Focus | Clinical Tourette syndrome in children Valsamma Eapen, Tim Usherwood UP TO 20% OF CHILDREN exhibit rapid jerky peak severity at the age of approximately movements (motor tics) that are made 10–12 years, and typically improve by without conscious intention as part of a adolescence or thereafter.6 Background Gilles de la Tourette syndrome (GTS), developmental phase that often lasts a few 1 characterised by motor and vocal tics, weeks to months. Similarly, involuntary has a prevalence of approximately 1% sounds, vocalisations or noises (vocal or Clinical features in school-aged children. Commonly phonic tics) such as coughing and even In addition to simple motor and vocal/ encountered comorbidities of GTS brief screams or shouts may be observed in phonic tics, complex tics may be present include attention deficit hyperactivity some children for brief periods of time. Tics (Table 1). Some complex tics – such as disorder (ADHD) and obsessive- lasting for a few weeks to months are known spitting, licking, kissing, etc – may be compulsive behaviour/disorder (OCB/ OCD). Genetic factors play an important as ‘transient tic disorder’. When single misunderstood or misinterpreted and part in the aetiology of GTS, and family or multiple motor or vocal tics – but not a may result in the young person getting members may exhibit tics or related combination of both – have been present in trouble, especially if these tics include disorders such as ADHD, OCB or OCD. for more than one year, the term ‘chronic involuntary and inappropriate obscene tic disorder’ is used. When both (multiple) gesturing (copropraxia) or copying the Objective The aim of this article is to present a motor and (one or more) vocal tics have been movements of other people (echopraxia). summary of the current evidence to assist present for more than a year, with onset Similarly, complex vocal tics may include the assessment and management of GTS before the age of 18 years, the condition is repeating words or phrases or even in primary care. known as Gilles de la Tourette syndrome full sentences (echolalia) or repeating (GTS) or, in short, Tourette syndrome.2 the last word or syllable (palilalia) or, Discussion A comprehensive assessment should The aim of this article is to review in approximately 10–15% of cases, include exploration of not only tics current evidence regarding the involuntary and inappropriate swearing or but also associated features and assessment and management of GTS. blurting out of obscenities (coprolalia). comorbidities. The stigmatising and Tics are usually preceded by a impairing nature of tics can have a premonitory sensation or urge, such as significant impact on the quality of life Prevalence a feeling of tightness, stretch, tension of the young person and their parents/ carers, as well as on family functioning. Once considered rare, GTS is now or itching that is relieved by performing Management includes education and understood to be relatively common, the tic, thereby leading to an urge-tic- explanation, behavioural treatments occurring in approximately 1% of relief cycle.7,8 Patients with GTS may and (sometimes) medication. school-aged children.3 GTS is more also exhibit a number of associated common in boys, and the male-to-female behaviours such as rage, self-injurious ratio is estimated to be 4:1.4,5 Tics have behaviours or non-obscene socially a mean age of onset of 6–7 years, reach inappropriate behaviours that involve 120 Reprinted from AJGP Vol. 50, No. 3, March 2021 © The Royal Australian College of General Practitioners 2021 Tourette syndrome in children Focus | Clinical socially inappropriate comments or observable tics but then show increased members. Tics should be distinguished actions.9 Attention deficit hyperactivity frequency when they return home. This from involuntary movements due to other disorder (ADHD) is encountered in may lead to a misunderstanding that conditions (Table 2).5 Careful neurological approximately 60–75% of children with children can suppress the tics if they examination may provide reassurance for GTS, while obsessive-compulsive disorder try harder. If anxiety and stress worsen the patient, parent/carer and doctor. (OCD) is reported in 27%, obsessive- the tics, then they may be mistaken as Once a diagnosis is made, further compulsive behaviours (OCB) in 32%, ‘psychological’ in origin. Rarely, tics assessments should include those of and self-injurious behaviours in 25%.10 may be brought on, or made worse, by comorbid conditions, associated features Although less common, features of autism, a bacterial (eg group A streptococcus) and the impact of their symptoms (Figure 1). learning difficulties, anxiety, depression, infection such as in paediatric autoimmune