7 - Tics, Tourette Disorder, and Related Problems

Editors: Shader, Richard I.

Title: Manual of Psychiatric Therapeutics, 3rd Edition

Copyright 2003 Lippincott Williams & Wilkins

> Table of Contents > 7 - Tics, Tourette Disorder, and Related Problems

7

Tics, Tourette Disorder, and Related Problems

Barbara J. Coffey

Richard I. Shader

Tics are characterized by stereotyped, rapid, recurrent motor movements or vocalizations that are nonrhythmic, seemingly involuntary, and sudden in onset. Tic disorders, including transient tics, chronic motor or vocal (phonic) tic disorder, and Tourette disorder (TD), are childhood onset disorders that most often begin in the first decade of life.

TD, the most complex of the tic disorders, is characterized by multiple motor and vocal tics and, in clinical settings, a variety of behavioral and emotional symptoms. Although tic disorders may be quite common, limited epidemiologic data suggest a highly variable prevalence rate of TD ranging from 0.4% in Israel to 3% in the United Kingdom. Prevalence rates decline with age from about 4 in 10,000 in childhood to about 0.5 in 10,000 in adulthood. A recent community-based survey reported that about 3% of school-aged children meet the criteria for TD. Although TD is commonly thought to be a lifelong disorder, limited and somewhat contradictory information exists on its longitudinal course. Several recent studies suggest that although tics may persist into adulthood, the tic severity often declines significantly during adolescence.

The past two decades have been marked by significant progress in the understanding of the clinical phenomenology, epidemiology, and comorbidity of TD. Treatment studies have expanded to include the atypical antipsychotic agents and, most recently, targeted combined pharmacotherapy. Nevertheless, TD and tic disorders continue to pose many challenges, especially in the areas of pathophysiology, genetics, developmental neuroscience, psychopathology, and treatment.

This chapter briefly reviews the classification, clinical phenomenology, epidemiology, genetics, etiology, differential diagnosis, clinical assessment, and treatment of TD and chronic tic disorders.

I. Classification and Clinical Phenomenology

Tics typically involve one muscle (simple tic) or a group of muscles (complex tics), and they can be characterized by anatomic location, number, frequency, intensity, and complexity. For tic frequency or intensity to vary during the day is not unusual; afternoon or evening worsening is common. Tics can occur in volleys; many patients report having fewer tics during summer months. Table 7.1 lists some common examples of simple and complex tics. Tic disorders can be classified as transient (i.e., lasting at least 4 weeks but less than 1 year) or chronic (i.e., lasting longer than 1 year). Current diagnostic criteria for TD, chronic motor or vocal tic disorder, and transient tic disorder are detailed in Tables 7.2, 7.3, and 7.4, respectively.

II. Natural History

Most youth with TD experience the onset of symptoms before adolescence, typically by about the age of 6 or 7 years. Most children will experience a progression of simple to complex and rostral to caudal tics over several years, with typical waxing and waning of tics over days to months. Many children with TD who are evaluated in clinical settings will also meet the criteria for attention deficit hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), or other psychiatric disorders. Recent data suggest that the tics in TD may abate or may show reduced severity in many youths over time. Chronic motor tic disorder typically has a similar natural history, and it is often considered a variant of TD.

III. Comorbid Disorders

Most youth seen in clinical settings will meet full or subthreshold criteria for at least one psychiatric comorbid disorder. The most common comorbid diagnoses are ADHD and OCD. Excessive motoric hyperactivity, inattention, and impulsivity are also problematic for many clinically referred TD patients; some investigators report that from 50% to 75% of TD patients also meet criteria for

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ADHD. Recognition of other comorbid emotional disorders in TD, such as mood and non-OCD anxiety disorders, has also been increasing. Although the nature of the relationship between TD and comorbid disorders is still not clear, tics and behavioral, cognitive, or emotional dysfunction may represent manifestations of an underlying disinhibition problem. Comorbid disorders are often the primary clinical concern when these youths are evaluated in clinical settings.

