Em estudos etiológicos sobre a deficiência mental (DM), as anomalias cromossômicas, tanto numéricas quanto estruturais, são fatores que apresentam frequência relativa significante. O objetivo deste trabalho foi estudar as frequências e os tipos de anomalias cromossômicas em afetados por DM nas APAEs (Associação de Pais e Amigos dos Deficientes) de Batatais, Altinópolis e Serrana, objetivando conhecer melhor a contribuição destas anomalias na DM nessa região, caracterizando os tipos e as freqüências das aberrações cromossômicas observadas e compará-las entre as APAEs. Pacientes com suspeita de anomalias cromossômicas foram selecionados para o estudo. O critério usado para a seleção da amostra foi a realização do cariótipo em todos os afetados por DM com anomalias estruturais maiores e/ou menores. A análise citogenética foi feita através de cultura de linfócitos do sangue periférico e a coloração utilizada foi banda G, sendo analisadas 20 metáfases por paciente. Dos 505 indivíduos avaliados nas três APAES, 265 realizaram estudo citogenético, sendo encontradas 61 alterações cromossômicas (12,1% do total e 23,0% dos selecionados para cariótipo). Na APAE de Batatais, dos 305 indivíduos avaliados, 174 realizaram cariótipo, sendo encontradas 33 (10,8% do total) anomalias cromossômicas. Em Altinópolis, dos 107 indivíduos avaliados, 54 realizaram cariótipo, sendo observados 16 cariótipos anômalos (14,9% do total). Na APAE de Serrana, dos 93 indivíduos avaliados, 37 realizaram cariótipo, sendo encontradas 12 (12,9% do total) anomalias cromossômicas. Esses resultados demonstram que anomalias cromossômicas contribuem significativamente para a etiologia da DM e que a citogenética clássica possui importantes implicações na prática médica para o diagnóstico dos indivíduos afetados, assim como, para o aconselhamento genético das famílias. Além disso, observa-se que a APAE de Batatais, por apresentar uma porcentagem menor de indivíduos afetados por DM grave, 60,7%, possui uma menor incidência de anomalias cromossômicas quando comparada as APAEs de Altinópolis e Serrana que apresentam uma frequência de 87,8% e 83,9% de indivíduos com DM grave, respectivamente, indicando que alterações cromossômicas são mais frequentes em indivíduos afetados por DM grave. / In etiological studies on mental retardation (MR), the chromosomal abnormalities, both numerical and structural, are factors that have significant relative frequencies. The objective was to study the frequencies and types of chromosomal abnormalities in patients affected by MR in APAEs (Associação de Pais e Amigos dos Deficientes) of Batatais, Altinópolis and Serrana. This aims to better understand the contribution of these abnormalities to MR in these regions, and thus characterizing the types and frequencies of chromosomal aberrations observed in order to compare them between APAEs. Patients suspected of chromosomal abnormalities were selected for the study. The criterion used for sample selection was the achievement of the karyotype of all patients affected by MR with major and/or minor structural abnormalities. Cytogenetic analysis was performed on cultures of peripheral blood lymphocytes, where the band G was used for staining. Twenty metaphases were analyzed per patient. Of the 505 individuals evaluated in three APAEs, a cytogenetic study was performed on 265 patients, and 61 chromosomal abnormalities were found (12.1% of the total and 23.0% of the selected karyotypes). In APAE of Batatais, karyotypes were performed on 174 of the 305 subjects studied, and we found 33 chromosomal abnormalities (10.8% of total). In Altinópolis, 54 karyotypes were performed out of the 107 subjects studied, and we observed 16 abnormal karyotypes (14.9% of total). In APAE Serrana, 37 karyotypes were performed out of the 93 subjects studied, and 12 chromosomal abnormalities (12.9% of total) were found. These results show that chromosomal abnormalities contribute significantly to the etiology of MR and that classical cytogenetics have important implications in medical practice for diagnosis of affected individuals as well as for genetic counseling of the families. Moreover, it is noted that in the APAE of Batatais, because of the smaller percentage of individuals affected by severe MR, 60.7% have a lower incidence of chromosomal abnormalities when compared to the APAEs of Altinópolis and Serrana which have frequencies of 87.8% and 83.9% of individuals with severe MR, respectively. This indicates that chromosomal abnormalities are more frequent in individuals affected by severe MR.

