Chapter 89. Repetitive Behaviors

Parents often raise concerns about rhythmic and repetitive behaviors such as rocking, head banging, and thumb sucking. Some are common; others are unusual and idiosyncratic. The high prevalence and heterogeneity of repetitive behaviors raise questions about the origins and biological significance of these common pediatric concerns. Learning theorists contend that rhythmic movements begin as normal behaviors that are reinforced over time. If these activities are pleasurable or tension relieving, they tend to be repeated. However, prenatal ultrasounds show the human fetus sucking its thumb by the 28th week of gestation, suggesting that some complex repetitive movements represent genetically coded, species-specific behavior. Observations of picking and grooming behaviors among primates suggest that hair twisting and pulling may originate from social behavior of our human ancestors.

The brain is a rhythmic organ. The brainstem, hypothalamus, and other mesencephalic regions pulse and cycle, suggesting that some repetitive behaviors arise from normal neuronal regulation of the primitive brain. The emergence of abnormal repetitive behaviors after severe brain injury and the frequent stereotypies in individuals with severe cognitive disabilities indicate that human cortical functions may inhibit “deeper” and more primitive repetitive behaviors.

Regardless of their origins, repetitive behaviors are mediated by neurotransmitters. Observations of rhythmic movement disorders in cases of human amphetamine overdose and following intracerebral injection of dopamine and amphetamines in animal studies have implicated dopamine (which mediates behavioral reward systems), serotonin, gamma-aminobutyric acid (GABA), and endorphins.

The clinical significance of repetitive behaviors in children depends on the characteristics of the behavior (the kind of movements, their frequency, and timing), the context (the age of the child, when it occurs, what makes it worse), and the impact of the behavior on health and function. For example, thumb sucking occurs commonly enough in children of age 3 or 4 years to be considered normal, but it may cause an orthodontic problem if it occurs very frequently. It is normal for a school-aged child to suck his thumb briefly as he falls asleep, but habitual thumb sucking in the classroom may indicate a psychological problem related to anxiety or inhibition and may interfere with social function.

A consideration of the function and meaning of the behavior helps us in classifying repetitive behaviors as habits, stereotypies, tics, or compulsions.

Habits

Habits are assembled routines that link sensory inputs with motor outputs through procedural learning. Common habits, including rocking, head rolling, head banging, hair twirling, thumb sucking, nail biting, nose touching, teeth grinding, and joint popping, are not strongly associated with neurodevelopmental disorders or neurological disability.

Most habits serve a self-soothing function at times of stress or boredom. Children engage in these behaviors voluntarily but may learn to inhibit the behaviors through social reinforcement (pleasing parents or avoiding humiliation in school). Indeed, some habits may develop through imitation and social reinforcement: Children may learn to pop their knuckles from other children, for example. Some habits are most evident as children are settling for sleep or during sleep (sucking movements and teeth grinding), when cortical inhibition is diminishing. Among school-aged children, habits may be seen during times of concentration and focused effort. Habits may be associated with negative mood states, especially anxiety. Anxious children are more likely to engage in a variety of habits, but “nervous habits” do not necessarily imply the presence of an anxiety disorder.

When a child engages in a habit to the extent that it interferes with daily functioning, the term habit disorder is appropriate. Some habits may evolve into patterns of behavior that are sufficiently recognizable or unusual to warrant a specific diagnostic term, especially if the habits are causing harm to the child. Onychophagia (nail biting) can cause paronychia, bruxism (severe teeth grinding) can wear down the surfaces of teeth and cause temporomandibular joint pain, trichotillomania (hair pulling) can cause highly visible and stigmatizing alopecia, and pica (persistent craving for and ingestion of nonfood items) can cause nutritional problems and poisoning.

Stereotypies

Stereotypies are repetitive, rhythmic movements that may resemble common habits (rocking, head banging) or appear bizarre and idiosyncratic (unusual hand movements). They include intrusive and potentially harmful behaviors, such as self-injurious behavior. They may involve specific objects and props, such as strings used for twirling and corners of furniture used for head banging.

Children with neurodevelopmental disorders and disabilities are much more likely to have stereotypies than are typically developing children. Indeed, engagement in repetitive behaviors is one of the cardinal diagnostic criteria for an autism spectrum disorder. Blind children may engage in repetitive movements that are sometimes referred to as blindisms. The term stereotypy implies the likelihood of underlying abnormality of brain function. When stereotypies interfere with activities of daily living or result in significant injury, the diagnosis of stereotypic movement disorder is appropriate. Serious self-injurious behavior occurs in fewer than 5% of people with mental retardation but is more common in those with severe mental retardation.