TABLE 7.1. SOME EXAMPLES OF TICS

  Motor Vocal
Simple Eye blinking Throat clearing
Nose twitching Coughing
Shoulder shrugging Grunting
Sniffing
Complex Touching objects or self Uttering syllables or words
Squatting or jumping Uttering phrases
Swearing

TABLE 7.2. DIAGNOSTIC FEATURES OF TOURETTE DISORDER

  1. Both multiple motor and one or more vocal tics are present at some time during the illness, although they are not necessarily concurrent.
  2. The tics occur many times a day (usually in bouts), nearly every day, or intermittently throughout a period of more than 1 yr; no tic-free intervals lasting longer than 3 consecutive mo should occur.
  3. The anatomic location, number, frequency, complexity, and severity of the tics change over time.
  4. The symptoms cause significant distress in terms of social, educational, or occupational functioning.
  5. Age at onset is before 18 yr.
  6. Symptoms are not the result of a medical condition, such as Huntington chorea or postviral encephalitis; substance intoxication; or a medication-induced movement disorder.
From the American Psychiatric Association. Diagnostic and statistical manual of mental disorders, 4th ed., Text revision. Washington, D.C.: American Psychiatric Association, 2000, with permission.

TABLE 7.3. DIAGNOSTIC FEATURES OF CHRONIC MOTOR OR VOCAL TIC DISORDER

  1. Either motor or vocal tics, but not both, are present at some time during the illness.
  2. The tics occur many times a day, nearly every day, or intermittently throughout a period that is longer than 1 yr; no tic-free intervals lasting longer than 3 consecutive mo should occur.
  3. The symptoms cause significant distress in terms of social, educational, or occupational functioning.
  4. Age at onset is before 18 yr.
  5. Symptoms are not the result of a medical condition, such as Huntington chorea or postviral encephalitis; substance intoxication; or a medication-induced movement disorder.
From the American Psychiatric Association. Diagnostic and statistical manual of mental disorders, 4th ed., Text revision. Washington, D.C.: American Psychiatric Association, 2000, with permission.

TABLE 7.4. DIAGNOSTIC FEATURES OF TRANSIENT TIC DISORDER

  1. Single or multiple motor and/or vocal tics that occur many times a day, nearly every day for at least 4 wk, but for no longer than 12 consecutive mo.
  2. No history of signs or symptoms lasting 1 yr or more and meeting criteria for Tourette disorder or chronic motor or vocal tic disorder.
  3. The symptoms cause significant distress in terms of social, educational, or occupational functioning.
  4. Age at onset is before 18 yr.
  5. Symptoms are not the result of a medical condition, such as Huntington chorea or post-viral encephalitis; substance intoxication; or a medication-induced movement disorder.
From the American Psychiatric Association. Diagnostic and statistical manual of mental disorders, 4th ed., Text revision. Washington, D.C.: American Psychiatric Association, 2000, with permission.

A bidirectional relationship between TD and OCD in which OCD is a risk factor for TD and TD is a risk factor for OCD has been described. As many as 90% of clinically referred TD patients meet either the full or subthreshold criteria; up to 40% may meet the full OCD criteria. In addition, family studies indicate that OCD is found at a higher rate in close relatives of patients with TD than it is in control patients. Clinically, making the distinction between complex motor tics and compulsions (e.g., walking forward and then backward through a doorway, four paces in each direction) with certainty can be difficult. The increasing recognition of the subjectively experienced component of tics (e.g., sensory tics or premonitory urges) can also confound these distinctions.

Recently, mood and anxiety disorders in clinically referred TD patients have been described, including major depression, bipolar disorder, panic attacks, panic disorder, and simple and social phobias. Furthermore, some youths with TD who are evaluated in clinical settings may meet criteria for explosive outbursts.

IV. Epidemiology

As was noted above, prevalence estimates for TD vary greatly. Most recent lifetime prevalence rate estimates are 4 to 10 in 1,000 for TD and 1 in 200 for the full spectrum, including chronic motor tics and TD. Across the developmental spectrum, males are at least three to four times more likely than females to manifest TD. Limited data suggest that adults reveal the syndrome approximately one-tenth as often as children and adolescents.

V. Genetics

Genetic data derive from family pedigree, twin studies, and studies of sibling pairs. That TD, chronic tic disorders, and OCD congregate in families is well established. Twin studies have shown a high concordance rate for tic disorders among monozygotic pairs (ranging from 50% to 90%), in contrast to a relatively low rate among dizygotic twins (approximately 10%). Other studies have shown that first-degree relatives of TD patients have a higher percentage of TD, chronic tics, and OCD than do those of normal control subjects. Historically, genetic analyses of family pedigrees have appeared to be consistent with an autosomal dominant mode with incomplete penetrance of inheritance of TD; other investigators have proposed a single major gene locus in combination with other genes or environmental factors. To date, no unique locus has been specifically identified for TD; however, early findings in a study of 110 sibling pairs suggest that the 4q and 8p regions might be involved, although the results of this study did not reach statistical significance.

