Tourettes syndrome


Tourette's Syndrome

Description, causes and Risk Factors:

A tic disorder appearing in childhood, characterized by multiple motor tics and vocal tics present for more than 1 year. Obsessive-compulsive behavior, attention-deficit disorder, and other psychiatric disorders may be associated; coprolalia and echolalia rarely occur; autosomal dominant inheritance.

Alternative Names: Gilles de la Tourette disease, Tourette disease, Gilles de la Tourette syndrome.

Abbreviation: TS.

ICD-9-CM: 307.23.

Tourette's syndrome is a hereditary tic disorder that begins during childhood. Tourette syndrome is a genetic neurological disorder characterized by motor and vocal tics. A tic is a sudden, repetitive, and involuntary muscle movement or sound displayed unconsciously.

One of the genes associated with TS is SLITRK1. A mutation of this gene was identified in a small number of patients with Tourette syndrome. This gene provides the instruction necessary for a certain protein that is active in the brain, to be produced. The SLITRK1 protein is considered to play an important role in the development of nerve cells, including the growth of specialized extensions (axons and dendrites) that allow the cell to communicate with nearby nerve cells. However, it is not known how the mutation of this gene leads to the development of this disorder.

More recent studies show that the pattern of inheritance is much more complex and environmental factors may also play an important role.

Symptoms:

Tourette syndrome' symptoms evolve from simple to complex and from motor tics to vocal tics. They first occur in the head and neck region and can spread to more muscle groups from other area of the body including trunk and extremities. Patients that develop TS syndrome display a combination/cluster of different motor and vocal tics.

Tics can occur once or many times during the day and they follow a waxing and waning course decreasing or increasing in frequency and intensity. Tics are more severe in the first years after the onset of the syndrome and starting with the mid-teen to the late teen years and early adulthood the tics improve for the majority of the patient. Although Tourette syndrome is a chronic disorder with life long symptoms, there are patients that may have weeks or even years with few or no symptoms.

Symptoms of Tourette syndrome usually emerge in childhood or in the teenage years. TS isn't common — only about 3 in every 1,000 people has the condition. Boys are more likely to have it.

Only 10% of the patients affected by Tourette syndrome have a progressive course that lasts into adult years. For most patients, symptoms may completely resolve during adulthood.

Diagnosis:

Before TS can be diagnosed, a person must have tics for at least a year. Although tics may occur every day or intermittently throughout the year, for TS to be diagnosed, there must not be a tic-free period longer than 3 months. The neurologist may ask you to keep track of the frequency and kinds of tics your child is having.

There isn't a specific diagnostic test for TS — instead, the doctor diagnoses it after taking a medical history and doing a physical exam. Sometimes, doctors use imaging tests like magnetic resonance imaging tests (MRIs), computerized tomography (CT) scans, electroencephalograms (EEGs), or blood tests to rule out other conditions that might have symptoms similar to TS.

Treatment:

Just as TS is different for every person, the treatment for it varies, too. Most tics do not interfere with a child's life, and do not require any medication. While there isn't a cure for TS, sometimes doctors suggest medications to help control symptoms that begin to interfere with a child's schoolwork or daily life. Talk to your child's doctor about whether medication would be right for your child.

TS is not a psychological condition, but doctors sometimes refer kids and teens with TS to a psychologist or psychiatrist. Seeing a therapist won't stop tics, but it can help kids and teens to talk to someone about their problems, cope with stress better, and learn relaxation techniques.

Treatment to suppress tics is recommended only if they are significantly interfering with the children's activities or self-image; treatment does not alter the natural history of the disorder. Clonidine 0.05 to 0.1 mg p.o. t.i.d. or q.i.d. is effective in some patients. Adverse effects of fatigue may limit dosage; hypotension is uncommon.

Antipsychotics (e.g., risperidone 0.25 to 1.5 mg p.o. b.i.d., haloperidol 0.5 to 2 mg p.o. b.i.d. or t.i.d., 1 to 2 mg p.o. b.i.d., olanzapine 2.5 to 5 mg once/day) may be required. The lowest dose required to make tics tolerable is used; doses are tapered as tics wane. Adverse effects of dysphoria, Parkinsonism, akathisia, and tardive dyskinesia may limit use of antipsychotics; using lower daytime doses and higher bedtime doses may decrease adverse effects.

Note: The following drugs and medications are in some way related to, or used in the treatment. This service should be used as a supplement to, and NOT a substitute for, the expertise, skill, knowledge and judgment of healthcare practitioners.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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