Theories of behavioral regulation postulate that an organism requires an optimal amount of stimulation and that repetitive behaviors may emerge when stimulation is suboptimal. These behaviors may serve to increase sensory stimulation, as evidenced by the frequent rocking observed in blind children or in deprived, institutionally reared children. Stereotypies typically increase during unstructured times and periods of boredom, and they subside when the children are provided with appropriate structure and stimulation. For many children with autism, engaging in their particular stereotypies provides sensory inputs that appear to be deeply rewarding.

Unlike habits, stereotypies are less subject to social reinforcement: Children with autism, for example, are unlikely to voluntarily control their stereotypies in public. This may reflect that children with neurodevelopmental disorders and cognitive disabilities may have diminished capacities for social learning and voluntary inhibition. However, the frequencies of stereotypies are certainly modifiable by environmental factors and behavioral reinforcement (see “Treatment” later in this chapter).

Tics

Tics are more easily recognized than precisely defined. They can be described as brief, rapid, coordinated, isolated fragments of normal motor or vocal behaviors. They can be easily mimicked. They do not occur continuously and rhythmically (like thumb sucking) but as discrete movements interspersed with tic-free periods. They rarely occur in sleep. Motor tics are typically brief clonic movements of eyes, face, neck, and shoulders, with eye blinking, facial grimacing, and head jerking the most common. Vocal or phonic tics include repetitive throat clearing, grunting, or barking.

Children as young as 6 years with tics describe premonitory sensory urges, a kind of pressure or “itch.” These distracting urges may interfere with concentration and contribute to attentional problems. Tics are not entirely involuntary and may be experienced as intentional surrender to almost irresistible sensory urges, usually accompanied by a fleeting and incomplete sense of relief.

Tics may be described in terms of location, number, frequency, and duration. They may also be characterized by their intensity, forcefulness, and complexity. Whereas tics are most commonly simple (brief and meaningless), they may be complex (longer and more elaborate). A few specific terms describe particular kinds of tic, such as coprolalia (uttering obscenities) and copropraxia (making obscene gestures). Fortunately, these are relatively rare in children.

Tics may increase at times of psychosocial stress, anxiety, emotional excitement, boredom, and fatigue. Activities that require focused attention and skilled motor performance are often associated with a decrease in tic frequency. There are accounts of surgeons and pilots with Tourette syndrome whose tics did not interfere with their capacity to perform highly skilled work.1

Compulsions

Compulsions are complex, ritualistic, and purposeful behaviors. They are most often seen as part of obsessive-compulsive disorder (OCD), a behaviorally defined psychiatric disorder characterized by obsessional anxiety and compulsive behaviors. Compulsions may occur in individuals who do not fully meet criteria for OCD, and they are commonly present in children with tic disorders and anxiety disorders. Compulsive behaviors in children may begin with checking under the bed at night to alleviate normal childhood fears, or not drinking from the water fountain to avoid getting sick, but then develop into more complex rituals. Common compulsions include repetitive checking, elaborate bedtime rituals, counting, matching, and other perfectionistic preoccupations. Several clinical series have documented that individuals with tic-related OCD are especially likely to report obsessions of symmetry and exactness and a compulsion to repeat activities to achieve a sense of things feeling “just right.”

The child who engages in compulsive behaviors understands that the behavior is excessive, inappropriate, or “doesn’t really make sense,” but still he or she feels compelled to do so, sometimes “to stop bad things from happening.” Engaging in the behaviors is in this sense involuntary, associated with embarrassment and shame, but also with some relief from the anxious sense that “something is wrong.”

Inevitably, there are gray areas in which habits, stereotypies, tics, and compulsions may be hard to distinguish. Compulsions may be difficult or impossible to distinguish from some complex and ritualistic tics. Some stereotypies in autistic children may have an anxious or compulsive quality.

Tourette Syndrome

Tourette syndrome is a developmental neuropsychiatric disorder characterized by multiple motor and vocal tics (not necessarily concurrent), lasting a period of at least 1 year, in which the individual is never tic-free for more than 3 consecutive months. Onset is before age 18 years, in some cases as young as 2 or 3 years, with peak onset around 5 to 8 years. Although there is wide variability in symptoms and clinical course, there is a tendency for severity to peak around 9 to 11 years. Improvement or even resolution by mid to late adolescence is likely, with only 20% continuing to show moderate global impairment by age 20 years.2,3 For further discussion see Chapter 566.