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VI. Etiology and Pathophysiology

The etiology and pathophysiology of TD and tic disorders remain unknown. Data from neuroimaging and neurochemical studies of TD accumulated during the past decade point to a diffuse process in the brain involving pathways in the basal ganglia, striatum, and frontal lobes. Several neurotransmitters and neuromodulators have been implicated, including dopamine, norepinephrine, serotonin, and endogenous opioids. Neuroimaging studies that use cerebral blood flow and energy (glucose) metabolism parameters (e.g., positron emission tomography and single photon emission tomography) suggest altered activity (increased or decreased, and at times unilaterally) in various areas of the brain, such as the frontal and orbital cortex, the striatum, and the basal ganglia (e.g., putamen), in TD patients compared with that of control subjects or across brain regions within TD patients. Magnetic resonance imaging studies have indicated volume or asymmetry abnormalities in caudate or lenticular nuclei in subjects with TD compared with results for control subjects. A recent functional magnetic resonance imaging study has indicated changes in signal intensity in the basal ganglia, thalamus, and connecting cortical regions during tics.

In 1998, Swedo et al. described a putative subgroup of children with TD and OCD with hypothetical antecedent group A -hemolytic Streptococcus (GABHS) infection who experienced symptom onset or exacerbation that was precipitated by streptococcal infection (pediatric autoimmune neuropsychiatric disorders associated with Streptococcus [PANDAS]). The Swedo group provided specific diagnostic criteria for PANDAS, including prepubertal onset, sudden explosive onset, and/or exacerbations and remissions, as well as a temporal relationship between symptoms and GABHS. One double-blind, placebo-controlled, crossover study of prophylactic oral penicillin that attempted to reduce recurrences of PANDAS failed to show any differences between the drug and the placebo; low penicillin doses and the time of year have made some clinicians question the relevance of the findings from this study. Some investigators believe that the connection of GABHS infection to TD is not direct; instead, they view it as indirect and postulate that the linkage is through ADHD comorbidity.

VII. Differential Diagnosis

Differential diagnosis of TD and tic disorders can be challenging; no specific laboratory tests are confirmatory. Diagnosis is made on clinical grounds, including the characteristic historical features and the clinical examination. Table 7.5 lists other involuntary movement disorders that must be considered in the differential diagnosis.

TABLE 7.5. DIFFERENTIAL DIAGNOSIS OF TIC DISORDERS

Descriptive Terms Observable Movement Patterns
Akathisia Motor restlessness (an unpleasant need to move), usually in the lower extremities
Athetosis Slow writhing movements, usually in the hands and fingers
Ballismus Large amplitude, jerking, shaking, flinging
Chorea Irregular, spasmodic, usually limbs or face
Dyskinesia Choreiform or dystonic, stereotyped, and not suppressible
Dystonia Sustained tonic contraction that progresses to abnormal postures
Myoclonus Sudden, brief, clonic, shock-like jerks or spasms, that usually involve the limbs
Periodic movements of sleep Periodic dorsiflexion of the foot and flexion of the knee that occur during sleep
Stereotypy Repetitive, usually meaningless, gestures, habits, or automatisms

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Other movement disorders that are sometimes confused with TD include the following:

  • Sydenham chorea, a neurologic complication of streptococcal infection in which choreiform, writhing, often truncal movements are observed;

  • Huntington disease, an autosomal dominant disorder presenting with chorea and dementia that typically has its onset in the fourth or fifth decade of life;

  • Parkinson disease, typically a disorder of late life, that is characterized by flat facies, gait disturbance, rigidity, cogwheeling, and pill-rolling resting tremor;

  • PANDAS.

In psychiatric settings, patients who have been exposed to conventional antipsychotic agents (i.e., those showing the extrapyramidal effects at typical therapeutic doses) are at risk for drug-induced dyskinesias, including tardive and withdrawal dyskinesias. These undesirable effects may be seen in TD patients who have been treated with conventional antipsychotic agents. Careful baseline evaluation for and documentation of abnormal movements before initiating any form of therapy (especially before the use of conventional antipsychotic agents) for patients with tic disorders are essential.

VIII. Evaluation and Assessment

A comprehensive detailed history with observations derived from multiple sources is the cornerstone of the clinical evaluation of patients with tic disorders. Because TD is a diagnosis made primarily on the basis of its unique history and since tics may be suppressed during physical examination, reliable sources of information are essential.

