The prevalence of Tourette syndrome in 13-14-year-olds in mainstream schools.

This study's aim was to estimate the prevalence and describe the clinical characteristics of Tourette syndrome (TS) in 13-14-year-old schoolchildren attending mainstream secondary schools. A three-stage ascertainment procedure was used to identify those who had TS. First, all 1012 Year 9 pupils were screened for tics using validated self-report questionnaires, which were completed by parents, teachers, and pupils. Data were available from at least one informant for 918 (90.7%) subjects. Tics were identified in 189 (18.7%) pupils. Second, families were contacted and a semistructured interview was carried out to determine whether they had TS. Finally, to ensure that the diagnosis of TS was correct, all those assessed as having TS were systematically assessed by an expert clinician in the field of TS. Seven young people were identified as fulfilling the criteria for TS, giving a minimum prevalence rate amongst 13-14-year-olds of 0.76% (95% CI 0.31 to 1.57) and a more realistic estimate of 1.85% (95% CI 1.00 to 2.95). Behavioural problems, in particular hyperkinetic disorder, were frequently associated with the TS group. These findings lend further support to the contention that the prevalence of TS in the community has hitherto been underestimated, though the symptoms may be generally milder than cases of TS presenting to clinics. This study supports the need for vigilance for TS in school-age children in primary care and in educational settings, so that children with this potentially serious disorder can be identified and assessed and effective management packages can be formulated to address their needs, when necessary.

Psychiatric comorbidity with substance misuse in children and teenagers.

Substance misuse in children and young people is frequently associated with emotional and behavioural disorder not attributable directly to the effects of the substance. Such comorbid disorders include depression, suicidal behaviour, conduct disorder, attention deficit hyperactivity disorder, eating disorders, and psychosis. Some indication of causal links in specific samples has been found but there is not overall agreement. The heterogeneity of young people with comorbidity for psychiatric disorder and substance misuse suggest that some would better be considered as multiproblem children for whom the necessary conditions are vulnerability, lack of family protection and exposure to a source of drugs. Children who present with comorbidity are at very high risk. This may be reduced by early identification and treatment of the comorbid condition and vigilance for substance misuse in all cases. Development of specific services for young substance misusers, coordinating efforts from Health Social Services and Education is merited in view high risk for future physical and psychiatric disorder. Few such services currently exist in the UK.

The prevalence of Tourette syndrome in a mainstream school population.

The aim of this study was to ascertain accurately the prevalence of Tourette syndrome (TS) in a mainstream school population. All year 9 pupils (aged 13 to 14 years) in a mainstream secondary school were investigated using a two-stage procedure. Standardized questionnaires were completed by parents, teachers, and pupils. Class observations were also carried out to identify tics. Those pupils identified as having tics underwent a semistructured interview to determine whether they had TS according to DSM-III-R criteria. Data were available from at least one source (parent, teacher, or self-reports) on 166 of the 167 pupils in the year. Five subjects were identified as having TS according to DSM-III-R criteria, yielding a prevalence estimate of 299 per 10,000 pupils in this age group. The results of this study suggest that TS in the community as a whole is more common and milder than those prevalence estimates and descriptions of the disorder based on TS encountered in secondary or tertiary health-care service settings.

Gilles de la Tourette's syndrome in special education schools: a United Kingdom study.

In order to determine the prevalence of tic disorders in children with severe school problems requiring a residential facility and comparison groups of children in regular day schools, we performed direct clinical examinations for the presence of tics and Gilles de la Tourette's syndrome (GTS) in 20 children from a residential school for emotional and behavioral difficulties (EBD); 25 children from a residential school for learning disabilities; 17 "problem" children (PC) (identified by teachers as having academic or behaviour problems) and 19 normal children (NC) selected at random (using random numbers) from a regular school. Of the EBD students, 65% were judged to have definite tics as compared with 24% of students with learning difficulties (P < 0.05), 6% of PC (P < 0.003) and none of the NC (P < 0.0006) group. Most of the affected students met diagnostic criteria for GTS. Our findings suggest that GTS is commonly associated with the need for special education and that this association is particularly robust for children with severe school problems. In these children, the presence of tics may be an indicator of an underlying dysfunction of neurological development.

