Tourette’s syndrome
A neurological disorder characterized by tics, or rapid, sudden movements that occur involuntarily and repeatedly in a consistent fashion. To be diagnosed with Tourette’s syndrome, an individual must have multiple motor tics as well as one or more vocal tics over a period of more than one year. These need not all occur simultaneously, but in general the tics may occur many times a day, usually in brief, intense groupings, nearly every day, or intermittently.
Common simple tics include eye blinking, shoulder jerking, picking movements, grunting, sniffing, and barking. Complex tics include facial grimacing, arm flapping, coprolalia (use of obscene words), palilalia (repeating one’s own words), and echolalia (repeating another’s words or phrases).
For individuals with Tourette’s, tics may vary over time in terms of their frequency and severity, as well as in type and location. In some cases, symptoms may disappear for a period of weeks or even months. Although there is an involuntary quality to the tics experienced by individuals with Tourette’s, most persons have some control over their symptoms, at least briefly, and even for hours at a time. However, suppressing them tends simply to postpone a more severe outburst, since the impulse to express tics is ultimately irresistible and must be expressed. Tics often will increase in response to stress and become less frequent with relaxation or intense focus on a task.
Many patients with Tourette’s syndrome have other conditions at the same time, such as ATTENTION DEFICIT HYPERACTIVITY DISORDER (ADHD), OBSESSIVE-COMPULSIVE DISORDER, or LEARNING DISABILITIES.
Up to 20 percent of children have at least a transient tic disorder at some point. Once believed to be rare, Tourette’s syndrome is now known to be a more common disorder that represents the most complex and severe manifestation of the spectrum of tic disorders.
Symptoms
Obsessions, compulsions, impulsive behavior, and mood swings. Tourette’s is commonly associated with other syndromes, including attention deficit hyperactivity disorder, anxiety, mood, or panic disorders, obsessive-compulsive disorder, behavior problems, and learning disabilities.
In most children, Tourette’s syndrome fluctuates; anxiety, stress, and fatigue often intensify tics, which are usually significantly reduced during sleep or when the child is focused on an activity. Psychoactive drugs (especially cocaine and stimulants) tend to worsen tics.
In most cases, tics peak in severity between ages nine and 11, but between 5 and 10 percent of children continue to have unchanged or worsening symptoms into adolescence and adulthood. There is no reliable way to predict which children will have a poorer prognosis.
Cause
An abnormal metabolism of the neurotransmitters dopamine and serotonin is linked to the disorder, which is genetically transmitted. Parents have a 50 percent chance of passing the gene on to their children. Girls with the gene have a 70 percent chance of displaying symptoms, boys with the gene have a 99 percent chance of displaying symptoms.
Diagnosis
The single most important component of managing the condition is to get an accurate diagnosis. Tics occur suddenly during normal activity, unlike other movement disorders such as:
• Chorea A pattern of nonrepetitive irregular movements
• Stereotypy Constant, repetitive behaviors performed for no obvious reason
• Dystonias A slow, constant repetitive behavior
The doctor will want to rule out any secondary causes of tic disorders. A complete general physical examination is important, with specific attention to the central nervous system. The thyroid-stimulating hormone (TSH) level should be measured in most patients, since tics often occur together with hyperthyroidism. A throat culture should be checked for group A beta-hemolytic streptococcus, especially if symptoms get worse or better with ear or throat infections. The evidence of strep infection with a single occurrence of worsening tics is not enough to make a diagnosis of streptococcus-induced, autoimmune-caused Tourette’s syndrome.
An electroencephalogram is useful only in children in whom it is difficult to differentiate tics from manifestations of EPILEPSY. Imaging studies are not likely to be helpful, and the importance of other studies depends on symptoms. For example, a urine drug screen for cocaine and stimulants should be considered in the case of a teenager with sudden onset of tics and inappropriate behavior symptoms. A child with a family history of liver disease associated with a Parkinsonian or hyperkinetic movement disorder should undergo a test for copper in the blood to rule out Wilson’s disease.
The basic workup is usually appropriate in a child with a gradual onset of symptoms, a developmental progression of tics, and a family history of tics or obsessive-compulsive disorder.