Historical inquiry should include detailed medical and developmental information, past experiences with medications (including substances of abuse or recreation), educational and occupational data, social and interpersonal history, and a thorough family pedigree covering at least three generations. A careful, descriptive, longitudinal assessment of the movement disorder is important. The physical examination should include height, weight, presence or absence of dysmorphic features, posture, gait, reflexes, and a systematic rating of current abnormal movements. The Abnormal Involuntary Movement Scale is a systematic assessment procedure and rating form that can be adapted for use with children's tics.

Psychiatric evaluation should include a formal assessment of the comorbid disorders that frequently occur with TD, including OCD, ADHD, and mood and non-OCD anxiety disorders (see Chapters 6, 14, 21, and 22). The use of structured or semistructured diagnostic interviews, such as the Schedule for Affective Disorders and Schizophrenia for Children, the Diagnostic Interview Schedule for Children, and the Diagnostic Interview for Children and Adolescents, can improve the classification and assessment of comorbidity, especially in a research context. Standardized global rating scales developed specifically for the TD population have improved diagnostic reliability in research studies, and they can be easily administered by the clinician. The Yale-Global Tic Severity Scale is a clinician-administered ordinal scale that rates tic number, frequency, intensity, complexity, and interference in the past week; it has an established reliability and validity. Specific rating scales for OCD (e.g., the Leyton Obsessional Inventory, the Yale-Brown Obsessive-Compulsive Scale, the OCD Inventory) and ADHD (e.g., the Conners) can also be used for assessing the severity of these comorbid disorders when they are present.

Auxiliary data from outside sources are extremely useful. Pediatric and medical records may document the developmental and medical history, the adequacy of medication trials and responses, hospitalizations, and laboratory findings. A review of school records is essential for children, because many TD patients manifest their difficulties in school settings. Report cards can document academic performance; direct phone contact with teachers may provide data about attentional, social, and emotional functioning. Neuropsychologic or speech and language testing may be indicated for patients with impairments in school or occupational functioning.

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IX. Treatment

A. General Guidelines

Treatment goals should be to reduce symptoms, to support adaptive functioning and strengths, and to enhance developmental progress. Some clinicians do not advocate treatment unless they are confident that an improved outcome will occur. Treatment should not be forced on either the parents or the child.

  • Self-esteem must always be supported because most patients with tic disorders will have at least some sense of personal and emotional vulnerability.

  • Education focusing on the phenomenology, natural history, and treatment options for TD is essential.

  • Clarification that the patient's symptoms are not voluntary eases the psychologic burdens for both the patient and the family. This is especially important because families observe that tics can be suppressed at times. A practical goal involves personal management of and responsibility for symptoms and behavior, regardless of their origins.

  • Containment and management comprise another cornerstone of treatment. Even with the use of effective medication, complete remission of tics is rare. Use of a tic room or a time out area at home or school provides a way to contain problematic tics or compulsions. Because emotional conflicts and stress frequently increase symptom intensity, time-limited withdrawal from stressful situations can be beneficial.

B. Specific Medications

  • General comments. The decision to treat tic disorders should be based on a thorough and comprehensive assessment. Patients with symptoms that significantly interfere with adaptation to family, school, work, peer relationships, or developmental progress should be treated. Patients who suffer significant emotional distress may also be candidates for treatment, even when the symptoms are relatively mild. Most patients with very mild tics will need monitoring, education, guidance, and support. Moderate to severe tics are usually treated more aggressively.

    In general, patients should be started on the lowest possible dose of any medication and should be titrated upward gradually. Most maximum doses for TD patients will be low compared with the dosages needed for other indications for the same agent. Table 7.6 lists specific drugs and dosages. When possible, monotherapy should be attempted initially. However, given the complexity and frequent comorbidity in most TD patients seeking treatment, targeted combined pharmacotherapy with two or more medications more often is the rule rather than the exception.

    Determining an adequate duration for a medication trial for patients with tics can be quite challenging because the natural course of TD involves the waxing and waning of tics over time. As a general guideline, one should wait at least several weeks after dosage adjustment before drawing conclusions about a given dose efficacy. External stressors as potential determinants of increased tic symptomatology must be taken into account and addressed accordingly. In general, achieving complete remission of tics with pharmacotherapy is rare, but attempting to reduce tics substantially is reasonable.

  • Recommended pharmacotherapies for TD and other tic disorders

    • -Adrenergic agonists. Clonidine, a centrally acting 2-adrenergic receptor (presynaptic) agonist, has been used for about two decades for the treatment of TD. Clonidine is the recommended first-line treatment for mild to moderate tics, with or without comorbid ADHD. Unfortunately, this is not a United States Food and Drug Administration-approved indication (i.e., in the approved wording for the product label) for clonidine or any other agents in its class. When given in low doses, the postulation is that the presynaptic noradrenergic effects mediate the regularly observed clinical improvement in tics. In addition, unlike the antipsychotic agents, clonidine

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      also ameliorates the disinhibition, impulsivity, hyperarousal, and motoric overactivity often seen in TD patients.

