Paola R. Silvestri

Parent and self-report health-related quality of life measures in young patients with tourette syndrome

Tourette syndrome is a neurodevelopmental disorder characterized by tics and comorbid behavioral problems. This study compared child- and parent-reported quality of life and everyday functioning. We assessed 75 children with Tourette syndrome, of which 42 (56%) had comorbid conditions (obsessive-compulsive disorder = 25; attention-deficit hyperactivity disorder = 6; both comorbidities = 4). All patients completed psychometric instruments, including the Gilles de la Tourette Syndrome–Quality of Life Scale for Children and Adolescents (child report) and the Child Tourette’s Syndrome Impairment Scale (parent report). Data were compared for patients with pure Tourette syndrome, Tourette syndrome + obsessive-compulsive disorder, Tourette syndrome + attention-deficit hyperactivity disorder, and Tourette syndrome + both comorbidities. There were no group differences in quality of life. However, there were differences for total, school, and home activities impairment scores. Children and parents may not share similar views about the impact of Tourette syndrome on functioning. The measurement of health-related quality of life in Tourette syndrome is more complex in children than adults.

The Gilles de la Tourette Syndrome-Quality of Life Scale for children and adolescents (C&A-GTS-QOL): development and validation of the Italian version.

Background: Gilles de la Tourette syndrome (GTS) is a chronic childhood-onset neuropsychiatric disorder with a significant impact on patients’ health-related quality of life (HR-QOL). Cavanna et al. (Neurology 2008; 71: 1410–1416) developed and validated the first disease-specific HR-QOL assessment tool for adults with GTS (Gilles de la Tourette Syndrome-Quality of Life Scale, GTS-QOL). This paper presents the translation, adaptation and validation of the GTS-QOL for young Italian patients with GTS. Methods: A three-stage process involving 75 patients with GTS recruited through three Departments of Child and Adolescent Neuropsychiatry in Italy led to the development of a 27-item instrument (Gilles de la Tourette Syndrome-Quality of Life Scale in children and adolescents, C&A-GTS-QOL) for the assessment of HR-QOL through a clinician-rated interview for 6–12 year-olds and a self-report questionnaire for 13–18 year-olds. Results: The C&A-GTS-QOL demonstrated satisfactory scaling assumptions and acceptability. Internal consistency reliability was high (Cronbach’s alpha > 0.7) and validity was supported by interscale correlations (range 0.4–0.7), principal-component factor analysis and correlations with other rating scales and clinical variables. Conclusions: The present version of the C&A-GTS-QOL is the first disease-specific HR-QOL tool for Italian young patients with GTS, satisfying criteria for acceptability, reliability and validity.