[pt] Esta tese tem por objetivo principal analisar os processos de produção da longa permanência de crianças e adolescentes com deficiência na rede assistencial de abrigamento. Partindo da descrição da própria experiência profissional, impulsionada pelas contribuições de Erving Goffman e Franco Basaglia, a autora introduz o conceito de hibridismo assistencial para analisar o problema da deficiência institucionalizada nas interfaces entre os campos da Psiquiatria e da Assistência Social. Tendo como campo de estudo a rede de abrigos específicos para a deficiência no estado do Rio de Janeiro, a autora utiliza metodologias quantitativas e qualitativas para analisar o quadro atual da assistência asilar para crianças e adolescentes com deficiência, definindo-se o seguinte corpus de análise: (1) elementos da historiografia da psiquiatria infantil brasileira, tendo por referência as contribuições de Michel Foucault. Nesta perspectiva, discute-se a figura histórica do Pavilhão-Escola Bourneville - dispositivo vinculado ao Hospício Nacional de Alienados e marco inaugural da psiquiatria infantil brasileira para a internação de crianças anormais; (2) dados do Datasus referentes às internações de crianças e adolescentes com deficiência, no período de 1998 a 2010, em território nacional, ressaltando-se a dimensão quantitativa do problema, e (3) entrevistas realizadas junto a agentes sociais do Sistema de Garantia dos Direitos da Criança e do Adolescente do estado do Rio de Janeiro, tendo por referência de análise o conceito de campo de Pierre Bourdieu, e de Complexo Tutelar, de Jacques Donzelot. O estudo conclui que (1) existe uma correlação entre os abrigos específicos e a história da institucionalização da deficiência mental, mantendo-se a figura híbrida do abrigo-hospital, (2) as atuais políticas de desinstitucionalização não vêm incluindo crianças e adolescentes com deficiência, sendo necessário rever o conceito de crônicos para crianças e adolescentes no regime de internação hospitalar e, (3) a presença da deficiência é um fator de maximização das práticas tutelares, o que é verificado a partir dos discursos dos agentes sociais. A autora articula o tema em uma agenda de interesse público e acadêmico, buscando contribuir para a superação do modelo assistencial centrado na longa permanência, na rede asilar, de crianças e adolescentes com deficiência. / [en] This thesis aims at examining the processes that produces long-term placement of children and adolescents with mental disabilities in the shelter care facility network. The author begins hers analysis with the description of her own professional experience, driven by the contributions of Erving Goffman and Franco Basaglia. She introduces the concept of hibridismo assistencial (the idea of social assistance as an entangled reality) to analyze the problem of institutional disability looking at the interconnection between the areas of psychiatry and social work. Taking as her field of study the network of special purpose shelters (intended exclusively for children and youth with disabilities) in the State of Rio de Janeiro, the author combines quantitative and qualitative methodologies to analyze the current situation of the shelter care system for children and adolescents with disabilities, with the following corpus of analysis: (1) elements of the Brazilian historiography of child psychiatry, with reference to an analysis of the concept of power based on Michel Foucault. Under this perspective, the author discusses the historical figure of the Bourneville School - an institution linked to the National Asylum for the Insane and the founding landmark of the Brazilian child psychiatric for the hospitalization of the so called abnormal children, (2) Datasus data on hospitalization of children and adolescents with mental disabilities, highlighting the numerical dimensions of the problem, and (3) interviews with social agents connected to the System of Guarantee of the Rights of Children and Adolescents in the State of Rio de Janeiro, using as reference analysis the concepts of field by Pierre Bourdieu, and guardianship complex by Jacques Donzelot. Based on her study, the author concludes that: (1) there is a correlation between the existing shelters and the history of the institutionalization of children with mental disabilities as well as the presence of the ‘hybrid’ institution – the ‘shelter-hospital’, (2) the current policies aiming at closing down residential institutions have not included children and adolescents with disabilities, therefore it is necessary to critically review the concept of crônicos (chronically impaired) as referred to the young population that is hospitalized, and (3) based on testimonies of the social agents interviewed, it is possible to conclude that the presence of a disability becomes a factor of maximizing paternalistic practices. The author concludes her analysis linking some of the main issues to an agenda of academic and public interest, in an attempt to contribute to overcome old models centered on the long-term placement of children and adolescents with disabilities in institutions.