      TABLE 7.6. RECOMMENDED PHARMACOTHERAPIES FOR TOURETTE DISORDER AND OTHER TIC DISORDERS

      Class Agent Typical Range (mg) Starting Dose (mg) Maximum Recommended Dose Common Side Effects
      Partial
         2-adrenergic
         agonists
            Clonidine 0.05 0.45 0.25 0.5 0.45 mg Sedation, headaches, dysphoria
            Gunafacine 0.5 4 0.25 0.5 4 mg Like clonidine but less sedating
      Atypical anti-psychotic agents
            Risperidone 0.5 4 0.25 0.5 6 mg Weight gain, sedation, prolactinemia
            Olanzapine 2.5 5 1.25 2.5 10 mg Similar to risperidone
      Conventional anti-psychotic agents
            Haloperidol 0.25 5 0.25 0.5 5 mg Sedation, weight gain, dysphoria, extrapyramidal effects
            Pimozide 1 10 0.5 1 10 mg Sedation, weight gain, ECG changes
      Antidepressants: selective serotonin reuptake inhibitors
            Fluoxetine 5 40 2.5 10 60 80 mg Behavioral activation, insomnia, gastrointestinal distress
            Sertraline 25 200 12.5 25 200 250 mg Same as fluoxetine
            Fluvoxamine 50 300 12.5 25 300 mg Same as fluoxetine
      Antidepressants: tricyclics
            Clomipramine 50 200 25 50 250 300 mg Dry mouth, sedation, weight gain, ECG changes
            Desipramine 50 300 10 25 5 mg/kg/d Same as clomipramine
            Nortriptyline 25 100 10 25 2.5/mg/kg/d Same as clomipramine
      Psychostimulants
            Methylphenidate 5 40 2.5 10 Individualized Insomnia, anorexia, dysphoria
      d-amphetamine 2.5 30 2.5 5 Individualized Same as methyl-phenidate
      Abbreviation: ECG; electrocardiogram.

      Clonidine should be started at 0.025 mg once or twice a day for prepubertal children and should be increased by 0.025 mg every 1 to 2 weeks. Clonidine is typically administered to prepubertal children three or four times a day. Clonidine's half-life varies from 10 to 30 hours; in younger children, it tends to have shorter half-lives. In adolescents, it tends to have intermediate values (i.e., 13 to 14 hours). Adolescents or adults can be started on 0.05 mg once or twice a day, and the dosage may be increased by 0.05 mg increments. Older patients may require less frequent dosing. The total daily dose typically is 0.05 to 0.45 mg (up to 8.0 g per kg per day). Sedation, headaches, or stomach discomfort are the common side effects. Sedation usually will diminish over several weeks; when it does not, dosage reduction may be helpful. Cardiovascular effects (i.e., hypotension) are minimal in this dosage range, but an electrocardiogram, the blood pressure, and pulse should be evaluated before the initiation of treatment and should be monitored periodically. A withdrawal syndrome can occur after abrupt discontinuation. This complication is usually seen in patients taking more than 0.3 mg per day over an extended time period. A transdermal form of clonidine, Catapres TTS, is available for children who cannot swallow pills or for those who require very frequent dosing or more even absorption.

      Guanfacine, another 2-adrenergic receptor (presynaptic) agonist, is an alternative when clonidine has not been effective or when the response has been limited by side effects. Guanfacine appears to have a longer half-life than clonidine, and thus it can be given on a twice a day schedule. Additionally, it appears to cause sedation about half as often as clonidine. Guanfacine is also less likely than clonidine to lead to a withdrawal syndrome after abrupt discontinuation. Nevertheless, tapering is advised. Guanfacine is preferred by some clinicians.

    • Antipsychotic agents. Since the late 1960s when orally administered haloperidol was first introduced as a treatment for motor and vocal tics in TD patients, conventional antipsychotic agents have been the only medications that are formally approved for labeling for TD. These agents, including haloperidol and pimozide (see Chapter 4), antagonize dopamine (D2) receptors in the basal ganglia. From the risk-versus-benefit perspective, they are effective and they are relatively safe for children, adolescents, or adults with moderately severe to severe tics. However, conventional antipsychotic agents are not recommended for mild to moderate tics because of the possibility of long-term side effects, such as tardive dyskinesia. When an antipsychotic

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      agent is necessary for a given child, an atypical antipsychotic agent should be tried first.