Treatment
Positive reinforcement programs appear to be most helpful in managing tic disorders. Target behaviors may be categorized into two groups, skill deficiencies (areas that initially require concentration to build social and academic skills) and behavior excesses, in which the goal is to help the patient decrease the frequency of these behaviors. Managing behavior excesses needs to be handled carefully, however, since some children who undergo behavior modification to directly target the Tourette’s symptoms experience a worsening of symptoms.
Drug Treatment
The goal in tic control is to use the lowest dosage of medication that will bring the patient’s functioning to an acceptable level. Often this will require only modest levels of tic reduction. The most common drug treatments are haloperidol (Haldol), pimozide (Orap), risperidone (Risperdal), and clonidine (Catapres). Guanfacine (Tenex) is not labeled for use in children under 12 years of age. Less often, clonazepam (Klonopin) may be prescribed. For tics of mild to moderate severity, or in patients who are wary of drug side effects, an initial trial of clonidine or guanfacine may be tried. These medications are modestly effective in tic control and have a range of less specific benefits. Many children taking them may be less irritable or less impulsive, and manifestations of ADHD may improve as well.
Side Effects
These include sedation, weight gain, poor school performance, social anxiety with school refusal in children, and unusual body movements, including tardive dyskinesias, a potentially irreversible drug-caused movement disorder that may be difficult to distinguish from tics. When pimozide is used, baseline and follow-up electrocardiograms are recommended.
Most children with Tourette’s syndrome require medication for up to one to two years. About 15 percent of patients require long-term medication for tic control. When tics appear to be stable and adequately controlled for a period of four to six months, a slow and gradual reduction in medication should follow. With such a strategy, occasional drug holidays may be possible in some patients as tics lessen. If tics increase, incremental increases in medication may be needed.
Because many children with Tourette’s syndrome have other conditions, treatment for these conditions may be necessary. Treatment of ADHD with Tourette’s has been controversial because of reports that stimulants hasten the onset or increase the severity of tics in some patients. This observation alone may not be a contraindication for stimulant treatment in patients with significant symptoms of ADHD. Stimulants alone may not substantially worsen the course or severity of the disorder. In some cases, it may be necessary to treat both the ADHD and the Tourette’s syndrome with a stimulant in combination with either clonidine or guanfacine, or with a neuroleptic agent. A trial of clonidine or guanfacine alone may be sufficient to adequately treat both conditions. When possible, multiple drugs should not be used, especially in children.
Common simple tics include eye blinking, shoulder jerking, picking movements, grunting, sniffing, and barking. Complex tics include facial grimacing, arm flapping, coprolalia (use of obscene words), palilalia (repeating one’s own words), and echolalia (repeating another’s words or phrases).
For individuals with Tourette’s, tics may vary over time in terms of their frequency and severity, as well as in type and location. In some cases, symptoms may disappear for a period of weeks or even months. Although there is an involuntary quality to the tics experienced by individuals with Tourette’s, most persons have some control over their symptoms, at least briefly, and even for hours at a time. However, suppressing them tends simply to postpone a more severe outburst, since the impulse to express tics is ultimately irresistible and must be expressed. Tics often will increase in response to stress and become less frequent with relaxation or intense focus on a task.
Many patients with Tourette’s syndrome have other conditions at the same time, such as ATTENTION DEFICIT HYPERACTIVITY DISORDER (ADHD), OBSESSIVE-COMPULSIVE DISORDER, or LEARNING DISABILITIES.
Up to 20 percent of children have at least a transient tic disorder at some point. Once believed to be rare, Tourette’s syndrome is now known to be a more common disorder that represents the most complex and severe manifestation of the spectrum of tic disorders.
Symptoms
Obsessions, compulsions, impulsive behavior, and mood swings. Tourette’s is commonly associated with other syndromes, including attention deficit hyperactivity disorder, anxiety, mood, or panic disorders, obsessive-compulsive disorder, behavior problems, and learning disabilities.
In most children, Tourette’s syndrome fluctuates; anxiety, stress, and fatigue often intensify tics, which are usually significantly reduced during sleep or when the child is focused on an activity. Psychoactive drugs (especially cocaine and stimulants) tend to worsen tics.
In most cases, tics peak in severity between ages nine and 11, but between 5 and 10 percent of children continue to have unchanged or worsening symptoms into adolescence and adulthood. There is no reliable way to predict which children will have a poorer prognosis.