Comment on: Tics in the Pediatric Population: Pragmatic Management

We read the recent review by Ganos et al., who gave an accurate description of the practical management required to address the needs of children with Tourette syndrome (TS). The review highlights the importance of a holistic approach and of a multidisciplinary team to assess and manage the complexity of TS and its comorbid disorders. We would like to underline another clinical variable of patients with TS: learning disabilities may occur frequently in these children, and they may contribute to worsening the global functioning of these patients. Few studies have examined the relationship between TS, cognitive profiles, and learning disabilities in detail. High rates of learning disabilities have been reported in children with TS, mainly in those with attention-deficit/hyperactivity disorder, although no specific pattern was found. This contribution stems from observations and analyses of problems and difficulties detected in a sample of 60 children and adolescents ages 3 to 12 years with TS or chronic tic disorder who were enrolled on multicenter research trials. Those studies focused on analyzing the role of Group A b-hemolytic Streptococcus infections in association with possible genetic susceptibility and immunological factors in the pathogenesis of TS and obsessive-compulsive symptoms (the European Multicenter Tics in Children Studies). During follow-up, 18 of 60 families pointed out that their children had problems at school. The difficulties cited were caused by delays in the development of learning skills in 13 children and by short attention spans and/or hyperactivity in 5 children. In 2 children, teaching staff had requested counseling because they were unable to deal with students who had tic disorders and with the problems related to them. Characteristics of this subgroup are illustrated in Table 1. To detect specific problems in every child and to meet with the requests of families and schools, we also assessed academic and neuropsychological characteristics, strengths, and weaknesses. Upon observation, children exhibited mild to moderate tic symptoms. Their mean Yale Global Tic Severity Scale score was 12.5 (range, 0–29). None of these children were receiving medication for the treatment of tics. Fifteen of 18 evaluated children received a diagnosis of 1 or more additional disorders. Learning disabilities were identified in 9 children (see Table 1). Although almost every child was referred because of tics, many were advised to undergo a rehabilitating treatment for causes different from tics; in other children, appropriate educational interventions were recommended. Our observations confirm that neuropsychological functioning is a main component in understanding the neurobehavioral outline of TS. Children with learning disabilities and/or specific academic weaknesses can rely on several educational interventions and accommodations, and these also can be effective in children with tics. Consequently, assessments of neuropsychological functioning and academic achievement should always be taken into consideration in the evaluation of children with TS —even those with mild symptoms and without comorbidities— in order to implement educational interventions and prevent school failure and dropout.

Health-related quality of life in patients with Gilles de la Tourette syndrome at the transition between adolescence and adulthood

Gilles de la Tourette syndrome (GTS) is a neurodevelopmental condition characterised by tics and co-morbid behavioural problems, affecting predominantly male patients. Tic severity typically fluctuates over time, with a consistent pattern showing improvement after adolescence in a considerable proportion of patients. Both tics and behavioural co-morbidities have been shown to have the potential to affect patients’ health-related quality of life (HR-QoL) in children and adults with persisting symptoms. In this study, we present the results of the first investigation of HR-QoL in patients with Gilles de la Tourette syndrome at the transition between adolescence and adulthood using a disease-specific HR-QoL measure, the Gilles de la Tourette Syndrome-Quality of Life-Children and Adolescents scale. Our results showed that patients with GTS and more severe co-morbid anxiety symptoms reported lower HR-QoL across all domains, highlighting the impact of anxiety on patient’s well-being at a critical stage of development. Routine screening for anxiety symptoms is recommended in all patients with GTS seen at transition clinics from paediatric to adult care, to implement effective behavioural and pharmacological interventions as appropriate.

A Randomized Controlled Trial Comparing Behavioral, Educational, and Pharmacological Treatments in Youths With Chronic Tic Disorder or Tourette Syndrome

Context The existing literature on the treatment of pediatric chronic tic disorder (CTD) and Tourette syndrome (TS) indicates that both behavioral therapy (BT) and pharmacotherapy (PT) are effective for reducing symptoms. Objective To evaluate the efficacy of BT compared to psychoeducation (PE) or PT for reducing tics and co-occurring symptoms and for improving quality of life (QoL) in a sample of youths with CTD and TS. Design A 10 weeks, 2 sites (Catania, Rome) randomized controlled trial. Participants were randomized to receive one of the following treatments: BT, PE, or PT. Participants 110 outpatients aged between 8 and 17 years affected by CTD or TS. Results Patients in the BT and PT groups showed a significant reduction in the severity of tic symptoms, while the PE group did not show any improvement. PT was more effective for reducing obsessive compulsive symptoms than BT, while PE group did not show any improvement. Both BT and PT groups showed an improvement in most QoL domains, whereas no differences were found in the PE group. Conclusions BT is as effective as pharmacological therapy in the treatment of tic disorders in children and adolescents, thus offering an alternative to medications for CTD and TS.