      TABLE 7.7. APPROXIMATE RATIOS OF 5-HYDROXYTRYPTAMINE 2a TO DOPAMINERGIC RECEPTOR AFFINITIESa FOR SELECTED ANTIPSYCHOTIC AGENTS

      Agent 5-HT2a D2 Approximate Ratio (5-HT2a/D2)
      Haloperidol 36.0 4.0 9.0
      Pimozide 13.0 2.5 5.2
      Risperidone 0.2 3.3 0.06
      Ziprasidone 0.4 4.8 0.09
      Olanzapine 4.0 11.0 0.36
      aAffinities are based on published data from product literature and are expressed as approximate Ki values in M. Lower values of Ki indicate higher receptor affinities.
      Abbreviations: D2, dopamine-2 receptor; 5-HT2a, 5-hydroxytryptamine-2a.

      • Atypical antipsychotic agents. A characteristic of atyp-ical antipsychotic agents is their stronger affinity for 5-hydroxytryptamine-2 (5-HT2) receptors compared with their affinity for D2 receptors. Table 7.7 compares the ratios of these affinities for some of the antipsychotic agents used in tic disorders. Remembering that antipsychotic agents also differ from each other in many other ways is also important; another key difference among them is their degree of receptor occupancy at therapeutic concentrations. Haloperidol's usual occupancy rate is greater than 80%, the level above which extrapyramidal side effects are more likely.

        Open studies of risperidone suggest that it can be beneficial for TD at low to moderate doses (1.5 mg per day). Risperidone can be initiated at 0.25 mg per day in children and can be titrated upward gradually. Older youth and adults can be started on 0.5 mg per day. Open-label studies and case reports also suggest that olanzapine can be beneficial in the treatment of TD, with doses in the 2.5-mg to 10-mg range. A placebo-controlled trial of ziprasidone showed that agent to be significantly more effective than the placebo, and some clinicians report favorable use of quetiapine.

        The most common and potentially problematic side effect with the atypical antipsychotic agents is weight gain, which can be significant in young patients. Increased insulin resistance may develop as a secondary side effect. These effects should be anticipated with parents so that an active exercise program and dietary monitoring can be initiated with treatment.

      • Conventional agents. Children can be started at doses of 0.25 mg per day of haloperidol, an amount that can be increased by 0.25 mg weekly or biweekly. Adolescents or adults can be started on 0.5 mg per day, with weekly or biweekly increases in 0.5 mg increments. Most patients will respond to a total daily dose of 5 mg or less, although a few adults may need a higher daily dose (i.e., up to 10 to 15 mg).

        Pimozide (see Chapter 4) is an alternative agent that may cause fewer side effects than haloperidol. However, because pimozide also has T-type calcium channel blocking properties,

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        normal cardiac function, which can be ascertained from cardiac assessment and an electrocardiogram, is a prerequisite to its use. Pimozide, which is approximately half as potent as haloperidol, should be started at 0.5 to 1 mg per day for children and at about 1 mg per day for adolescents or adults. Most patients will require less than 10 mg per day.

        The common side effects of conventional antipsychotic agents include weight gain, sedation, and dysphoric syndromes. Dosage reduction or switching to an alternative drug is the best way to respond to these side effects. Fortunately, acute dystonic reactions and tardive dyskinesia do not appear to be common sequelae of conventional antipsychotic agent use at the typical dosages used for tic disorder patients; nevertheless, clinicians should be alert to the possibility of these reactions and should consider appropriate interventions (e.g., antiparkinsonian agents, dosage reduction) as needed.

    • Alternative treatments. Nicotine has been reported to reduce tics through cholinergically mediated effects when administered in a transdermal form either alone or in combination with an antipsychotic agent. Mecamylamine, a nicotinic cholinergic receptor antagonist, has also been reported to reduce tics in recent studies. Additionally, baclofen, which has -aminobutyric acid (GABA)ergic effects, was found in a recent double-blind placebo-controlled trial to reduce some TD symptoms more effectively than the placebo.

    • Proserotonergic agents. Some of these agents, such as fluoxetine, fluvoxamine, sertraline, and clomipramine, represent alternative treatments for some of the OCD symptoms in TD. Because many TD patients have obsessions or compulsions as their primary symptoms of clinical concern, they are candidates for treatment with a selective serotonin reuptake inhibitor (SSRI). Fluvoxamine, sertraline, and fluoxetine have been studied in pediatric OCD (see Chapter 21), and thus they are recommended as first-line agents if a therapeutic trial is indicated. The established efficacy and side effect profiles are quite similar; the choice of agent may rest on pharmacokinetic or drug interaction differences (see Chapter 29).

