Thesis advisor: Penny Hauser-Cram / Williams syndrome (WS) is a genetic disorder marked by a microdeletion of approximately 25 genes on chromosome 7. Down syndrome (DS) is a genetic disorder marked by a chromosomal abnormality in which an additional copy of chromosome 21 is present in some or all cells. A comparative analysis of language acquisition between populations of children with WS and populations of children with DS yielded largely different patterns in language development. Phonology was found to be largely intact in children with WS, while children with DS struggled to produce intelligible and articulate speech. Semantics proved an area of relative strength in comparison to other areas of language development in both populations. Syntax was found to be a relative strength in the WS population, while children with DS struggled with syntactic output. Both groups exhibited difficulties in syntactic processing. In contrast to common conceptions regarding pragmatic strengths in children with WS, results indicated that gesture, narrative and discourse were areas of relative weakness in this population. Gesture, narrative and discourse proved areas of relative strength for children with DS. / Thesis (BA) — Boston College, 2011. / Submitted to: Boston College. College of Arts and Sciences. / Discipline: College Honors Program.

A síndrome de Down (SD) é a anomalia genética mais comum em seres humanos e acomete todas as etnias e níveis socioeconômicos. Esta síndrome também se manifesta na cavidade bucal dos indivíduos, alterando tecidos moles e duros, a constituição salivar e a microbiologia oral. Em relação à cárie dentária, muitos estudos na literatura avaliaram a experiência desta doença nas pessoas com SD, mas os resultados são conflitantes. Enquanto a maioria dos trabalhos reportou uma menor experiência de cárie nas pessoas com a síndrome comparadas a indivíduos não sindrômicos, outros estudos não mostraram diferença. Assim, três estudos foram conduzidos para elucidar algumas controvérsias. O primeiro estudo foi uma revisão sistemática de literatura que teve como objetivo determinar se existem ou não diferenças na experiência de cárie entre pessoas com e sem SD. Foram incluídos 13 artigos para extração dos dados. Entretanto, devido à baixa qualidade metodológica de todos os estudos, concluiu-se que não existe evidência cientifica para suportar a hipótese que pessoas com SD apresentem menor experiência de cárie do que indivíduos não sindrômicos. O segundo estudo teve caráter observacional e transversal e foi conduzido com131 crianças entre 6 e 12 anos de idade (60 com SD e 71 sem SD). Foram verificados os índices de placa (IP), índice de sangramento gengival (ISG), experiência de cárie e quantificação salivar Streptococcus mutans (S. mutans). Questionários que incluíram hábitos de escovação, dieta e renda dos participantes foram realizados. A experiência de cárie foi similar entre os dois grupos. Os IP e ISG foram menores nas crianças com SD e isto foi associado a uma maior supervisão dos pais ou responsáveis durante a escovação. Significativamente mais crianças com SD tiveram altas contagens salivares de S. mutans. O estudo concluiu que crianças com e sem SD da mesma faixa-etária apresentam experiência de cárie similar. Entretanto, crianças com SD frequentemente apresentam maiores contagens salivares de S. mutans do que crianças sem a síndrome. Além disso, crianças com SD que têm escovação supervisionada por pais ou cuidadores apresentam menores índices de placa e sangramento gengival. O terceiro estudo foi observacional laboratorial conduzido com o objetivo de avaliar a diversidade genotípica de S. 5 mutans e a acidogenicidade de diferentes genótipos deste microrganismo em crianças com e sem SD. Dezessete crianças (9 com SD e 8 sem SD) com altas contagens salivares de S. mutans foram selecionadas. Elas foram divididas em dois grupos: livres de cárie e com alta experiência de cárie. Isolados de S. mutans foram obtidos de cada paciente e 99 cepas (50 de SD e 49 sem SD) foram confirmadas utilizando PCR. Por meio de AP-PCR, observaram-se os diferentes perfis genotípicos. A acidogenicidade de uma cepa representativa para cada genótipo obtido foi avaliada. As crianças com SD tiveram uma menor diversidade genotípica de S. mutans do que as crianças sem a síndrome, e os genótipos avaliados das crianças com SD foram significativamente menos acidogênicos do que nas crianças sem síndrome. O estudo concluiu que crianças com SD apresentam genótipos de S. mutans menos acidogênicos quando comparados aos de crianças sem a síndrome. Os resultados dos trabalhos realizados permitem concluir que: não existem evidências científicas que suportem a hipótese que pessoas com SD apresentem menor experiência de cárie do que indivíduos sem a síndrome; pessoas com SD apresentam experiência de cárie similar a pessoas sem a síndrome; existe uma maior frequência de crianças com SD que apresentam altas contagens salivares de S. mutans do que crianças sem a síndrome; crianças com SD que têm escovação supervisionada por pais ou cuidadores