TOURETTE’S SYNDROME, TICS AND OBSESSIVE-COMPULSIVE DISORDERS

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CHAPTER 17 TOURETTE’S SYNDROME, TICS AND OBSESSIVE-COMPULSIVE DISORDERS

Anette Schrag, Mary M. Robertson

TICS AND GILLES DE LA TOURETTE SYNDROME

Once considered a rarity, Gilles de la Tourette syndrome is now recognized to be a relatively common disorder, which may be associated with considerable psychiatric comorbidity and impaired psychosocial functioning. Gilles de la Tourette syndrome is defined by the Diagnostic and Statistical Manual of Mental Disorders, 4th edition, Text Revision (DSM-IV-TR) as a combination of multiple motor tics and at least one vocal tic that cannot be explained by another cause and has persisted for at least 1 year.1,2 The current criteria no longer require that these tics produce significant distress or impairment, and a large proportion of people who fulfill these criteria are not aware of their tics or are not disturbed by them. However, in the tertiary referral setting and in patients with more severe or frequent tics or with associated comorbidity, there are both considerable psychosocial impairment and reduced quality of life.3 Gilles de la Tourette syndrome is frequently undiagnosed; tics may be misinterpreted as a “nervous habits” or, particularly in children, as inattention or inability to sit still. On occasion, tics are mistaken for another movement disorder or a psychiatric disease.

In addition to Gilles de la Tourette syndrome, tics can occur in chronic tic disorders that are purely motor or verbal and in transient tic disorders that do not last more than 1 year. The latter are common phenomena in childhood. Tics may also occur as a side effect of drugs or toxins and may be a manifestation of an underlying neurological disorder, usually associated with other phenomena but occasionally as the first or only manifesting symptom (see later discussion).

Characteristics of Tics

Tics are abrupt, recurrent, and stereotyped but nonrhythmic movements. They can affect any body part, can be simple or complex, and manifest with motor or vocal actions. Examples of simple motor tics are eye blinking, nose twitching, eye rolling, and head nodding, and simple verbal tics include sniffing, throat clearing, or coughing. Examples of complex motor tics are jumping, touching, or twirling, and complex verbal tics include barking or uttering a string of words. Many patients experience a premonitory sensation before a tic, which can be localized or generalized. Characteristically, tics increase with stress and anxiety, but also with relaxation, and can be suppressed for a period of time by concentration. However, this typically leads to a buildup of tension, and there is often a rebound after the suppression of tics.

In Gilles de la Tourette syndrome, tics wax and wane over time, moving from one body part to another or changing characteristics. In addition to tics, other phenomena in this syndrome include echolalia (copying what other people say), echopraxia (copying what other people do), palilalia (repeating the last word or part of a sentence) and palipraxia (repeating the last action). Coprolalia (inappropriate, involuntary swearing) and copropraxia (inappropriate, involuntary obscene gestures) occur only in about 10% to 15% of patients with Gilles de la Tourette syndrome and are frequently disguised (e.g., by coughing or transformation of the word). The onset of motor tics in this syndrome is in childhood and occurs between ages 2 and 21 years; on average, onset occurs at ages 5 to 7 years. Verbal tics typically manifest a few years later, and coprolalia has a mean onset at age 15 years.

Differential Diagnosis of Tics

Other brief movement disorders may be difficult to distinguish from tics. Tics may particularly resemble dystonia, tremor, myoclonus, chorea, and akathisia (for a review of hyperkinetic movement disorders, see Chapters 33 to 37 Chapter 34 Chapter 35 Chapter 36 Chapter 37). They also need to be distinguished from mannerisms (bizarre execution of purposeful acts), stereotypies (purposeless, repetitive movements often over long periods of time, as in a learning disability), and other medical conditions, such as coughing or sniffing in upper respiratory tract infections or eye blinking in allergy or blepharospasm. Particularly difficult may be the distinction between tics and other features of Gilles de la Tourette syndrome, such as obsessive-compulsive behaviors (OCBs), attention deficit/hyperactivity disorder (ADHD), antisocial behaviors and movement disorders associated with treatment.4 These differentiations are particularly important for appropriate pharmacological management.