Tic Disorder Spectrum

Transient tic disorders include single or multiple motor and/or vocal tics, lasting at least 4 weeks up to 12 months. Most transient tics are simple rather than complex, and they do not usually cause great distress. A child with complex and distressing motor and vocal tics lasting a few months may be at risk for developing Tourette syndrome. Chronic tic disorders are single or multiple motor or vocal tics that last more than a year. It is thought that these disorders share the same pathogenesis and occur on a spectrum.2

Trichotillomania

Trichotillomania is a mental health disorder and is considered among the spectrum of obsessive-compulsive disorders.5 It is characterized by pulling out one’s hair, resulting in noticeable hair loss and occasional trichobezoars (hair balls). The behavior is associated with tension immediately prior to or when attempting to resist and with gratification or relief immediately after pulling out the hair.

Stereotypic Movement Disorder/Self-Injurious Behavior

Stereotypies and self-injurious behavior (SIB) can cause severe distress to individuals and their caregivers, even resulting in institutional care or severe injury (eg, Lesch-Nyhan syndrome). Typically, individuals who engage in SIB display a specific pattern of behavior, including head banging, hand or wrist biting, skin picking, hitting themselves with their fists, or poking their eyes. Although most SIB does not cause significant injury, tissue destruction, infection, loss of vision, and life-threatening injury may occur.6

Pica

Sporadic pica is common in toddlers, but persistent pica suggests the presence of psychiatric disorder, nutritional deficiency, or a neurodevelopmental disorder (eg, autism and mental retardation). Toxicity from ingestion of lead-containing plaster or paint chips, erosive esophagitis from caustic materials underscore the importance of close supervision and environmental safety precautions.

Rumination

Rumination is habitual regurgitation of food, which is then reswallowed or chewed. This habit may begin when infants insert their hands into their mouths or as a result of gastroesophageal reflux (common in children with neurodevelopmental disorders). Regurgitation and vomiting may be reinforced by attention from caregivers or other stimulation, leading to rumination (repetitive regurgitation). Rumination can be challenging to caregivers and may be associated with problems of oral and dental health.7

Prevalence of Common Habits

Rocking, head rolling, and head banging occur in 5% to 15% of young children, with typical onset around 6 to 9 months of age.8 Rocking and head rolling usually cease by 2 to 3 years, head banging by age 4. Head banging is 3 times more frequent in males than females. Thumb sucking occurs in 13% to 31% of North American children under 4 years of age, with an equal distribution between boys and girls.9 Although more breast-fed infants than bottle-fed infants suck their thumbs (34% and 17% respectively), the mean duration of thumb sucking is less for the breast-fed infant (21 vs 51 months). Thumb sucking peaks between 18 and 21 months of age, and most children spontaneously drop the habit by 4 years. Persistent thumb sucking into adolescence occurs more frequently in girls and may indicate underlying insecurities or other psychological problems.

Approximately 50% of children have habitual nail biting at some time in childhood. Onset is as early as 3 to 4 years, with peak prevalence at 10 to 18 years. By age 18, 23% of people continue to bite their nails. Nail biting is more common in males than females after this age and may be familial.

The prevalence of bruxism is difficult to ascertain because it occurs mostly during sleep. Parents acknowledge this habit in 15% of children between the ages of 3 and 17 years. Bruxism is more common in boys, and familial patterns are common. Trichotillomania may present before age 5 and recur or persist for many years.

Epidemiology of Tic Disorder Spectrum

Simple tics are very common in childhood: 5% to 13% of all children experience a transient tic during childhood.10 In a longitudinal observational study, the incidence of motor tics was higher during the winter months (November through February) than in the spring.11 The childhood incidence of chronic tic disorder is around 1% to 2%, with a ratio of approximately 3:1 of boys to girls. In contrast, TS is much less common, around 5 to 10 per 10,000. Neither race nor socioeconomic status is strongly related to risk of tic disorders.

