Clinical effectiveness and patient perspectives of different treatment strategies for tics in children and adolescents with Tourette syndrome: a systematic review and qualitative analysis.

BACKGROUND: Tourette syndrome (TS) is a neurodevelopmental condition characterised by chronic motor and vocal tics affecting up to 1% of school-age children and young people and is associated with significant distress and psychosocial impairment. OBJECTIVE: To conduct a systematic review of the benefits and risks of pharmacological, behavioural and physical interventions for tics in children and young people with TS (part 1) and to explore the experience of treatment and services from the perspective of young people with TS and their parents (part 2). DATA SOURCES: For the systematic reviews (parts 1 and 2), mainstream bibliographic databases, The Cochrane Library, education, social care and grey literature databases were searched using subject headings and text words for tic* and Tourette* from database inception to January 2013. REVIEW/RESEARCH METHODS: For part 1, randomised controlled trials and controlled before-and-after studies of pharmacological, behavioural or physical interventions in children or young people (aged < 18 years) with TS or chronic tic disorder were included. Mixed studies and studies in adults were considered as supporting evidence. Risk of bias associated with each study was evaluated using the Cochrane tool. When there was sufficient data, random-effects meta-analysis was used to synthesize the evidence and the quality of evidence for each outcome was assessed using the Grading of Recommendations Assessment, Development and Evaluation approach. For part 2, qualitative studies and survey literature conducted in populations of children/young people with TS or their carers or in health professionals with experience of treating TS were included in the qualitative review. Results were synthesized narratively. In addition, a national parent/carer survey was conducted via the Tourettes Action website. Participants included parents of children and young people with TS aged under 18 years. Participants (young people with TS aged 10-17 years) for the in-depth interviews were recruited via a national survey and specialist Tourettes clinics in the UK. RESULTS: For part 1, 70 studies were included in the quantitative systematic review. The evidence suggested that for treating tics in children and young people with TS, antipsychotic drugs [standardised mean difference (SMD) -0.74, 95% confidence interval (CI) -1.08 to -0.41; n = 75] and noradrenergic agents [clonidine (Dixarit(®), Boehringer Ingelheim) and guanfacine: SMD -0.72, 95% CI -1.03 to -0.40; n = 164] are effective in the short term. There was little difference among antipsychotics in terms of benefits, but adverse effect profiles do differ. Habit reversal training (HRT)/comprehensive behavioural intervention for tics (CBIT) was also shown to be effective (SMD -0.64, 95% CI -0.99 to -0.29; n = 133). For part 2, 295 parents/carers of children and young people with TS contributed useable survey data. Forty young people with TS participated in in-depth interviews. Four studies were in the qualitative review. Key themes were difficulties in accessing specialist care and behavioural interventions, delay in diagnosis, importance of anxiety and emotional symptoms, lack of provision of information to schools and inadequate information regarding medication and adverse effects. LIMITATIONS: The number and quality of clinical trials is low and this downgrades the strength of the evidence and conclusions. CONCLUSIONS: Antipsychotics, noradrenergic agents and HRT/CBIT are effective in reducing tics in children and young people with TS. The balance of benefits and harms favours the most commonly used medications: risperidone (Risperdal(®), Janssen), clonidine and aripiprazole (Abilify(®), Otsuka). Larger and better-conducted trials addressing important clinical uncertainties are required. Further research is needed into widening access to behavioural interventions through use of technology including mobile applications ('apps') and video consultation. STUDY REGISTRATION: This study is registered as PROSPERO CRD42012002059. FUNDING: The National Institute for Health Research Health Technology Assessment programme.

Intense Imagery Movements (IIM): More to motor stereotypies than meets the eye.

AIM: A subgroup of children who present with motor stereotypies in the context of episodes of intense imagery have recently been described in the literature,(1) termed Intense Imagery Movements (IIM). All children report conscious engagement in acts of imagery or imagination, with stereotyped movements occurring simultaneously with limited conscious awareness. This article reports preliminary cognitive data to inform clinical management and guide future research. METHOD: Intellectual functioning was assessed for ten children with IIM (7 boys, 3 girls; mean age = 10;01, age range = 6;06 to 14;04). In-depth neuropsychological assessments were conducted for four of these cases (3 boys, 1 girls; mean age = 9;05), with standardised questionnaires completed to assess mood, behaviour, attention/concentration, sensory functioning, motor functioning and stereotyped movements. RESULTS: All children exhibited discrepant intellectual profiles, especially on perceptual reasoning tasks, with significant impairments in processing speed. In-depth neuropsychological assessments indicated impaired performance on tests of attention and inhibition, but strengths in memory or oral expression. Three of the four children had sensory processing impairments, two had features of developmental co-ordination disorder and one had poor general well-being. None of the children had emotional or behavioural problems. INTERPRETATION: Children with IIM exhibit uneven intellectual and cognitive profiles, with particular discrepancies in perceptual reasoning skills. The case studies suggest that weaker attention, inhibition and processing speed skills may contribute to engagement in IIM, with good memory and/or language skills potentially contributing to the complexity of imagery abilities. Implications for the identification and management of these children in clinical practice, and future research ideas, are discussed.

Intense imagery movements: a common and distinct paediatric subgroup of motor stereotypies.

AIM: The aim of this article is to describe a subgroup of children who presented with stereotyped movements in the context of episodes of intense imagery. This is of relevance to current discussions regarding the clinical usefulness of diagnosing motor stereotypies during development. METHOD: The sample consisted of 10 children (nine males, one female; mean age 8y 6mo [SD 2y 5mo], range 6-15y). Referrals were from acute paediatricians, neurologists, and tertiary epilepsy services. Children were assessed by multidisciplinary teams with expertise in paediatric movement disorders. RESULTS: Stereotypies presented as paroxysmal complex movements involving upper and lower limbs. Imagery themes typically included computer games (60%), cartoons/films (40%), and fantasy scenes (30%). Comorbid developmental difficulties were reported for 80% of children. Brain imaging and electrophysiological investigations had been conducted for 50% of the children before referral to the clinic. INTERPRETATION: The descriptive term 'intense imagery movements' (IIM) was applied if (after interview) the children reported engaging in acts of imagery while performing stereotyped movements. We believe these children may form a common and discrete stereotypy subgroup, with the concept of IIM being clinically useful to ensure the accurate diagnosis and clinical management of this paediatric movement disorder.