Alexithymia and tic disorders: a study on a sample of children and their mothers

Tic disorders are neurodevelopmental disorders characterised by the presence of motor or phonic tics, or both. Patients with tic disorders commonly report premonitory urges of tics. Alexithymia is a psychological trait characterised by a difficulty in identifying and expressing one’s own feelings and by an externally oriented thinking. We aimed to explore alexithymia in children with tic disorders and in their mothers. Global alexithymia scores of both children with tic disorders and of their mothers did not differ from those of the participants from the control group. In the tic disorder group, however, both children and their mothers showed a cognitive style characterised by operational thinking and a lack of imaginative abilities. The mothers of children with tic disorder reported significantly higher parental stress. Alexithymia was not predictive of tic severity but was predictive of the severity of the premonitory urges. The implications of these findings are discussed.

Self-, parent-, and teacher-reported behavioral symptoms in youngsters with Tourette syndrome: A case-control study

Dear Editor, We read with great interest in the Eur J Paediatr Neurol the recent contribution by Termine et al., who investigated the usefulness of selfand proxy-report instruments in assisting the clinical assessment of young people with Tourette Syndrome (TS). The authors found that self-report instruments have limited usefulness in differentiating the TS group from the control group in regards to behavioural symptoms. They concluded that young patients with TS tend to underestimate the severity of their behavioural and emotional problems, possibly due to lack of insight, denial or intrinsic limitations of self-report instruments in this age group. On the premise that the selfand proxy-report scales are a valid supplement of the direct clinical examination, we would like to pay attention to some clinical variables of TS that could offer a further key of reading of these results. Recently, we examined a sample of 31 young patients with TS, comparable for age, gender and age at onset of tics to patients of Termineet al., andwe evaluated themwith a similar assessment protocol, using self-report (SAFA and CDI) and proxy-report (CBCL, CPRS and CTRS) instruments. The two samplesdiffered for someclinical characteristics; ourgrouphad a higher tic severity: indeed, even if themean total YGTSS score wassimilar (37.7 15.7vs37.1 22.5 inourvsTermine’ssample, respectively), the number of patients on pharmacotherapy for ticmanagementwashigher inour sample than in theTermine’s sample (14/31 vs 7/23, respectively). On the other hand, Termine’s TS patients had significantly higher CY-BOCS scores. Most of the total and subscale scores of self-report and proxy-report instruments strikingly overlapped in the two samples. On the contrary, our group obtained significantly higher mean scores in comparison with that of Termine et al. in the CBCL’s clinical scales for Internalization, AnxiouseDepressed and Total score. Moreover, in a large part of SAFA subscales our patients obtained higher mean scores, even if these differences were not significant: however, it must be noticed that these scores could differentiate our TS group from Termine’s control group. In attempt to explain these differences, we examined our sample according to those clinical meaningful variables that could be correlated to the severity of symptoms. Specifically,we stratified our patients based on median age at evaluation ( 12 yrs vs>12 yrs),medianduration of symptoms ( 6 yrs vs>6 yrs), and use of anti-tic pharmacological therapy (NO vs YES). When considering age at evaluation, we noticed that most of item scores of SAFA Anxiety and SAFA Depression were higher in older patients. In particular, the subitems of the SAFA Anxiety e Separation anxiety and Critical itemand of the SAFA Depression e Sense of inadequacy and Critical itemwere significantly different. On the contrary, the duration of TS symptoms was correlated to significantly higher scores in the total and compulsion subscales of the CY-BOCS. Finally, no significantly differences in selfand proxyinstrument scores were found between patients treated or not with anti-tic pharmacotherapy. In conclusion, the discrepancies between Termine’s data and ours seem to be related to the characteristic phenotypic variability and the plasticity of the clinical expression of the TS spectrum. However, in this regard, in such a limited number of participants it is not possible to recognize univocal characteristics through all ages and levels of impairment of the TS. Thus, in our opinion the underestimation of behavioural and emotional problems severity in TS could be attributed to some clinical characteristics e age of patients and severity of symptoms e more than to a lack of insight, denial or intrinsic limitation of self-report instruments.