apresentam menores índices de placa e sangramento gengival; os genótipos de S. mutans encontrados na cavidade bucal de crianças com SD são menos acidogênicos do que os das crianças sem síndrome. / Down Syndrome (DS) is the most common genetic anomaly in human, affecting all ethnicities and socioeconomic levels. The syndrome has also manifestations in the oral cavity of the subjects, altering soft and hard tissues, salivary constitution and oral microbiology. Regarding dental caries, previous studies have found conflicting results when evaluating the caries experience in DS subjects. While the majority of researchers reported a lower caries experience in DS versus non-DS subjects, other studies did not find difference. In order to elucidate the controversies, three studies were conducted. The first study was a systematic review with the aim of determining if there was difference in the caries experience in DS and non-DS subjects. In total, 13 articles were included for data extraction. However, due to the low methodological quality of all the studies, it was concluded that there was no scientific evidence to support the hypothesis that DS subjects have a lower caries experience than non-DS subjects. The second study was observational and cross-sectional and was performed with 131 children aged between 6-12 (60 with DS and 71 non-DS). The plaque index (PI), gingival bleeding index (GBI), caries experience and salivary Streptococcus mutans (S. mutans) quantification were investigated. Questionnaires that included tooth brushing habits, diet and income of the subjects were performed. Caries experience was similar in both groups. PI and GBI were lower in DS subjects, what was associated with a higher parental/caregiver supervision. Significantly more children with DS had high salivary counts of S. mutans. The study concluded that children with and without DS of the same age range have similar caries experience. However, children with DS often have higher salivary counts of S. mutans than children without the syndrome. In addition, children with DS who have tooth brushing supervised by parents or caregivers have lower PI and GBI. The third study was laboratory observational aimed to evaluate the S. mutans genotypical diversity and the associated acidogenicity in DS and non-DS children. Seventeen children (9 DS and 8 non-DS) with high salivary counts of S. mutans were selected and divided in two groups: caries-free and high caries experience. S. mutans isolates were obtained from each subject and 99 strains (50 in DS and 7 49 in non-DS) were confirmed through PCR. Different genotypical profiles were observed through AP-PCR technique. The acidogenicity of a representative strain from each genotype was analysed. DS children presented a lower S. mutans genotypical diversity than non-DS children. Moreover, the DS genotypes were significantly associated with less acidogenic than non-DS. The study concluded that children with DS present less acidogenic S. mutans genotypes when compared to children without the syndrome. In summary, the results of this studies allow conclude that: DS subjects have a similar caries experience than non-DS subjects; a significant higher frequency of DS children have higher S. mutans salivary counts than children without the syndrome; children with DS who have tooth brushing supervised by parents or caregivers have lower PI and GBI; the genotypes of S. mutans found in the oral cavity of children with DS are less acidogneic than those of children without the syndrome.

No description available.

by Lam Lai Wah. / Thesis (M.Phil.)--Chinese University of Hong Kong, 1997. / Includes bibliographical references (leaves 121-128). / ACKNOWLEDGMENTS --- p.i / ABSTRACT --- p.ii / TABLE OF CONTENTS --- p.iv / LIST OF TABLES AND FIGURES --- p.vi / LIST OF APPENDICES --- p.vii / Chapter CHAPTER 1 --- INTRODUCTION --- p.1 / Chapter CHAPTER 2 --- LITERATURE REVIEW / Introduction --- p.5 / Parental reactions to the birth of a child with congenital disability --- p.6 / Introduction to Down syndrome --- p.7 / Parental stress in rearing a child with handicaps --- p.11 / Outcomes of parental stress --- p.13 / Variables in relation to parental stress --- p.15 / Lazarus and Folkman's theory of stress and coping --- p.17 / Coping resources --- p.18 / Studies on perceived stress --- p.20 / Studies on coping --- p.24 / Studies on coping resources --- p.29 / Maternal focus --- p.30 / Summary --- p.32 / Chapter CHAPTER 3 --- METHODOLOGY / Aim --- p.34 / Objectives --- p.34 / Design --- p.35 / Sample --- p.36 / Instrument --- p.37 / Ethical issues --- p.38 / Gaining access --- p.39 / Pilot study --- p.41 / Results of the pilot study --- p.42 / Data collection --- p.44 / Data analysis --- p.49 / Validity and reliability --- p.54 / Chapter CHAPTER 4 --- FINDINGS / Demographic characteristics of the mothers --- p.58 / Demographic and biographic characteristics of the children --- p.59 / Description of the major categories and sub-categories identified --- p.61 / Chapter CHAPTER 5 --- DISCUSSION / Introduction --- p.87 / The first stage --- p.87 / The second stage --- p.95 / The third stage --- p.104 / Summary --- p.108 / Chapter CHAPTER6 --- IMPLICATIONS FOR NURSING PRACTICE --- p.111 / LIMITATIONS OF THE STUDY --- p.115 / RECOMMENDATIONS FOR FURTHER STUDY --- p.117 / CONCLUSIONS --- p.118 / REFERENCES --- p.121

