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AIM: To investigate the interdependence between risk factors associated with long-term intellectual development in individuals with tuberous sclerosis complex (TSC). METHOD: The Tuberous Sclerosis 2000 Study is a prospective longitudinal study of individuals with TSC. In phase 1 of the study, baseline measures of intellectual ability, epilepsy, cortical tuber load, and mutation were obtained for 125 children (63 females, 62 males; median age=39mo). In phase 2, at an average of 8 years later, intellectual abilities were estimated for 88 participants with TSC and 35 unaffected siblings. Structural equation modelling was used to determine the risk pathways from genetic mutation through to IQ at phase 2. RESULTS: Intellectual disability was present in 57% of individuals with TSC. Individuals without intellectual disability had significantly lower mean IQ compared to unaffected siblings, supporting specific genetic factors associated with intellectual impairment. Individuals with TSC who had a slower gain in IQ from infancy to middle childhood were younger at seizure onset and had increased infant seizure severity. Structural equation modelling indicated indirect pathways from genetic mutation, to tuber count, to seizure severity in infancy, through to IQ in middle childhood and adolescence. INTERPRETATION: Early-onset and severe epilepsy in the first 2 years of life are associated with increased risk of long-term intellectual disability in individuals with TSC, emphasizing the importance of early and effective treatment or prevention of epilepsy. WHAT THIS PAPER ADDS: Intellectual disability was present in 57% of individuals with tuberous sclerosis complex (TSC). Those with TSC without intellectual disability had significantly lower mean IQ compared to unaffected siblings. Earlier onset and greater severity of seizures in the first 2 years were observed in individuals with a slower gain in intellectual ability. Risk pathways through seizures in the first 2 years predict long-term cognitive outcomes in individuals with TSC.

Protected areas are a key strategy in conserving biodiversity, and there is a pressing need to evaluate their social impacts. Though the social impacts of development interventions are widely assessed, the conservation literature is limited and methodological guidance is lacking. Using a systematic literature search, which found 95 relevant studies, we assessed the methods used to evaluate the social impacts of protected areas. Mixed methods were used by more than half of the studies. Almost all studies reported material aspects of wellbeing, particularly income; other aspects were included in around half of studies. The majority of studies provided a snapshot, with only one employing a Before-After-Control-Intervention design. Half of studies reported respondent perceptions of impacts, while impact was attributed from researcher inference in 1/3 of cases. Although the number of such studies is increasing rapidly, there has been little change in the approaches used over the last 15 years, or in the authorship of studies, which is predominantly academics. Recent improvements in understanding of best practice in social impact evaluation need to be translated into practice if a true picture of the effects of conservation on local people is to be obtained.

Background: Increasing numbers of people are being referred for assessment for autism spectrum disorder (ASD). The NICE (UK) and the American Academy of Pediatrics recommend\ud gathering a developmental history using a tool that operationalises ICD/DSM criteria. However the best-established diagnostic interview instruments are time-consuming, costly and rarely used outside national specialist centres. What is needed is a brief, cost-effective measure validated in community settings. We tested the Development and Well-Being Assessment (DAWBA) for diagnosing ASD in a sample of children/adolescents representative of those presenting in community mental health settings.\ud Methods: A general population sample of twins (TEDS) was screened and 276 adolescents were selected as at low (CAST score