Epidemiologic studies support a joint etiology of Tourette syndrome (TS) and obsessive-compulsive disorder (OCD). More than 50% of children with TS have extensive obsessions and/or compulsions. Forty percent of children with TS and more than 20% of all children with tic disorders have OCD. Children with TS who have a strong family history of TS are more likely to have OCD than are children with sporadic TS.12 Many children with TS have clinical depression or anxiety. Sleep disturbance13 and learning disabilities may be present in more than 20% of patients with TS.14,15 There is a 4-fold increase in the frequency of migraines in patients with TS.15 Cooccurrence with autism has also been reported, with a 6.2% prevalence of TS in children with autism.16 Psychiatric comorbidity in children with OCD is common, including tic disorders (18%), attention deficit hyperactivity disorder (ADHD), depression, and other anxiety disorders.

Children with TS are frequently diagnosed with ADHD, although comorbidity rates are controversial, ranging from 8% to 70%.17 Higher estimates probably reflect clinic referral bias. Children with comorbid TS and ADHD often have aggression, explosive behavior, low frustration tolerance, academic difficulties, and peer rejection.18 Case-control family studies have indicated that families of probands with either TS or ADHD have higher rates of comorbid TS and ADHD.19 The evidence suggests overlapping neurobiology between TS and ADHD but distinct genetic etiologies.

Self-Injurious Behavior

The underlying cause of habit disorder, stereotypies, and self-injurious behavior (SIB) varies among individuals and may include both environmental and biological factors. A child may learn that he gets the attention he wants or avoids an activity that he dislikes by engaging in SIB. Another child may engage in SIB because it appears to increase sensory stimulation. A third child may begin head banging because of a previously undiagnosed painful medical condition (eg, gastroesophageal reflux, dental decay, or ear infection).

The pathophysiology of SIB is obscure. Hypotheses focus on abnormalities of neurotransmitters, including dopamine and endorphins. Robust clinical response to dopamine blockers strongly support the role of dopaminergic subcortical systems. Severe self-mutilation in Lesch-Nyhan syndrome (an X-linked enzyme deficiency associated with abnormal purine metabolism) may be related to dopamine dysregulation in the basal ganglia. Compulsive picking in Prader-Willi syndrome may be partially responsive to selective serotonin reuptake inhibitors.

Genetics of Tic Disorders, Tourette Syndrome, and Obsessive-Compulsive Disorder

The precise etiology of these conditions is not known. Twin studies show moderate heritability, but nongenetic factors influence phenotypic expression. Previous twin studies indicating high concordance in monozygotic but not dizygotic twins suggested a single autosomal dominant gene with incomplete penetrance. Linkage studies have not identified a single strong candidate region, but several areas of interest on many chromosomes have been identified, including 11q23, 4q, 8p, 3q, 9q and 13q.20 Recent provocative evidence regarding a candidate gene SLITRK1 mapping from 13q31 may provide clues about the biologic pathways that lead to Tourette syndrome.21 It is likely that genetic vulnerability, environmental stressors, and epigenetic influences cause increased dopamine hypersensitivity in the basal ganglia of the developing brain. Similar genetic and nongenetic factors appear to be operating in obsessive-compulsive disorder.

Epigenetic factors implicated in the pathogenesis of Tourette syndrome include psychosocial stress, gestational and perinatal insults, exposure to androgens, heat and fatigue, and postinfectious autoimmune mechanisms. The increased prevalence in males raises questions about the potential role of androgenic steroids during critical periods of fetal brain development.22Patients with Tourette syndrome have higher levels of psychosocial stress, and antecedent stress can increase future tics and obsessive-compulsive disorder symptom severity.23

Neurobiology of Tic Disorders and Tourette Syndrome

Advances in understanding the neurobiology of Tourette syndrome using in vivo neuroimaging and neurophysiology techniques implicate specific cortico-striato-thalamo-cortical circuits important for procedural learning, initiating, and inhibiting psychomotor activity and harm detection/avoidance.2 Although there are few neuropathologic studies of Tourette syndrome brains, a recent study showed marked alteration in the number and density of GABAergic cells in basal ganglia structures, suggesting a developmental defect in migration of some GABAergic neurons.24 In the largest study of basal ganglia volume, involving 154 participants with TS and 130 healthy controls, caudate volume was significantly decreased in both children and adults with TS. Children with Tourette syndrome have been found to have decreased corpus callosum, decreased globus pallidus, and increased left thalamic volumes compared with healthy controls. Also, recent findings of increased cerebral subcortical hyperintensities suggests that subcortical injury, perhaps due to autoimmune processes, may play a role in pathogenesis.26 Cortical disinhibition may be related to hypersensitivity of dopamine D2 striatal receptors. Limited electrophysiological research supports the hypothesis that tic and habit disorders involve states of heightened activation, which impede initiation and control of complex motor actions.27 Tourette syndrome patients have deficits in sensory gating across a number of sensory modalities, suggesting that the inadequately gated sensory inputs may be “released” as tics.28 They may have a shortened cortical silent period of decreased excitability following stimulation, which may be related to the reduction in GABAergic interneurons in the cortex.24