É previsto que o desenvolvimento global das crianças com síndrome de Down (SD) ocorra com atraso, repercutindo nas habilidades cognitivas, sociais, de autocuidados, motoras e linguísticas. As diferenças significativas no padrão usual de aquisição de linguagem de crianças com SD ainda não foram totalmente dimensionadas. Apesar do extenso corpo de conhecimento sobre a SD e desempenho comunicativo, principalmente no âmbito internacional, há consideráveis lacunas para a compreensão destes processos. A proposição deste estudo foi verificar e analisar o desempenho de vocabulário receptivo e expressivo de crianças com SD e comparar com o desempenho de crianças com desenvolvimento típico (DT) pareados pela idade mental (Stanford-Binet) e sexo. Cumpriram-se os aspectos éticos. Participaram do estudo 20 crianças com SD (grupo experimental - GE), de ambos os sexos, com idade cronológica variando entre 36 e 71 meses e 20 crianças com DT (grupo controle - GC), com idade cronológica variando entre 13 e 44 meses. Foi realizada entrevista com os responsáveis e aplicado o Inventário de Desenvolvimento de Habilidades Comunicativas MacArthur (MacArthur). Os procedimentos de avaliação utilizados foram a Observação do Comportamento Comunicativo (OCC), o Teste de Vocabulário por Imagens Peabody (TVIP), o Teste de Linguagem Infantil ABFW Vocabulário Parte B (ABFW) e o Teste de Screening de Desenvolvimento Denver II (Denver II). O tratamento estatístico foi realizado com utilização de testes paramétricos e não paramétricos. Os resultados apontaram diferença estatisticamente significante entre o desempenho do GE e GC para todos os testes aplicados, exceto para a área pessoal-social e motora grossa do instrumento Denver II e para vocabulário expressivo do MacArthur. Em relação às habilidades receptivas e expressivas, o desempenho em vocabulário receptivo foi melhor do que no vocabulário expressivo para ambos os grupos. Na correlação entre os instrumentos aplicados, não foi encontrada correlação estatisticamente significante para desempenho no MacArthur e TVIP para GC e GE e no Denver II e TVIP apenas para GC. Ao analisar o desempenho de crianças com SD verificou-se alteração quanto ao vocabulário receptivo e expressivo. Ao comparar o desempenho das crianças com SD com crianças com DT, pareadas quanto ao sexo e idade mental, verificou-se que as crianças com SD apresentaram desempenho inferior quanto ao vocabulário receptivo e expressivo, com distinções em seus padrões de respostas. / Is provided that the overall development of children with Down syndrome (DS) occurs with delay, reflecting the cognitive, social, self-care, motor and language skills. Significant differences in the usual pattern of language acquisition of children with DS have yet to be scaled. Despite the extensive body of knowledge about DS and communicative performance, especially at the international level, there are considerable gaps in understanding these processes. The purpose of this study was to check and analyze the performance of receptive and expressive vocabulary of children with DS and compare with the performance of children with typical development (TD) matched for dental age (Stanford-Binet) and sex. Were fulfilled the ethical aspects. Study participants were 20 children with DS (experimental group - EG), of both sexes, with age varying between 36 and 71 months and 20 children with TD (control group - CG), with age varying between 13 and 44 months. Was performed an interview with those responsible and apply to the Inventory of Development Communication Skills MacArthur (MacArthur). The evaluation procedures used were the Communicative Behavior Observation (CBO), the Peabody Picture Vocabulary Test (PPVT), the Child Language ABFW Test - Vocabulary Part B (ABFW) and Development Screening Test Denver II (Denver II). Statistical analysis was conducted using parametric and nonparametric tests. The results showed a statistically significant difference between the performance of GE and GC for all tests, except for the personal-social and gross motor Denver II instrument and expressive vocabulary of MacArthur. With regard to receptive and expressive skills, performance on receptive vocabulary was better than the expressive vocabulary for both groups. In the correlation between the instruments applied, no statistically significant correlation was found for performance on MacArthur and PPVT to CG and EG and the Denver II and PPVT only for GC. Analyzing the performance of children with DS was found to change as receptive and expressive vocabulary. Comparing the performance of DS children with TD children, matched by sex and mental age, it was found that children with DS showed lower performance on the receptive and expressive vocabulary, with distinctions in their response patterns.