phobia, irritability, impulsivity, rage, neuropsychiatric disorder associated with aggression, sleep difficulties, oppositional streptococcal infection. Social impact and quality of life or conduct problems, substance use Tics can affect a child’s life in a number or personality issues may co-exist.11 of ways; these include the impact on Some individuals’ overall academic Aetiology school and academic work, social life and social functioning as well as quality Genetics play a major factor in the and relationships as well as physical and of life (QoL) may be affected more by development of GTS, but the mechanisms mental wellbeing.12 The visible nature these associated problems than the tics are complex. While no single susceptibility of the tics may draw attention to the themselves.12 Regarding the age of onset, gene of large effect has been identified as individual or lead to embarrassment, it is noteworthy that symptoms of ADHD yet, twin and family studies suggest that teasing/bullying or social exclusion. may appear between three and six years of it is highly heritable, with a population- Impact on school work may be due to the age (ie before the tics manifest), while the based heritability estimate of 0.771.13 effect of tics on learning or due to related OCD symptoms usually manifest in late Environmental factors are also involved, conditions such as ADHD, OCD/OCB or an childhood or adolescence. such as pre- and perinatal factors associated learning disorder.15 The ability Tics can come and go, and they take a including difficulties with pregnancy, to focus in class and engage in school waxing and waning course. While tics are smoking, exposure to infection, immune activities may be reduced as a result of the involuntary, they are sometimes referred or inflammatory factors and psychosocial child spending time and energy on trying to as ‘unvoluntary’ because the person may stressors, as well as birth complications.14 to hide or suppress the tics while at school. be able to suppress the tics for a period of Corticostriatal circuitry is implicated in GTS, Other students or teachers may accuse the time, and some of the complex tics may with excess dopamine levels thought to be individual with tics of deliberately ‘pulling be camouflaged to look like purposeful the underlying neurochemical abnormality. faces’, spitting or disturbing the class movements. Thus children may voluntarily through grunts or vocalisations. suppress their tics for short periods of QoL has been shown to be lower in time that may vary from seconds to Assessment people living with GTS when compared minutes or hours, but this is at the expense Assessment should include a detailed with the general population.16 Among of mounting inner tension and is often history inquiring about not only tics patients with GTS, individuals without followed by a rebound or increase in tics. but also other associated conditions comorbidities have been found to For example, some children may manage including ADHD, OCD and OCB, in have better QoL than those with the school hours fairly well without many the patient as well as among family comorbidities.17 Parent-reported Table 1. Common tics of Tourette syndrome* Simple Complex (involuntary meaningless movements) (unvoluntary seemingly purposeful movements) Motor tic Eye blinking, eye rolling, squinting, facial grimaces, Pulling of clothes, touching people/objects, poking/jabbing, shoulder shrugging, arm extending, mouth opening, smelling fingers/objects, punching self, jumping/skipping, nose twitching, lip licking, head jerks, brushing or kicking, hopping, walking on toes, kissing self or others, feet tossing hair out of eyes shuffling, flapping arms, twisting around, twirling hair, self- injurious behaviour, biting, picking skin or scabs Vocal/phonic tic Throat clearing, grunting, snorting, yelling/screaming, Making small animal-like sounds, unusual changes of pitch sniffing, barking, laughing, coughing, spitting, and volume of voice, stuttering, repetition of sounds squeaking, humming, whistling, honking *This is not an all-inclusive list © The Royal Australian College of General Practitioners 2021 Reprinted from AJGP Vol. 50, No. 3, March 2021 121 Focus | Clinical Tourette syndrome in children comorbidity in children and adolescents GTS have ‘Tourette-plus’, where one or Association of Australia, and the health with GTS has been found to be associated more comorbid conditions are present professional that they have consulted with decreased QOL, increased emotional (Figure 3).9 Management in these situations may provide information to schools or symptomatology and impaired emotional, should take into account the symptoms that other environments in which the child social (including peer relationship) are causing most distress or dysfunction.