Neuropsychological evaluation of patients with Tourette syndrome indicates impairment in procedural learning relative to normal controls.29 Also, many patients with Tourette syndrome have difficulties with fine motor control, motor inhibition, and visuomotor integration.30

Pediatric Autoimmune Neuropsychiatric Disorders Associated with Group a Streptococcus

Pediatric autoimmune neuropsychiatric disorders associated with group A streptococcus (PANDAS) is a controversial diagnosis based upon circumstantial evidence that suggests that antineuronal antibodies associated with streptococcal infection are affecting basal ganglion function. A case-controlled study of 144 children diagnosed with obsessive-compulsive disorder, Tourette syndrome, or tic disorder, indicated that cases had a higher likelihood of streptococcal infection in the 3 months before onset date than did controls.31 Multiple infections with group A streptococcus within a 12-month period was associated with an increased risk of Tourette syndrome with odds ration of 13.6 (95% confidence interval 1.93–51.0). But a more recent clinical study that found that only a small proportion of “PANDAS” exacerbations are temporally related to streptococcal infection.32 PANDAS may be episodic, and recurrences are common. Anti-DNAase B and other antistreptococcal antibody titers may be very high, and elevated antineuronal antibodies have been reported.33 However, a recent study did not confirm a relationship between antineuronal antibodies and clinical exacerbations.34

Habits, stereotypies, tics, and compulsions are clinical diagnoses, and there are no specific tests. Video resources can be very helpful in developing the clinical skills needed to distinguish normal from abnormal or to clarify the nature of an observed repetitive behavior.35,36

The differential diagnosis includes allergic rhinitis, seizure disorder, movement disorders, and Sandifer syndrome (gastroesophageal reflux–associated lateral movements of head and neck). Children with allergies often have recurrent throat clearing and sniffing, but tics are more repetitive and less variable. Brief epileptic seizures are usually accompanied by mental status changes such as diminished responsiveness. Complex partial seizures of temporal lobe epilepsy are usually staring episodes lasting 1 to 2 minutes with little or no movement, followed by confusion or tiredness.

One common repetitive behavior that deserves special mention is masturbation and other related movements associated with genital stimulation in infants and children.37 Parents may be alarmed by their child’s repetitive pelvic movements, usually while lying prone, sitting on a firm surface, or straddling an object. Such movements may be intense and associated with mental status and autonomic changes, such as staring and sweating. A review of a home video is generally sufficient to clarify the nature of the repetitive behavior, and explanation and reassurance can be a relief to incredulous parents.

Evaluation of repetitive movements includes detailed medical and developmental history, child interview if possible, observation, and careful neurological examination. For sporadic behaviors that are not evident in the clinic, home video can be useful.

Clinical evaluation of common habit disorders should include an assessment of potential impact on the child’s health. Persistent thumb sucking beyond 4 years of age may cause orthodontic and speech problems. Severe bruxism can cause dental problems and temporomandibular joint pain. Pica can be associated with nutritional deficiencies and toxic ingestion. Referral to pediatric dentists, nutritionists, and behavioral specialists may be important in thorough evaluation.

Evaluation of stereotypies includes assessment of the context in which the behavior occurs and of the impact on the child (his or her emotional, social, and school experiences) and family. A useful framework is the functional behavioral assessment, including an analysis of antecedent circumstances, factors that increase or decrease the behavior, and caregiver responses to the behavior.38 The Functional Assessment Screening Tool (FAST) is a useful, brief tool to assist the clinician in this process.39 Such an assessment may suggest a strategy or behavioral plan to treat the symptoms. For example, a child may engage in head banging during unstructured times, and caregiver attention may be reinforcing the behavior. The head banging may subside after a behavioral plan is introduced, including more structured activities, positive attention from caregivers to reward desired behavior, and selective ignoring of the head banging by the caregivers.