É previsto que o desenvolvimento global das crianças com síndrome de Down (SD) ocorra com atraso, repercutindo nas habilidades cognitivas, sociais, de autocuidados, motoras e linguísticas. As diferenças significativas no padrão usual de aquisição de linguagem de crianças com SD ainda não foram totalmente dimensionadas. Apesar do extenso corpo de conhecimento sobre a SD e desempenho comunicativo, principalmente no âmbito internacional, há consideráveis lacunas para a compreensão destes processos. A proposição deste estudo foi verificar e analisar o desempenho de vocabulário receptivo e expressivo de crianças com SD e comparar com o desempenho de crianças com desenvolvimento típico (DT) pareados pela idade mental (Stanford-Binet) e sexo. Cumpriram-se os aspectos éticos. Participaram do estudo 20 crianças com SD (grupo experimental - GE), de ambos os sexos, com idade cronológica variando entre 36 e 71 meses e 20 crianças com DT (grupo controle - GC), com idade cronológica variando entre 13 e 44 meses. Foi realizada entrevista com os responsáveis e aplicado o Inventário de Desenvolvimento de Habilidades Comunicativas MacArthur (MacArthur). Os procedimentos de avaliação utilizados foram a Observação do Comportamento Comunicativo (OCC), o Teste de Vocabulário por Imagens Peabody (TVIP), o Teste de Linguagem Infantil ABFW Vocabulário Parte B (ABFW) e o Teste de Screening de Desenvolvimento Denver II (Denver II). O tratamento estatístico foi realizado com utilização de testes paramétricos e não paramétricos. Os resultados apontaram diferença estatisticamente significante entre o desempenho do GE e GC para todos os testes aplicados, exceto para a área pessoal-social e motora grossa do instrumento Denver II e para vocabulário expressivo do MacArthur. Em relação às habilidades receptivas e expressivas, o desempenho em vocabulário receptivo foi melhor do que no vocabulário expressivo para ambos os grupos. Na correlação entre os instrumentos aplicados, não foi encontrada correlação estatisticamente significante para desempenho no MacArthur e TVIP para GC e GE e no Denver II e TVIP apenas para GC. Ao analisar o desempenho de crianças com SD verificou-se alteração quanto ao vocabulário receptivo e expressivo. Ao comparar o desempenho das crianças com SD com crianças com DT, pareadas quanto ao sexo e idade mental, verificou-se que as crianças com SD apresentaram desempenho inferior quanto ao vocabulário receptivo e expressivo, com distinções em seus padrões de respostas. / Is provided that the overall development of children with Down syndrome (DS) occurs with delay, reflecting the cognitive, social, self-care, motor and language skills. Significant differences in the usual pattern of language acquisition of children with DS have yet to be scaled. Despite the extensive body of knowledge about DS and communicative performance, especially at the international level, there are considerable gaps in understanding these processes. The purpose of this study was to check and analyze the performance of receptive and expressive vocabulary of children with DS and compare with the performance of children with typical development (TD) matched for dental age (Stanford-Binet) and sex. Were fulfilled the ethical aspects. Study participants were 20 children with DS (experimental group - EG), of both sexes, with age varying between 36 and 71 months and 20 children with TD (control group - CG), with age varying between 13 and 44 months. Was performed an interview with those responsible and apply to the Inventory of Development Communication Skills MacArthur (MacArthur). The evaluation procedures used were the Communicative Behavior Observation (CBO), the Peabody Picture Vocabulary Test (PPVT), the Child Language ABFW Test - Vocabulary Part B (ABFW) and Development Screening Test Denver II (Denver II). Statistical analysis was conducted using parametric and nonparametric tests. The results showed a statistically significant difference between the performance of GE and GC for all tests, except for the personal-social and gross motor Denver II instrument and expressive vocabulary of MacArthur. With regard to receptive and expressive skills, performance on receptive vocabulary was better than the expressive vocabulary for both groups. In the correlation between the instruments applied, no statistically significant correlation was found for performance on MacArthur and PPVT to CG and EG and the Denver II and PPVT only for GC. Analyzing the performance of children with DS was found to change as receptive and expressive vocabulary. Comparing the performance of DS children with TD children, matched by sex and mental age, it was found that children with DS showed lower performance on the receptive and expressive vocabulary, with distinctions in their response patterns.