      Fluoxetine typically is started at 2.5 to 5 mg per day for children and at 5 to 10 mg per day for adolescents or adults. Sertraline is typically started at 12.5 to 25 mg per day in children and at 50 mg per day in adolescents or adults; fluvoxamine is started at similar doses. An SSRI used in combination with an antipsychotic agent can be useful for controlling both tics and obsessive-compulsive symptoms. Common side effects in youths include behavioral activation, agitation, insomnia, headaches, gastrointestinal effects, or sexual dysfunction. Because the behavioral activation may abate over time, reducing the dose temporarily for observation is helpful. Switching to another SSRI or to clomipramine may be necessary if the OCD symptoms do not abate.

      When several SSRI trials have not been helpful, a trial of clomipramine should be considered. Its efficacy in pediatric OCD has been established since the mid-1980s. Clomipramine should be started at 10 to 25 mg per day for children and 25 to 50 mg per day for adolescents or adults and should then be titrated gradually upward. Total dosage ranges are typically 100 to 300 mg per day; blood levels may be obtained, but, at present, no therapeutic range is established. Common side effects are those seen typically with other tricyclic antidepressants, including sedation, dry mouth, constipation, blurring of vision, and weight gain. Baseline and follow-up electrocardiograms should be monitored.

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  • Attention deficit hyperactivity disorder treatments (see Chapter 22). Historically, controversy has existed about the role of stimulants in the treatment of tic disorders and TD; older reports indicated that stimulants may induce or increase tics in children. However, several investigators recently reported the significant beneficial effects of stimulants either alone or in combination with clonidine when the ADHD symptoms are the primary clinical concern. Results of a recently completed National Institutes of Health-sponsored randomized clinical trial comparing methylphenidate, clonidine, and their combination with a placebo indicated that the combination was more effective than the placebo in treatment of both tics and ADHD symptoms. Results also indicated that methylphenidate treatment did not increase tics when used either alone or in combination with clonidine. In addition, tricyclic antidepressants, such as desipramine and nortriptyline, have been reported in both open and double-blind trials to be effective in children with ADHD and comorbid tics.

C. Behavioral Therapies

Behavioral paradigms can be useful for some patients with tic disorders. Simple behavioral approaches can be used to reduce stress and to contain the tic symptoms. Specific paradigms are indicated for obsessions, compulsions, and possibly some complex motor tics. Relaxation techniques, such as deep breathing, guided imagery, and the use of relaxation tapes, can be useful for the anxious or stressed TD patient. Habit reversal therapy, which consists of isometric muscle tensing to oppose motor tics, may be useful in the treatment of tic disorder. Opposing muscles are contracted when the urge to have a tic develops (e.g., having a child chew gum [preferably sugarless] to reduce facial tics). This competing response theoretically prevents the emergence of the tic. Preliminary results of a study currently underway comparing habit reversal therapy with monitoring indicate that habit reversal may be an effective behavioral treatment for tics in older patients. Tic substitution (i.e., substituting a more socially tolerable tic for a less socially tolerable one) is another alternative that may be helpful.

Contingency management emphasizes positive reinforcement and avoids increased guilt or anxiety. Massed negative practice (i.e., self-imposed forceful repetition of the tic) for a period of time (with intervals for rest) is beneficial for some patients. The patient performs the tic deliberately for as many times as possible. The ensuing tiredness theoretically produces a decrease in tic frequency. Although behavioral approaches typically work best for older youth and adults, even younger children can be taught some of the basic techniques with their parents. Behavioral treatment may be more acceptable than pharmacotherapy to some families, especially when the tic symptoms are mild.

D. Psychoeducational Approaches

Education of patients and their families is always essential. Opportunities for discussion of patients' questions and concerns should be made available early in the diagnostic process and in ongoing treatment. Referral to the national Tourette Syndrome Association (TSA) or to local chapters of the TSA is an excellent way to maintain ongoing education and support. The national TSA is located at 40-42 Bell Boulevard; Bayside, New York, 11361 (718-224-2999). The TSA provides useful information through its website (http://www.tsa-usa.org).