Submitted by admin tede (tede@pucgoias.edu.br) on 2016-08-18T13:16:28Z No. of bitstreams: 1 Roberto Carlos Gomes da Silva.pdf: 3176847 bytes, checksum: b508a4a3eb30a62abd86ea9330f277e4 (MD5) / Made available in DSpace on 2016-08-18T13:16:28Z (GMT). No. of bitstreams: 1 Roberto Carlos Gomes da Silva.pdf: 3176847 bytes, checksum: b508a4a3eb30a62abd86ea9330f277e4 (MD5) Previous issue date: 2008-04-09 / Since DNA structure was described, several studies have been carried out in genetics that promoted a revolution in the practice of medicine. Human syndromes that were practically undiagnosed became easily diagnosed with molecular tools. However, most of the genetic diseases remain under diagnostic obscurity, increasing health concerns for affected people and public demand for preventive health care such as the case of Fragile-X Syndrome, the most common heritable form of mental retardation in humans. FXS is caused by an expansion of CGG repeat sequence in the promoter region of FMR1 gene, located in Xq27.3. Both men and women are affected by FXS and pre-mutation can expand to a full mutation in the next generation. Under full mutation status ( 200 repeats) the gene is silenced and FMRP protein is not produced causing mental retardation, speech delay, and behavior problems, the most frequent symptoms in FXS. Prevalence of FXS is estimated in 1:4000 and 1:8000 and of carriers in the general population as 1:813 and 1:259 for men and women, respectively. Because of FXS potential to affect subsequent generations it is crucial to properly diagnose the syndrome. Laboratory analysis of DNA from FXS, using PCR or Southern Blotting, allows reaching the diagnosis in 99% of cases carrying mutated genes. However, to date the Brazilian Public Health System does not recognize the molecular methods to reach complete diagnostic in FXS. Early diagnose would allow fore more appropriate and efficient therapy approaches, favoring satisfactory development of all affected people, minimizing their suffering and the burden on their families, increasing, on the other hand, their quality of life which should go beyond survival. / A partir da descrição da estrutura do DNA, várias pesquisas foram desenvolvidas na área da Genética, promovendo uma revolução na prática da medicina. Síndromes, antes difíceis ou até impossíveis de serem detectadas, com tecnologia e ferramentas moleculares, tornaram-se facilmente diagnosticadas. Entretanto, diversas doenças ainda persistem na obscuridade de diagnóstico e geram problemas de saúde pública como é o caso da Síndrome do X-Frágil (SXF), que é a causa mais comum de retardo mental masculino herdado, e que consiste na expansão do número de cópias de uma seqüência de bases CGG do DNA no gene FMR1, localizado no cromossomo Xq27.3. A SXF afeta tanto homens quanto mulheres e a pré-mutação poderá expandir-se à mutação completa nas próximas gerações. Os portadores da pré-mutação continuam produzindo a proteína FMRP e os portadores da mutação completa são afetados pela SXF, pois o gene FMR1 é silenciado, e a proteína não é produzida, causando retardo mental, problemas de linguagem e de comportamento. A prevalência da SXF é estimada em 1:4000 e 1:8000, e para portadores na população em geral é 1:813 e 1:259 para homens e mulheres respectivamente. A importância do reconhecimento clínico e diagnóstico da SXF vem do fato de que as gerações futuras poderão estar comprometidas. O estudo do DNA para X-frágil pela PCR e Southern blotting permite determinar com segurança superior a 99% quem é portador da pré-mutação do gene FMR1 e quem possui a mutação completa. Entretanto, o SUS não reconhece os métodos moleculares, apesar do diagnóstico permitir intervenções terapêuticas, com respostas bastante eficientes, favorecendo o desenvolvimento de modo integral das pessoas afetadas, minimizando seu sofrimento e de seus familiares, uma vez que a qualidade de vida deve ir além da sobrevivência.