Evaluation of self-injurious behavior should include an assessment of the risk of physical harm. Severe head banging may cause brain injury or eye injury, necessitating the use of protective headgear.

Evaluation of tics should also be comprehensive and holistic. It is important to ask the child and family about the time course of tics and compulsive behaviors, relationship to medication use, and possible exacerbating factors. The child’s subjective experience of the repetitive behaviors and their social or emotional consequences should be explored. Specific inquiry about recent or previous streptococcal exposures or infections should be made. The history should include screening questions regarding anxiety symptoms, obsessive-compulsive disorder, attention deficit hyperactivity disorder, learning problems and depression, and family history of tic disorders and associated conditions.

Physical and neurological examination is important to rule out other movement disorders. Children with Tourette syndrome usually have a normal examination other than tics. Many children effectively suppress tics during the clinic visit, and the absence of tics does not preclude a diagnosis of tic disorder. Neurodevelopmental examination can provide opportunities to observe attention, planning, organization, visuomotor function, and other processing problems. Also, many children begin to exhibit their tics when stressed in this way. Psychological testing is useful if a learning disability is suspected.

There are no specific diagnostic tests for tics, compulsions, and stereotypies. Electroencephalographs and imaging studies are not routinely indicated. If there are clinical indications of pediatric autoimmune neuropsychiatric disorders associated with group A streptococcus (PANDAS), including sudden onset of symptoms and known exposure to streptococcus, most clinicians obtain streptococcal culture, antistreptolysin O titers, and anti-DNAase B, however, the efficacy of therapy for this disorder is controversial.

Standardized rating scales and checklists may be very helpful regarding comorbid attention deficit hyperactivity disorder symptoms and general functional impairment at home and school (eg, Child Behavioral Checklist [CBCL], Conners scales, and Vanderbilt scales). Other self-report or clinician-rated measures of tics and obsessive-compulsive symptoms may be useful both for clinical and research purposes (eg, Yale Global Tic Severity Scale, Leyton Obsessional Inventory, Child Yale-Brown Obsessive Compulsive Score [CY-BOCS]). Referral to pediatric neurologists, developmental pediatricians, child psychiatrists, and/or child psychologists may be essential for thorough evaluation in complex cases.

Habit Disorders

Common habits generally require no more than explanation and reassurance that the behavior is benign and self-limited. Parental efforts to dissuade a child from thumb sucking may cause anxiety or resentment and reinforce the behavior. A better approach is for parents to ignore the habit, while selectively giving praise during times when the child is not sucking. Head banging is still harder for parents to ignore. It may be helpful for the parent to pad the area that the child uses for this habit or to set limits whereby such behavior is permitted in one place only.

Some repetitive behaviors may be reinforced by parental attention, and ignoring such behaviors may lead to escalation and intensification of the behavior. Parents should be advised that such an increase in problem behavior is likely to be short lived. Some children may respond positively to parental behavior modification efforts but then substitute a new habit for the old discarded one.

Aversive treatments have been used for some habits, such as thumb sucking and nail biting (eg, bitter nail polish). It is generally helpful for the parent to make the child a partner in these treatment efforts so that they are not experienced as punitive. Concerns about complications may make other more invasive interventions necessary, such as dental appliances (plate with palatal bar) for thumb sucking or bite guard for bruxism.

Hypnotherapy is a treatment modality that may be well suited to pediatric habit disorders. Hypnotherapy has also been used successfully to treat children and adolescents with trichotillomania.40 Additional behavioral techniques include relaxation training and habit reversal procedures, such as teaching a child to engage in a hand grasp whenever they feel the urge to bite nails.

Pica and Rumination

The same general principles of management apply to these habit disorders. Pica and rumination are often done during unstructured time and when the child is alone or unsupervised. A behavioral plan should be established that increases structured activities, significantly decreases unsupervised time, and provides redirection and rewards. Diaphragmatic breathing may be helpful to suppress rumination.

Stereotypies and Self-Injurious Behavior

Management of these conditions includes functional behavioral assessment and development of a behavioral intervention plan, sometimes requiring assistance from a psychologist with experience in developmental disabilities. In severe cases and those that do not respond to behavioral interventions alone, a trial of an atypical antipsychotic may be indicated, in consultation with a subspecialist experienced in pediatric psychopharmacology. Risperidone is approved for the treatment of aggression and severe behavior problems in children with autism, ages 5 through 16 years, and it has been very useful in decreasing stereotypies and SIB in children with autism and other developmental disabilities.41 Other medications, including naltrexone, carbamazepine, and gabapentin, have been used off-label with inconclusive results.