Epidemiology

Tics occur in 3% to 22% of children at some stage during their development5 but are transient in the majority. The more severe Gilles de la Tourette syndrome affects approximately 1% of chil dren, and prevalence rates range from 0.4 to 1.8%.6–13 However, although the disorder typically starts in childhood, on average between the ages of 5 and 7 years,14,15 and typically increases until the age of 13 years, it often improves in adolescence so that by the age of 18 years, 50% of those affected are virtually free of tics.16 The prevalence rate is higher in special educational populations, such as those with learning difficulties17 or autism.18 About three to four times as many boys as girls are affected.14 Prevalence rates and clinical characteristics are broadly similar across countries.14

In rare cases, tic disorders with both motor tics and verbalizations begin in adulthood. Some of these patients have been described to have had compulsive tendencies in childhood or a family history of tics or OCB. In comparison with patients with Gilles de la Tourette syndrome that started in childhood, patients with adult-onset tic disorder more often had a potential trigger event, have more severe symptoms and greater social morbidity, and increased sensitivity and poorer response to neuroleptics.19

Diagnosis

Tics and Gilles de la Tourette syndrome are clinical diagnoses. In cases of classic Gilles de la Tourette syndrome, no diagnostic tests are required. However, atypical cases, such as those without waxing and waning over time, those with adult onset, and particularly those with abnormalities on neurological examination, should be further investigated, including measurements of copper and ceruloplasmin for Wilson’s disease, full blood count for acanthocytes, and magnetic resonance imaging of the brain. Neuroimaging has also provided insight in the pathophysiology of Gilles de la Tourette syndrome: reduced volumes and abnormal asymmetry as well as altered dopamine metabolism of the basal ganglia, particularly the caudate20,21, and frontal lobe abnormalities,22 all of which implicate the frontal-striatal-thalamic-frontal circuitry.

Etiology

Tics can be symptomatic; for example, in neurodegenerative conditions, such as Huntington’s disease, they can be the first manifesting symptom. They can also represent a sequela of trauma or encephalitis, or they can represent extrapyramidal side effects of neuroleptic medication or cocaine abuse. However, the most common cause for chronic tic disorders is Gilles de la Tourette syndrome; this applies to adults, with childhood tics that have recurred or in whom previous tics may have been unnoticed or forgotten until an increase in tic severity brought them to medical attention.

Suggestions for the etiology of Gilles de la Tourette syndrome have included genetic influences, infections, and perinatal difficulties. There is a wealth of evidence pointing toward genetic causes, including family studies, which suggested an autosomal dominant inheritance pattern with variable expression and penetrance.23–25 There is also growing evidence for bilineal transmission, with the father typically affected by childhood tics and the mother by symptoms of obsessive-compulsive disorder (OCD).26–28 Genetic studies have led to the identification of several regions of interest on chromosomes 2, 4, 8, and 1129–32 and, more recently regions of interest on the chromosomes 5, 10, 13,33 7,34 and 18.35 In addition, the DRD4 and MOA-A genes have been implicated,34,36 and linkage to chromosome 17 has been demonstrated.37 However, despite many years of research by a number of groups worldwide, no single genetic cause has been found for Gilles de la Tourette syndrome. This suggests that other factors also play a role in the etiology of this disorder.