Motor deficits are a hallmark of Down syndrome (DS), yet little is known about their exact cause. Despite the rich understanding of the neurobiology of DS, there is still a lack of targetable mechanisms for early intervention aimed at alleviating motor changes in people with DS. Therefore, we utilized a mouse model of DS known as Ts65Dn to characterize for the first time the effects of trisomy 21 on spinal cord (SC) development. A central molecular player in SC patterning and cell-type specification, Oligodendrocyte transcription factor 2 (Olig2), is located on human chromosome 21 (Hsa21) and is triplicated in both people with DS and in Ts65Dn mice. To observe the effects of the supernumerary Olig2, we used immunohistochemistry to visualize the OLIG2-derived cellular populations (i.e., motor neurons (MNs) and oligodendrocytes (OLs)), as well as adjacent and interacting cell populations (i.e., ventral spinal interneurons (INs)). We limited our analyses to two embryonic ages—embryonic days (E) 12.5 and 14.5. Our results indicate that there is no overall change in the numbers of OLs at either E12.5 or E14.5. However, there tend to be more OL-fated cells within the pMN domain, where they originate, and migrating cells tend to be clustered closer to the pMN domain at E12.5. IN populations show some changes in Ts65Dn mice at E12.5, with both total and abventricular PAX6+ cell numbers and abventricular NKX2.2+ cell numbers increased in Ts65Dn embryos compared to euploid mice. However, at E14.5 the number of NKX2.2+ cells is unchanged. No difference in the NKX6.1+ population was seen at either time-point. In contrast, there are significant changes in the MN population at both E12.5 and E14.5. Specifically, at E12.5, the total ISL1+ MN population is significantly increased and shows altered regional distribution in the ventral horn of Ts65Dn SCs. Conversely, the Ts65Dn spinal MN population is normalized to euploid levels at E14.5. Overall, our results suggest that neurogenesis, gliogenesis, and cell-type specification of OLIG2-lineage cells are altered in the developing SC of Ts65Dn mice. Thus, this work identifies a novel target for future therapeutic interventions aimed at ameliorating motor changes in DS.

Previous studies have found that spatial processing in children and adults with Down syndrome is different in comparison to the normal population. Some previous studies have also found that there is a high prevalence of colour vision deficiencies in people with Down syndrome. The aim of the present study was to use an objective test, the transient visual evoked potential (VEP), to assess achromatic and chromatic visual processing in adults with Down syndrome. Achromatic VEPs were recorded in response to black-white stimuli presented in patternreversal mode. Chromatic VEPs were recorded in response to two types of colour pattern, presented in pattern onset-offset mode. The two colour types were intended to preferentially stimulate the two principal chromatic pathways of the visual system, the ???redgreen??? and ???blue-yellow??? colour-opponent pathways. These stimuli are here termed the ???LM??? and ???S-(L+M) stimuli, respectively, reflecting the cone types that input to the pathways they are intended to stimulate. Each subject also completed two subjective colour vision tests, the Colour Vision Test Made Easy (CVTME) and the City University Colour Vision Test (CUT). Morphology of the achromatic and chromatic VEPs was found to differ between the group with Down syndrome and an age-matched control group. The latency of the P100 component of the achromatic VEP was found to be significantly later in the group with Down syndrome compared to the control group (the N75 latency was earlier in the group with Down syndrome, but not significantly so). The group-averaged peak-to-peak amplitude of the achromatic VEP was significantly lower in the group with Down syndrome compared to the control group. The major positive component of the VEP in response to the L-M stimulus was of significantly longer latency compared to that of the control group. The major negative component and the peak-to-peak amplitude of this response were not significantly different between the groups. For the response to S-(L+M) stimuli, the latency of the major negativity was significantly earlier in the group with Down syndrome and the major positivity was later, but not significantly so. Amplitude of this response was significantly higher in adults with Down syndrome compared to the control group. Most subjects in both groups passed both the CVTME and CUT. Our findings indicate that chromatic VEPs are abnormal in Down syndrome, and this may reflect abnormal processing of chromatic stimuli in this population. Alternatively, these abnormalities may arise due to abnormal cortical morphology, which may occur with normal or abnormal processing of chromatic signals. These findings further indicate that abnormality of chromatic VEPs may be expected in Down syndrome, and is not necessarily indicative of pathology or other abnormal function that is unrelated to the syndrome.