Tourette syndrome, epilepsy, and emotional disorder, a case of triple comorbidity.

We present a case report illustrating the complexity encountered in the diagnosis and treatment of patients with multiple diagnoses. The clinical presentation in Tourette syndrome, and the behavioural manifestations in some cases of complex partial seizures can both be misunderstood and misdiagnosed. The paper, within the framework of a case, emphasises the need for a multidisciplinary approach in the assessment and management of such cases.

Cognitive function and behavioural status in paediatric heart and heart-lung transplant recipients: the Harefield experience.

OBJECTIVE: To assess the psychological impact of cardiac and cardiopulmonary transplantation on children. DESIGN: Retrospective cross sectional study. SETTING: One British centre performing paediatric heart and heart-lung transplant operations, four cardiac units in London, three London schools, two London health centres, and the dental department of a London children's hospital. SUBJECTS: 65 children who had been given heart or heart-lung transplants and two reference groups of 52 children who had had other types of cardiac surgery and 45 healthy children. MAIN OUTCOME MEASURES: Development, cognition, and behaviour at home and at school as assessed by measures with proved validity and reliability. RESULTS: Developmental and cognitive measures indicated that children given transplants had significantly lower scores on several parameters, particularly in terms of development in children under 4 1/2 years of age. Performance on all tests, however, was within the normal range. There were no significant differences in behavioural ratings between the transplant and reference groups, though problem behaviour at home was more prevalent in the transplant group. CONCLUSIONS: Though cognitive development may be within the normal range, there are adverse psychological effects associated with cardiac and cardiopulmonary transplantation. These data indicate the need for a controlled prospective study in which children and their families are seen before and at regular intervals after transplantation. Interventions should be developed that are tailored to the particular needs of this very specialised group of paediatric patients and their families.

Children with alcohol misusing parents.

Children of alcohol abusing parents (COAs) now receive more attention in their own right but data from methodologically sound studies is still thin. Genetic vulnerability increases risk of comorbidity for other psychiatric disorders and cognitive deficits as well as substance abuse. Neuropsychological effects of maternal alcohol consumption in pregnancy are more common than previously thought and paternal alcohol abuse may contribute to fetal damage. Family functioning is severely affected and COAs are at risk for child abuse though the strength of the association needs clarifying. Family drinking patterns are associated with teenage alcohol abuse and early induction increases the risk of addiction. COAs have raised morbidity rates for emotional and behavioural disturbance with impact on the developing child and separate prognostic significance for future adult morbidity other than alcoholism. Ethical considerations arise about the welfare of children cared for by alcohol abusing parents.

Clinical features and molecular analysis of the alpha thalassemia/mental retardation syndromes. II. Cases without detectable abnormality of the alpha globin complex.

We have identified five unrelated patients, all of north European origin, who have hemoglobin H (Hb H) disease and profound mental handicap. Surprisingly, detailed molecular analysis of the alpha globin complex is normal in these subjects. Clinically, they present with a rather uniform constellation of abnormalities, notably severe mental handicap, microcephaly, relative hypertelorism, unusual facies and genital anomalies. Hematologically, their Hb H disease has subtly but distinctly milder properties than the recognized Mendelian forms of the disease. These common features suggest that these five "nondeletion" patients have a similar underlying mutation, quite distinct from the 16p13.3 deletion associated with alpha thalassemia and mild to moderate mental retardation described in the accompanying paper. We speculate that the locus of this underlying mutation is not closely linked to the alpha globin complex and may encode a trans-acting factor involved in the normal regulation of alpha globin expression.