An increasingly popular hypothesis suggests that Gilles de la Tourette syndrome is the product of an interaction between a genetic vulnerability and environmental factors. Stressors at various times of the life cycle have been implicated, particularly perinatal injury, but also stressors during pregnancy, such as severe nausea, vomiting, and antiemetic medication, which may alter dopaminergic receptors.16,38–41

Particularly intriguing has been the association of group A β-hemolytic streptococcal infections with a syndrome of sudden-onset neuropsychiatric disturbances, including OCD, tics, and other psychopathology in children. This syndrome has been termed pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS).42 More recently, laboratory evidence of streptococcal infection in patients with Gilles de la Tourette syndrome has been reported, including elevated antistreptolysin O titers and anti–basal ganglia antibodies in up to 25% of patients with Gilles de la Tourette syndrome.43,44 An autoimmune mechanism has therefore been suggested as a contributor to the development of tics in this syndrome.44,45 Although the majority of studies have supported this notion,43–46 others have disputed this association,47,48 and whether anti–basal ganglia antibodies play a role in the development of Gilles de la Tourette syndrome currently remains a controversy.17

Although it seems clear that there is a strong genetic component in the etiology of Gilles de la Tourette syndrome and it seems that streptococcal infection causes the syndrome, it may be that individuals inherit a susceptibility to this syndrome and that environmental factors such as perinatal injury or an autoimmune response to streptococcal infections trigger the development of this syndrome in some individuals.

Psychopathology

Gilles de la Tourette syndrome is associated with increased rates of a number of comorbid psychiatric conditions (see Robertson, 200049 and 200350). Although some of these are likely to represent a manifestation of or be integral to Gilles de la Tourette syndrome, others may be the consequence of the social and emotional consequences of this disorder. An investigation of 3500 patients with Gilles de la Tourette syndrome worldwide demonstrated that across all ages, 88% of individuals had associated psychiatric comorbidity, and male patients were more likely to have comorbid disorders.14 Only those with comorbid disorders had more severe behavioral problems such as anger control problems and self-injurious behavior, as well as sleep difficulties and coprolalia. The presence of such behavioral problems should therefore alert the clinician to the possible presence of comorbidity, the management of which is often at least as important as tic reduction.

The spectrum of comorbid disorders includes OCD, other anxiety disorders, mood disorders, ADHD, and other behavior disorders, including self-injurious behavior.51 Although the exact relationship of these to Gilles de la Tourette syndrome is unclear, a strong association exists between Gilles de la Tourette syndrome and OCD and OCB, and a number of studies have suggested that these reflect the variable expression of a single disorder.24,26,27,50 OCD in patients with Gilles de la Tourette syndrome has, however, been found to differ in clinical manifestation from primary OCD, with predominant checking, counting, and symmetry obsessions and less frequent obsessions with contamination and violence.52–57 The rate of ADHD is also increased in patients with Gilles de la Tourette syndrome, and although the relationship between Gilles de la Tourette syndrome and ADHD is less clear, it has been suggested that some types are related genetically to Gilles de la Tourette syndrome. Depending on whether or not the International Classification of Diseases and Health-Related Problems, 10th Revision, or DSM-IV-TR criteria are used, the prevalence of ADHD in youngsters is between 1% and 9%.58 In patients with Gilles de la Tourette syndrome, the prevalence of ADHD is much greater and may be as high as 60%.14 This is not confined to chronic populations: Even in an epidemiological study conducted on members of the Israeli Defense Force, the prevalence of ADHD in Gilles de la Tourette syndrome was 8%, in comparison with 4% in the population without Gilles de la Tourette syndrome, a difference that was statistically significant.59 Personality disorders in adulthood in patients with Gilles de la Tourette syndrome are likely to be related to comorbid ADHD in childhood rather than to the syndrome itself. Whether other psychiatric comorbid conditions such as conduct disorder, oppositional-defiant disorder, personality disorder, rage, and impulsivity are clearly more prevalent in patients with Gilles de la Tourette syndrome or their apparent prevalence is a result of referral bias in Gilles de la Tourette syndrome clinics is currently unknown. The multiple medications used for Gilles de la Tourette syndrome (see later discussion) may lead to increase. Anxiety and cognitive disorders, and anxiety may occur as a result of having Gilles de la Tourette syndrome and its social and personal consequences. The rate of depression, on the other hand, is clearly increased among patients with Gilles de la Tourette syndrome and is likely to be multifactorial in origin.60–63