Changes in health care and increasing provision of community services have resulted in an increased number of community dwelling older adults with intellectual disabilities (ID), leading to questions about future planning for service delivery. Although selected aspects of functioning have been explored in various research studies, less longitudinal information pertaining to broad aspects of health is available to planners. This longitudinal project was designed over 10 years ago with the primary purpose of exploring individual and systemic issues in the health needs of this challenging population, leading to improved service planning.<p>Cross-sectional and longitudinal health data were collected from 360 adults with intellectual disabilities (ID) recruited from social services agencies from across the province of Saskatchewan. Data collection included caregiver information, chart information and directly administered tests of selected aspects of cognitive functioning. Formal data-collection occurred every second year for a maximum of four test times, and was supplemented by follow-up phone calls.<p>Analysis of study results showed that young, rather than older people without DS had a greater severity of health needs related to their underlying conditions, and more problematic behavioral and mental health issues. The reason for this was likely the increased survival of multiply handicapped young people, and the increasing trend for these people to be maintained in the community rather than in large institutions. This population was more likely to receive psychotropic medications, both for underlying problems such as seizure disorders, but also for difficult behaviors such as aggression.<p>On the other hand, adults with DS had relatively fewer problems in their younger adult years, but had increasing problems as they aged. Most aspects of functioning were decreased in the older compared to the younger cohorts, which was consistent with the longitudinal, individual level data showing yearly declines in most measured skills. These declines were greater than those found in adults without DS. Although yearly declines were noted in most age cohorts, the largest declines were noted in the oldest age groups, 50 years and over, suggesting that, while aging related decline was present from an early age, declines severe enough to suggest a dementing process probably do not start until after middle age. Declines in visual memory appear to precede those in praxis.<p>Mortality was increased with age, lower baseline functioning, DS, male gender, and baseline depressive symptoms.<p>The use of aging programs did not change much during the course of the study, but interesting differences in service use between people with and without DS were noted. Adults with DS were more likely than those without DS to participate in generic aging services, which was thought to be due to people with DS presenting with more typical, Alzheimer type behaviors, rather than severe behaviors such as aggression.

Changes in health care and increasing provision of community services have resulted in an increased number of community dwelling older adults with intellectual disabilities (ID), leading to questions about future planning for service delivery. Although selected aspects of functioning have been explored in various research studies, less longitudinal information pertaining to broad aspects of health is available to planners. This longitudinal project was designed over 10 years ago with the primary purpose of exploring individual and systemic issues in the health needs of this challenging population, leading to improved service planning.<p>Cross-sectional and longitudinal health data were collected from 360 adults with intellectual disabilities (ID) recruited from social services agencies from across the province of Saskatchewan. Data collection included caregiver information, chart information and directly administered tests of selected aspects of cognitive functioning. Formal data-collection occurred every second year for a maximum of four test times, and was supplemented by follow-up phone calls.<p>Analysis of study results showed that young, rather than older people without DS had a greater severity of health needs related to their underlying conditions, and more problematic behavioral and mental health issues. The reason for this was likely the increased survival of multiply handicapped young people, and the increasing trend for these people to be maintained in the community rather than in large institutions. This population was more likely to receive psychotropic medications, both for underlying problems such as seizure disorders, but also for difficult behaviors such as aggression.<p>On the other hand, adults with DS had relatively fewer problems in their younger adult years, but had increasing problems as they aged. Most aspects of functioning were decreased in the older compared to the younger cohorts, which was consistent with the longitudinal, individual level data showing yearly declines in most measured skills. These declines were greater than those found in adults without DS. Although yearly declines were noted in most age cohorts, the largest declines were noted in the oldest age groups, 50 years and over, suggesting that, while aging related decline was present from an early age, declines severe enough to suggest a dementing process probably do not start until after middle age. Declines in visual memory appear to precede those in praxis.<p>Mortality was increased with age, lower baseline functioning, DS, male gender, and baseline depressive symptoms.<p>The use of aging programs did not change much during the course of the study, but interesting differences in service use between people with and without DS were noted. Adults with DS were more likely than those without DS to participate in generic aging services, which was thought to be due to people with DS presenting with more typical, Alzheimer type behaviors, rather than severe behaviors such as aggression.