E. Psychotherapy

Individual, group, and family therapies are supportive adjuncts to pharmacotherapy. Individual supportive therapy that incorporates the principles of cognitive-behavior therapy is indicated for patients having difficulty adjusting to their disorder with their peers or family or with school or occupational functioning. Supportive therapy is particularly useful when evidence of moderate stress or anxiety or another comorbid psychiatric disorder responsive to this form of psychotherapy (e.g., mild depression) is

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observed. It can help to restore or to maintain self-esteem or to promote mastery and coping.

Family work or therapy is extremely useful in TD. Both parents and any other involved family members should be seen at least once as part of the initial evaluation. Ongoing family therapy is indicated when family development (i.e., growth and maturation) has slowed or halted because of the focus on the patient with the tic disorder. It may also be helpful with maladaptive reactions from or to siblings or with specific symptoms that are affecting the entire family.

Group therapy is another important adjunct. Support and education for parents and family members of patients with TD can be found in support groups sponsored by the national TSA. Informal, unstructured activities can be arranged through local TSA chapters. Formal structured support is also possible through psychoeducationally oriented groups.

F. School and Occupational Interventions

Learning problems and classroom difficulties are seen frequently in patients with tic disorders. Specific developmental disorders and ADHD-related or OCD-related symptoms may interfere with academic performance. Clinicians should be available to provide consultation, guidance, and education to teachers or employers of patients with tic disorders. Useful special educational interventions include creating moderate task-oriented support and individualized work or lesson plans. Flexibility is a key element in educational and occupational intervention. To promote tic control, optional time-outs from class or work settings might be needed. Time limits may have to be extended or eliminated for exams. Adaptive (as opposed to regular) physical education can be particularly helpful for children with TD; this can be arranged through special education departments in local schools. Programs should be tailored to each child's needs, strengths, and weaknesses. Specific workplace interventions include structured tasks, organization of tasks into smaller units, flexible time limits, and ample physical space.

ADDITIONAL READING

Apter A, Pauls DL, Bleich A, et al. An epidemiologic study of Gilles de la Tourette's syndrome in Israel. Arch Gen Psychiatry 1993;50:734 738.

Bruun R, Budman C. The course and prognosis of Tourette syndrome. In: Jankovic J, ed. Tourette syndrome. Philadelphia: WB Saunders, 1997:291 298. Neurologic Clinics series. Vol. 15.

Burd L, Kerbeshian J, Wikenheiser M, et al. Prevalence of Gilles de la Tourette's syndrome in North Dakota adults. Am J Psychiatry 1986;143:787 788.

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Coffey B, Biederman J, Geller D, et al. The course of Tourette's disorder: a literature review. Harv Rev Psychiatry 2000;8:192 198.

Coffey B, Miguel E, Savage C, et al. Tourette's disorder and related problems: a review and update. Harv Rev Psychiatry 1994;2:121 132.

Garvey M, Perlmutter S, Allen AJ, et al. A pilot study of penicillin prophylaxis for neuropsychiatric disorders triggered by streptococcal infections. Biol Psychiatry 1999;45:1564 1571.

Leckman J, Zhang H, Vitale A, et al. Course of tic severity in Tourette syndrome: the first two decades. Pediatrics 1998;102:14 19.

Robertson M. Tourette syndrome, associated conditions, and the complexities of treatment. Brain 2000;123:425 462.

Singer H. Current issues in Tourette syndrome. Move Disord 2000;6:1051 1063.

Spencer T, Biederman J, Coffey B, et al. The four-year course of tic disorders in boys with ADHD. Arch Gen Psychiatry 1999;56:842 847.

Swedo S, Leonard H., Garvey M, et al. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: a clinical description of the first 50 cases. Am J Psychiatry 1998;155:264 271.

Tourette Syndrome Association International Consortium for Genetics. A complete genome screen in sib pairs affected by Gilles de la Tourette syndrome. Am J Hum Genet 1999;65:1428 1436.

Tourette's Syndrome Study Group. Treatment of ADHD in children with tics: a randomized controlled trial. Neurology 2002;58:527 536.

The following brochures are available from the Tourette Syndrome Association and its local chapters:

Coping with Tourette Syndrome a Parent's Viewpoint

Coping with Tourette Syndrome in the Classroom

Facts You Should Know About the Genetics of Tourette Syndrome

Know Your Rights: Financial Resources

Know Your Rights: Services Available

Tourette Syndrome: Questions and Answers



Manual of Psychiatric Therapeutics Paperback
Manual of Psychiatric Therapeutics: Practical Psychopharmacology and Psychiatry (Little, Browns Paperback Book Series)
ISBN: 0316782203
EAN: 2147483647
Year: 2002
Pages: 37

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