Prognosis

Clearly, for symptomatic tics in the context of another neurological disorder or as a drug effect, the prognosis is associated with the underlying disorder. The prognosis of individuals with Gilles de la Tourette syndrome varies widely; whereas those with mild tics without coprolalia or associated comorbidity mostly do not suffer impairment of social or personal function, those at the other end of the spectrum can be severely disabled. Children may be disadvantaged in school, particularly if comorbidity is present,64,65 but, as mentioned previously, tics often improve in adolescence.15,57 When the affected individuals and their environments receive appropriate explanation of this disorder and understand it, most do not need regular follow-up. Adult-onset cases appear to have worse morbidity and worse response to treatment, but this is rare. Overall, health-related quality of life has been shown to be worse in patients with Gilles de la Tourette syndrome than in controls, although it is better than in patients with intractable epilepsy.3 Factors associated with poorer health-related quality of life in this study in a tertiary referral center were employment status, tic severity, obsessive-compulsive symptoms, anxiety, and depression.

Management

Many individuals with mild Gilles de la Tourette syndrome are not aware of their tics or do not find them bothersome and may never come to medical attention. For those who have come to medical attention, a diagnosis of their condition, explanation, and reassurance are often all that is required. When medication is considered in more severe cases, patient and physician should take into account the severity, frequency, and interference of tics; the presence and severity of comorbid conditions; the patient’s life style, requirements, expectations, and attitudes; and the long-term nature of pharmacological treatments, which may have reversible or irreversible side effects. Ideally, treatment should be multidisciplinary.

Nonpharmacological Treatment

Supportive psychotherapy and psychological education are very important for all patients and their families, particularly if the patients are young. More specific behavioral treatment has been shown to produce better results than psychotherapy in adult patients with Gilles de la Tourette syndrome and include habit reversal training, graded exposure, social skills training, imaginal exposure, massed negative practice, contingency management, relaxation training, and biofeedback.66,67

Pharmacological Treatment

Pharmacological treatment is based primarily on neuroleptic medication, and the individual’s response is idiosyncratic. Thus, an individual may respond to one particular neuroleptic agent but not another. Haloperidol, pimozide, sulpiride, and tiapride have all been shown to be more effective than placebo, and the doses required for tics in Gilles de la Tourette syndrome are much lower than those used for schizophrenia or mania. Although side effects at these doses are less common, all these agents carry the risk of extrapyramidal side effects, including acute dystonic reactions, parkinsonism, and tardive dyskinesia. In addition, sedation, cognitive side effects, depression, and social phobias can be dose limiting. Pimozide also must be used with caution because it has a higher rate of cardiac side effects than do other neuroleptic medications. In addition, an increase in prolactin levels with these drugs may necessitate discontinuation.

The newer “atypical” antipsychotic agents have been demonstrated to be useful in treating patients with Gilles de la Tourette syndrome. Their chief advantage is the lower risk of extrapyramidal side effects. The main side effect is weight gain and, in some individuals, the precipitation of diabetes mellitus. It is therefore recommended that fasting glucose levels be checked in patients, particularly if they have put on weight. The atypical antipsychotic agents successfully used for treatment of Gilles de la Tourette syndrome have included risperidone,68 olanzapine,69 quetiapine,70 aripiprazole,71 and ziprasidone.72 It has also been suggested that quetiapine does not lead to hyperprolactinemia73 and may therefore merit further studies in patients with Gilles de la Tourette syndrome. In patients with severe vocal tics, which may not respond well to oral pharmacological treatment, botulinum toxin injections may be useful.74 Other suggested alternatives for the treatment of Gilles de la Tourette syndrome have included the neuroleptics amisulpride, aripiprazole, ziprasidone, fluphenazine, metoclopramide, piquindone, and tetrabenazine and agents from other substance groups, such as clonazepam, calcium channel antagonists, celecoxib, dopamine agonists, and selegiline. In severe, medically intractable cases, various surgical approaches have been tried with little success.75 However, in a literature review, Rauch and associates76 suggested that there is no compelling evidence that any neurosurgical procedure is superior to all others, and such surgery is not recommended outside specialist centers. Deep brain stimulation of the thalamus, which is largely reversible, is currently being explored as a treatment option for severe tics and OCD.77

