavioural problems, such as obsessions and compulsions, inattention, hyperactivity and impulsivity. Symptom onset typically occurs during childhood or early adolescence.

The primary characteristics associated with TS are multiple motor tics and one or more vocal tics. Motor and vocal tics may develop at about the same time or predominate at different times during the course of the disease.

Patients initially develop sudden, rapid, recurrent, involuntary movements (motor tics) particularly of the head and facial area. Initially, motor tics usually consist of abrupt, brief, isolated movements known as simple motor tics, such as repeated eye blinking or facial twitching. Simple motor tics may also include repeated neck stretching, head jerking, or shoulder shrugging. Less commonly, motor tics are more "coordinated" with distinct movements involving several muscle groups, such as repetitive squatting, skipping or hopping. These tics, referred to as complex motor tics, may also include repetitive touching of others, deep knee bending, jumping, smelling of objects, hand gesturing, head shaking, leg kicking, or turning in a circle. In addition to affecting the head and facial area, motor tics also affect other parts of the body, such as the shoulders, torso, arms and legs. The anatomical locations of motor tics may change over time. Rarely, motor tics evolve to include behaviours that may result in self-injury, such as scratching and lip biting.

Vocal tics are sudden, involuntary, recurrent, often relatively loud vocalizations. Vocal tics usually begin as single, simple sounds that may eventually progress to involve more complex phrases and vocalizations. For example, patients may initially develop simple vocal tics, including grunting, throat clearing, sighing, barking, hissing, sniffing, tongue clicking or snorting. Complex vocal tics may involve repeating certain phrases or words out of context, one’s own words or sounds (palilalia) or the last words or phrases spoken by others (echolalia). Rarely, there may be involuntary, explosive cursing or compulsive utterance of obscene words or phrases (coprolalia).

Most individuals with TS gradually develop a combination of different motor and vocal tics. These tics may occur a few or many times during the day, often in "clusters". Symptoms typically follow a waxing and waning coarse, periodically decreasing and increasing in frequency and intensity. Tics often subside during absorbing activities such as reading or working, decline during sleep, worsen with stress or fatigue, and may be voluntarily suppressed for brief periods. Although TS is considered lifelong, some patients may have weeks or even years without symptoms. Tics frequency and severity often significantly diminish during adolescence or adulthood. In addition, in some patients, symptoms may completely resolve by early adulthood.

Many individuals with TS also develop associated behavioural problems, particularly obsessive-compulsive behaviours during which certain repetitive actions and rituals are performed. For example, compulsions may include touching particular objects in a predetermined sequence, repeatedly counting, or engaging in repetitive hand washing. In addition, as many as 60% of children with TS may also have symptoms of attention-deficit hyperactivity disorder (ADHD). ADHD is characterized by over activity as well as difficulty maintaining attention. Affected children may also have impulse control difficulties and learning disabilities.

Tics may not only be embarrassing but may significantly interfere with certain activities such as writing or reading as well as other activities of daily living. Because of anxiety about experiencing tics in public or social situations, patients may develop extreme self-consciousness or depression and attempt to withdraw from professional or social situations. In addition ADHD, impulsively, learning problems, and obsessive-compulsive behaviours may interfere with social interactions and impair academic and occupational performance.

The diagnosis of TS is based upon a thorough clinical evaluation, observation and assessment of characteristic symptoms, and a careful patient and family history. There is no definitive diagnosis test for TS. However, certain blood tests, other laboratory studies, or neuro- imaging techniques may be conducted to eliminate related disorders with similar symptoms. Such neuro- imaging studies may include computerized tomography (CT) scanning, magnetic resonance imaging (MRI), positron emission tomography (PET) scanning, electro- encrephalography (EEG) or other techniques. These tests, however, are rarely needed in most patients with TS.

According to the Tourette Syndrome Study Group (1993) certain criteria must be fulfilled for the diagnosis of definite TS. These include the following:

The presence of multiple motor tics and one or more vocal tics at some time during the course of the disorder that are:

Directly observed by a reliable examiner or recorded (e.g. by videotape)

Symptom onset before age 21

The goal of therapy in patients with TS is to reduce motor and vocal tics and alleviate associated behavioural problems, such as obsessive-compulsive behaviours, ADHD, and impulsivity. Many patients do not have significant functional impairment because their symptoms are mild and therefore may not require medication. However, for those with symptoms that are functionally disabling and affect academic, occupational, or social performance, there are a number of medications that may alleviate particular symptoms.

Drug therapy may include low doses of certain anti-psychotic (dopamine receptor antagonist) medications (neuroleptics), such as haloperidol (Haldol®), pimozide (Orap®), and fluphenazine (Prolixin®), which have been found to be effective in reducing the frequency and intensity of tics. These medications should be prescribed with caution since their use may be associated with certain severe side effects. Adverse effects associated with neuroleptic therapy include the development of tardive dyskinesia (TD), a movement disorder characterized by persistent, repetitive (stereotypic) involuntary movements usually involving the lower face and mouth. Although TD often resolves with the discontinuation of drug therapy, particularly in children, the condition is not always reversible. Therefore, those who receive long-term neuroleptic therapy should be periodically evaluated to determine whether dosage level may be decreased or therapy may be discontinued. Neuroleptic therapy may be associated with certain short-term side effects, such as drooling, contraction of the facial and neck muscles, slow movement (bradykinesia), restlessness (akathisia), and other symptoms.

Injections of botulinum toxin (BTX) into the muscles involved in tics may markedly alleviate not only the abnormal movements but also the "foreboding" (premonitory) sensations or urges that precede the tics. The administration of the anti-anxiety medication clonazepam (Klonopin®) or certain antidepressant medications may be helpful in the management of some of the associated behavioural symptoms. Therapy with clonidine (Catapres®) or guanfacine (Tenex®), alpha 2-adrenergic agonists, may relieve symptoms of ADHD and impulsivity, but these drugs are generally not very effective in controlling tics. In addition, in patients with obsessive-compulsive behaviours, treatment with certain antidepressant agents known as selective serotonin reuptake inhibitors (SSRIs) may be beneficial. Such medication include fluvoxamine (Luvox®), fluoxetine (Prozac®), clomipramine (Anafranil®), and many others.

Biofeedback, relaxation methods, or other behavioural techniques may occasionally be helpful in alleviating stress that potentially aggravates tics. In addition, for those with associated behavioural difficulties, individualized academic, vocational, social, or other supportive services are often beneficial.

Taken from We move 1999 http://www.wemove.org/