Tic Disorders and Obsessive-Compulsive Disorder

Tics that are at least moderately severe and interfering with daily life should be treated. Education and demystification for the affected child, his or her family, and school personnel help to promote support and tolerance, reinforcing the message that the tics are involuntary. Some children may benefit from individual psychotherapy to improve self-esteem, social coping, school adjustment, and family stress. As noted previously, tics tend to diminish during and after adolescence in approximately 85% of cases, and only one third of childhood cases of Tourette syndrome persist into adulthood. Childhood tic severity does not predict persistence, but severe tics in late adolescence may persist.

Habit reversal psychotherapy involves increasing the individual’s awareness of the tics and establishing a competing response. Effectiveness in Tourette syndrome is not well established. Cognitive behavior therapy involving repeated exposures and response prevention (using cognitive techniques to change patterns of response) is of benefit in obsessive-compulsive disorder, sometimes enhancing medication response or allowing responders to discontinue medications.42

Pharmacotherapy is the cornerstone of treatment of tic disorders, but tics should not be treated too aggressively if they are not causing major functional impairment. The presence, scope, and severity of comorbid diagnoses (attention deficit hyperactivity disorder, obsessive-compulsive disorder, depression) should be assessed in planning pharmacotherapy.

Clonidine and guanfacine may be useful first-line medications in tic disorders and TS. Clinical effects include decreased hyperactivity, impulsivity, hyperarousal, stress responses, and aggression in addition to decreasing tics. Effect sizes greater than 0.5 (suggestive of a strong effect) were demonstrated in double-blind, placebo-controlled studies.43,44 An adequate trial may be 2 months. Sedation is the major side effect, especially for clonidine.

In children who have attention deficit hyperactivity disorder (ADHD) and tics, the α-2 agonists are not likely to be effective in improving attentional problems. A trial of stimulant therapy may increase tics in one third, have no effect on tics in one third, and lead to improvement in tics in one third.45 The combination of methylphenidate and clonidine in children with Tourette syndrome and ADHD is generally more effective than either medication alone.

The selective serotonin reuptake inhibitors are of proven effectiveness in the treatment of OCD.42 In children with TS, OCD, and ADHD, who may be more impaired from their OCD and ADHD symptoms than from their tics, a combination of SSRI and stimulant may be helpful. In children with positive culture and/or serologic evidence of recent streptococcal infection and a clinical presentation consistent with PANDAS, a course of penicillin may be helpful.31

Neuroleptics such as haloperidol can be quite effective in treating severe tic disorders.2,46 Dopamine blockade decreases dopaminergic input from substantia nigra and ventral tegmentum to the basal ganglia. Long-term use can cause tardive dyskinesia and other extrapyramidal side effects. Pimozide may have fewer extrapyramidal side effects but can cause QT prolongation. These medications may also cause sedation, personality change, and weight gain.

Atypical antipsychotics block dopamine and serotonin receptors. Risperidone, a potent D2 antagonist, has proven effectiveness in short-term clinical trials in children and adults with TS.47 Doses ranging from 1.0 to 3.5 mg/day were effective, and extrapyramidal side effects were rare. The most common adverse effects were weight gain, lipid abnormalities, and sedation.

Habits generally resolve spontaneously during childhood, although nail biting and other oral habits are common in adults. Efforts to stop a habit or stereotypy through behavioral reinforcement may be successful, but the resolution of one habit may be followed by the emergence of another.

Children with tics and compulsions may work hard to suppress their symptoms. Social learning and behavioral reinforcement may modify expression of repetitive behaviors over time. Children may learn to mask their tics and compulsions in a way that makes them seem less unusual or intrusive. The frequency of tics tends to decrease during adolescence.

Severe stereotypic movement disorder and self-injurious behavior has the potential to cause neurological injury or cataract formation. Tourette syndrome and obsessive-compulsive disorder can be socially disabling and stigmatizing. Moreover, associated conditions, including attention deficit hyperactivity disorder, learning disabilities, and depression, may cause severe impairments. Medications used in the treatment of tic disorders, obsessive-compulsive disorder, and stereotypies may cause iatrogenic complications.