The treatment of comorbid conditions requires additional drug choices. OCD and OCB often respond to selective serotonin reuptake inhibitors or the tricyclic antidepressant clomipramine, which inhibits both serotonin and noradrenaline uptake. In some countries, the use of some of these agents (e.g., paroxetine) is contraindicated in children. When ADHD exists comorbidly, the α2-adrenergic agonist clonidine and, in the United States, guanfacine can be useful for tics, impulse control, and ADHD, but electrocardiography and blood pressure control are recommended for patients taking these drugs. These agents must not be discontinued suddenly, because of rebound hypertension. Children with ADHD may require the addition of a psychostimulant such as methylphenidate. Previous concerns about exacerbation of tics with this medication have not been substantiated, and the management of ADHD may be more important than that of tics. An alternative may be the nonstimulant selective norepinephrine reuptake inhibitor atomoxetine.78,79 Depression in Gilles de la Tourette syndrome should be treated like primary depression or, for depression associated with other chronic disorders, by using cognitive-behavioral approaches, education, psychotherapeutic treatments, and pharmacotherapy.

Conclusions and Recommendations

Tics and Gilles de la Tourette syndrome occur more frequently than previously believed but are frequently unrecognized. The diagnoses of tics and Gilles de la Tourette syndrome are clinical, but diagnostic differentiation of tics from other movement disorders, associated phenomena in Gilles de la Tourette syndrome, and side effects of medication can be difficult. Tics may also occur in the context of other neurological conditions and as a consequence of neuroleptic treatment or drug abuse. In adults and those presenting with atypical features, these differential diagnoses should be explored. However, only in patients with atypical features are investigations required. In Gilles de la Tourette syndrome, there is no doubt that genetic factors play an important role in its etiology, but no single gene has been identified, and environmental factors such as infection or perinatal injury may also play a role.

Tics in Gilles de la Tourette syndrome are often associated with significant psychiatric comorbidity and impaired psychosocial functioning and quality of life. Although tics in Gilles de la Tourette syndrome often improve in adolescence, patients with a more severe tic disorder that interferes with their lives and those with associated psychiatric comorbid conditions often require long-term treatment with pharmacological or nonpharmacological approaches. These should be tailored to the individual’s needs, with consideration of comorbid conditions and the context of the individual’s life.

OBSESSIVE-COMPULSIVE DISORDER

Obsessions are intrusive and recurrent thoughts, which include intrusive doubts, images, impulses, or ruminations (continuous pondering). They are recognized by affected individuals as their own thoughts, but they are characteristically egodystonic—that is, unwelcome and uncomfortable—to the individual, who usually tries to avoid or suppress them.52 Common obsessions are concerned with contamination, violence, sex, blasphemy, and numbers. Rarer obsessions are arithmomania (obsession with counting), onomatomania (the desire to utter a forbidden word), and folie de pourquoi (irresistible habit of repetitively asking the same banal question).

Compulsions are carried out in response to these obsessions in a stereotyped manner, with reluctance, in order to neutralize or prevent a dreaded event. However, they are often not realistically related to the obsession, are considered unreasonable by the individual, and are excessive and time consuming. If the compulsion is suppressed, anxiety results until the compulsive behavior is performed. Common compulsive behaviors include washing, checking, arranging, counting, or mental rituals. Patients try to avoid situations that may provoke obsessions.