Cause
An abnormal metabolism of the neurotransmitters dopamine and serotonin is linked to the disorder, which is genetically transmitted. Parents have a 50 percent chance of passing the gene on to their children. Girls with the gene have a 70 percent chance of displaying symptoms, boys with the gene have a 99 percent chance of displaying symptoms.
Diagnosis
The single most important component of managing the condition is to get an accurate diagnosis. Tics occur suddenly during normal activity, unlike other movement disorders such as:
• Chorea A pattern of nonrepetitive irregular movements
• Stereotypy Constant, repetitive behaviors performed for no obvious reason
• Dystonias A slow, constant repetitive behavior
The doctor will want to rule out any secondary causes of tic disorders. A complete general physical examination is important, with specific attention to the central nervous system. The thyroid-stimulating hormone (TSH) level should be measured in most patients, since tics often occur together with hyperthyroidism. A throat culture should be checked for group A beta-hemolytic streptococcus, especially if symptoms get worse or better with ear or throat infections. The evidence of strep infection with a single occurrence of worsening tics is not enough to make a diagnosis of streptococcus-induced, autoimmune-caused Tourette’s syndrome.
An electroencephalogram is useful only in children in whom it is difficult to differentiate tics from manifestations of EPILEPSY. Imaging studies are not likely to be helpful, and the importance of other studies depends on symptoms. For example, a urine drug screen for cocaine and stimulants should be considered in the case of a teenager with sudden onset of tics and inappropriate behavior symptoms. A child with a family history of liver disease associated with a Parkinsonian or hyperkinetic movement disorder should undergo a test for copper in the blood to rule out Wilson’s disease.
The basic workup is usually appropriate in a child with a gradual onset of symptoms, a developmental progression of tics, and a family history of tics or obsessive-compulsive disorder.
Treatment
Positive reinforcement programs appear to be most helpful in managing tic disorders. Target behaviors may be categorized into two groups, skill deficiencies (areas that initially require concentration to build social and academic skills) and behavior excesses, in which the goal is to help the patient decrease the frequency of these behaviors. Managing behavior excesses needs to be handled carefully, however, since some children who undergo behavior modification to directly target the Tourette’s symptoms experience a worsening of symptoms.
Drug Treatment
The goal in tic control is to use the lowest dosage of medication that will bring the patient’s functioning to an acceptable level. Often this will require only modest levels of tic reduction. The most common drug treatments are haloperidol (Haldol), pimozide (Orap), risperidone (Risperdal), and clonidine (Catapres). Guanfacine (Tenex) is not labeled for use in children under 12 years of age. Less often, clonazepam (Klonopin) may be prescribed. For tics of mild to moderate severity, or in patients who are wary of drug side effects, an initial trial of clonidine or guanfacine may be tried. These medications are modestly effective in tic control and have a range of less specific benefits. Many children taking them may be less irritable or less impulsive, and manifestations of ADHD may improve as well.
Side Effects
These include sedation, weight gain, poor school performance, social anxiety with school refusal in children, and unusual body movements, including tardive dyskinesias, a potentially irreversible drug-caused movement disorder that may be difficult to distinguish from tics. When pimozide is used, baseline and follow-up electrocardiograms are recommended.
Most children with Tourette’s syndrome require medication for up to one to two years. About 15 percent of patients require long-term medication for tic control. When tics appear to be stable and adequately controlled for a period of four to six months, a slow and gradual reduction in medication should follow. With such a strategy, occasional drug holidays may be possible in some patients as tics lessen. If tics increase, incremental increases in medication may be needed.
Because many children with Tourette’s syndrome have other conditions, treatment for these conditions may be necessary. Treatment of ADHD with Tourette’s has been controversial because of reports that stimulants hasten the onset or increase the severity of tics in some patients. This observation alone may not be a contraindication for stimulant treatment in patients with significant symptoms of ADHD. Stimulants alone may not substantially worsen the course or severity of the disorder. In some cases, it may be necessary to treat both the ADHD and the Tourette’s syndrome with a stimulant in combination with either clonidine or guanfacine, or with a neuroleptic agent. A trial of clonidine or guanfacine alone may be sufficient to adequately treat both conditions. When possible, multiple drugs should not be used, especially in children.
Tags: impulsive behavior, learning disabilities, mood swings, neurological disorder, Obsessive-Compulsive Disorder, sudden movements, Tics, Tourette’s Syndrome
Posted in Health and Wellness