Although obsessions and compulsions are common in the general population, a diagnosis of OCD according to DSM-IV-TR requires that obsessions and compulsions cause marked distress or significantly interfere with a person’s functioning and do not occur in the context of a medical illness. There is considerable phenotypic variability of obsessions and compulsions, and the existence of specific subtypes has therefore been postulated: for example, familial and related to tic disorders, familial and unrelated to tics, and sporadic OCD.80 However, the existence of these different subtypes has been controversial.81

Epidemiology

OCD has a lifetime prevalence of 1.8% to 3.5% in the population with an onset in childhood or adolescence and a slight preponderance among girls.82–84 OCBs are much more common and may be part of the spectrum of normal behavior. OCD occurs worldwide with similar core features, but the content of the obsessions appears to be related to cultural context.85

Diagnosis

Obsessions and compulsions are diagnosed clinically. Obsessions and compulsions should be distinguished from psychosis, in which voices or thoughts are experienced as coming from outside; from impulsive thoughts, which are egosyntonic (i.e., not uncomfortable or alien); and from rituals, which are purposeful actions, often with a cultural significance. Tics and compulsions can be difficult to distinguish, and they overlap. However, compulsions are typically preceded by obsessions and cognitions, and suppression of compulsions is typically followed by anxiety, whereas tics are typically preceded premonitory sensations and an urge to perform the tic, with no anxiety after suppression but physical discomfort and frequent rebound of tics afterward.

Obsessions and compulsions are also common in other psychiatric disorders, including depression, schizophrenia, and obsessional (anankastic) personality disorder, and they overlap with Gilles de la Tourette syndrome, as discussed previously. Segregation analysis in families with Gilles de la Tourette syndrome and OCD suggested that OCD and Gilles de la Tourette syndrome are variant expressions of the same syndrome. A concurrent obsessional (anankastic) personality is present in about 70% of cases.52 They may also occur in generalized anxiety disorder, puerperal illness (as a fear of harming the baby), anorexia nervosa, Huntington’s disease, encephalitis lethargica, PANDAS, manganese poisoning and after head injury. In these psychiatric and neurological disorders, other features are present, but even in pure OCD, soft neurological signs such as astereognosia or agraphesthesia may be present.52

Etiology

Obsessions were originally believed to be rooted in repressed impulses or in an aggressive or sexual nature,86 and other explanations have included obsessions as a result of aberrant learning.87 An increase in severity of OCD is also often seen when depression or stressful life events occur. However, obsessions and compulsions are seen in the context of a number of neurological disorders, such as Huntington’s disease or encephalitis lethargica, implicating underlying brain abnormalities, particularly in the frontal cortex and basal ganglia. Functional imaging studies and neuropsychological testing also provide increasing evidence that OCD is associated with abnormal functioning of the orbitofrontal cortex, the cingulate, and the caudate, and biochemical abnormalities, especially involving serotonin, are believed to be important in the pathophysiology of OCD.88

Increased rates of obsessions and compulsions in families of patients with OCD suggest that genetic factors play a role in the etiology of OCD, and twin studies with higher concordance rates in monozygous twins than in dizygous twins have supported the importance of genetic factors. In addition, abrupt onset or exacerbations of OCD or tics or both have been described after streptococcal infections (see previous discussion), suggestive of environmental causes. Neuroimaging studies reveal increased basal ganglia volumes, and the proposed cause involves the cross-reaction of streptococcal antibodies with basal ganglia tissue. A genetic susceptibility to PANDAS has been postulated.89

Prognosis

Mild cases of obsessions and compulsions are often self-limited within 1 year. OCD is a chronic disorder but typically runs a fluctuating course with periods of long remissions and the greatest prevalence in mid-adult life. A meta-analysis of studies with up to 16 years’ follow-up revealed persistence rates of 41% for full OCD and 60% for full or subthreshold OCD.90 Comorbid psychiatric illness and poor initial treatment response were poor prognostic factors.

Depression and abuse of alcohol and anxiolytics is common. Quality of life has been found to be significantly related to severity of obsessions, whereas the severity of compulsive rituals did not affect quality-of-life ratings. However, the single greatest predictor of poor quality of life was comorbid depression severity,91 and suicide rates are increased, particularly in patients with comorbid depression. This is contrary to previous notions that suicide is uncommon in patients with OCD.

Management

In many cases, obsessions and compulsions do not necessitate treatment, and the fluctuating course needs to be considered before treatment starts. In cases in which treatment is required, cognitive-behavioral therapy has been successful, including exposure and response prevention for compulsions, and habituation training and thought-stopping for obsessions.92 Psychoeducation can also be a valuable source.

Pharmacological treatment is often effective, although up to 50% of patients may require more than one treatment trial. Effect medication includes the serotonin reuptake inhibitors (e.g., citalopram, fluoxetine, fluvoxamine, paroxetine, and sertraline), serotonin-norepinephrine reuptake inhibitors (e.g., venlafaxine), and the tricyclic antidepressant clomipramine (which has a less favorable side effect profile). Of importance is that the doses of these medications required for treatment of OCD are often higher than those for depression. In addition, in severe cases, augmentation with neuroleptic medication, including risperidone and olanzapine, has been suggested.

In cases in which all other classic treatments have failed after a minimum of 5 years, psychosurgery is occasionally considered. Capsulotomy, cingulotomy, subcaudate tractotomy, and limbic leukotomy, performed by radiofrequency thermolesions or radiosurgery,93–96 and the largely reversible deep brain stimulation97 have all been used. These surgical approaches are aimed at altering the neural circuits between the frontal lobes and different structures of the limbic system, but they are used very rarely.

Conclusions and Recommendations

OCD is a common disorder with a wide phenotype, and it overlaps with Gilles de la Tourette syndrome. There may be different subtypes of OCD, and although streptococcal infection has been associated with OCD and with symptoms of Gilles de la Tourette syndrome, genetic factors are clearly implicated in OCD. OCD can also occur in the context of other psychiatric or neurological syndromes and is then associated with greater morbidity and worse quality of life. Management comprises cognitive-behavioral treatment and a number of pharmacological treatment options; in extremely rare cases of intractable and disabling OCD, patients may undergo surgery. Future research will address the etiological questions relating to genetic and environmental causes of OCD, its overlap with other psychiatric conditions, and improved management options.

KEY POINTS

• Gilles de la Tourette syndrome affects approximately 0.5% to 1% of all school-aged children, but severity of tics often improves in adolescence.
• Tics also occur in other neurological disorders and as a result of neuroleptic medication or drug abuse, particularly in adults.
• There is a strong genetic component in the etiology of Gilles de la Tourette syndrome, but no single gene has been identified; it is likely to be multifactorial, including possible infectious or perinatal factors.
• Gilles de la Tourette syndrome is frequently associated with obsessive-compulsive symptoms and other psychopathology, including depression and ADHD; these affective and behavioral disorders are major contributors to impaired psychosocial functioning.
• Management of Gilles de la Tourette syndrome focuses on the aspects of the syndrome that are individually most important, including tics and the behavioral and affective disorders.
• OCD affect approximately 1.9% to 3.5% of the population, but milder obsessive-compulsive symptoms are more common.
• Obsessions are repetitive, intrusive thoughts that are unpleasant to the individual but are recognized as the individual’s own; compulsive behaviors are carried out to neutralize or prevent obsessions.
• Treatment of obsessive-compulsive symptoms includes cognitive-behavioral therapy, supportive therapy, and pharmacotherapy, including selective serotonin reuptake inhibitors and clomipramine.

Suggested Reading

Husted DS, Shapira NA. A review of the treatment for refractory obsessive-compulsive disorder: from medicine to deep brain stimulation. CNS Spectr. 2004;9:833-847.

Leckman JF. Phenomenology of tics and natural history of tic disorders. Brain Dev. 2003;25(Suppl 1):S24-S28.

Pauls DL. An update on the genetics of Gilles de la Tourette syndrome. Psychosom Res. 2003;55:7-12.

Robertson MM. Tourette syndrome, associated conditions and the complexities of treatment. Brain. 2000;123(Pt 3):425-462.

Singer HS. Tourette’s syndrome: from behaviour to biology. Lancet Neurol. 2005;4:149-159.

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