5 mutations responsables de retard mental ont été identifiées dans le gène p21-activated kinase 3 (pak3). Nous avons récemment identifiés dans pak3 deux exons alternatifs très conservés appelés b et c. Ainsi, en plus du variants PAK3a (dépourvu des inserts b ou c), le gène pak3 code pour 3 nouveaux variants d’épissage PAK3b, PAK3c et PAK3cb qui sont constitutivement actifs et insensibles aux GTPases. De plus, contrairement à PAK1 et PAK3a, leur domaine d’auto-inhibition est incapable d’inhiber un domaine kinase. Ainsi, le but de ce projet était de comprendre le mécanisme de régulation de la kinase PAK3. Un modèle de régulation a récemment été proposé dans lequel PAK1 forme des homodimères pouvant être dissociés par les GTPases, permettant ainsi l’activation de la kinase. En se basant sur ces observations j’ai cherché à identifier les dimères PAK3 et j’ai montré que les kinases PAK3a, b, c et cb forment préférentiellement des hétérodimères avec PAK1. J’ai démontré l’existence de ces dimères dans le cerveau et j’ai mis en évidence que ces hétérodimères permettent à chaque monomère de réguler l’activité kinase de son partenaire in vitro. Ce travail permet de proposer un modèle de régulation symétrique pour PAK3a qui forme des hétérodimères avec PAK1 et un nouveau modèle de régulation asymétrique pour les variants d’épissage, également basé sur leur hétérodimérisation avec PAK1. Mes résultats montrant une corégulation des kinases PAK neuronales suggèrent d'une part que leur activation puisse être synchronisée et d'autre part que dans certaines situations physiopathologiques (Cancer et maladies neurologiques) leur dérèglement puissent interférer. / 5 mutations responsible for mental retardation have been identified in p21-activated kinase 3 (pak3) gene. We recently identified in pak3, two highly conserved alternative exons called b and c. In addition to the classical PAK3a variant (without any alternative exon), the pak3 gene encodes 3 new splice variants PAK3b, PAK3c and PAK3cb which are constitutively active and insensitive to GTPase activation. Moreover, unlike PAK1 or PAK3a, their autoinhibitory domain is unable to inhibit a kinase domain. The aim of this project was to understand how PAK3 regulation occurs. A model of regulation was recently proposed in which PAK1 forms homodimers that can be dissociated through GTPase binding, leading to kinase activation. Given these observations, I searched to identify PAK3 dimers and I showed that PAK3a, b, c and cb preferentially form heterodimers with PAK1. I demonstrated the existence of such dimers in the brain and that the different heterodimers allow each monomer to regulate the kinase activity of its partner. Through this study, I propose a symmetric regulation model for PAK3a which heterodimerizes with PAK1 and a new asymmetric regulation model for splice variants, also based on heterodimerization with PAK1. My results showing a co-regulation of neuronal PAK kinases suggest that their activation may be synchronized but also that, in some physiopathological situations (cancers and neurologic diseases), their misregulation may interfere.

Esta dissertação tem o objetivo de questionar o diagnóstico de debilidade mental, a partir das contribuições da psicanálise, segundo Freud e Lacan. Partindo de uma análise histórica da construção de um discurso social e científico do termo, discutimos se este diagnóstico, e os fenômenos a ele associados, indicam alguma particularidade que pode fornecer uma orientação para a direção do tratamento, ou para a clínica. Apontamos que nem Freud nem Lacan possuem uma teoria específica da debilidade mental. Por outro lado, Lacan sustentava que Freud errou em contraindicar a análise aos débeis, dizendo que essa análise é sim, possível. Além disso, Lacan utilizou o termo debilidade mental em diferentes sentidos e em diversos momentos de sua obra, para fazer ironia ou para destacar aspectos estruturais da relação do homem com o saber. Este trabalho foca-se nos comentários de Lacan, quando o autor se debruça de forma mais enfática ao diagnóstico da debilidade mental. Posteriormente, analisamos três eixos de análise de autores lacanianos que, a partir da mesma referência (da obra de Lacan), indicaram diferentes estatutos da debilidade mental. O primeiro eixo refere-se à diferenciação entre a psicose e à debilidade mental. O segundo eixo considera a debilidade mental enquanto um fenômeno, ou uma defesa, articulada a uma estratégia de imaginarização, que poderia estar presente nas três estruturas clínicas (neurose, psicose, perversão). O terceiro eixo relaciona a debilidade mental à inibição. A partir dessa discussão e da análise de casos clínicos, apontamos que o que se nomeia de debilidade mental pode ser associada a certos fenômenos, relacionados a uma inflação imaginária. De um lado, este caminho mostra-se interessante, pois possibilita nos distanciarmos do caráter de déficit articulado à concepção de debilidade mental, bem como considerarmos a inclusão de um sujeito neste campo. Por outro lado, quanto à clínica propriamente dita, concluiremos que ela terá seu valor mais acentuado ao não se reduzir a esta análise. Para isso, devemos considerar: o desafio, já indicado por Mannoni (1985), de que médicos e psicanalistas não estão isentos de preconceitos; a consideração de Bruno (1986) de que a clínica com os ditos débeis não revela nenhuma clínica específica; e a fala de Lacan, quando ele indica que sempre falamos mais do que queremos dizer e que o homem sabe mais do que ele crê saber / Drawing heavily on Freud and Lacans contributions psychoanalysis, this dissertation aims to explore the diagnosis of mental retardation. Therefore, beginning with an historical analysis of the construction of a social and scientific discourse of the very expression mental retardation1 we discuss whether such a diagnosis and its associated phenomena can point to some particularity that might furnish a guideline either to treatment or to some clinical direction. While we point out that neither Freud nor Lacan espoused a specific theory about mental retardation, Lacan for his part believed not only that psychoanalysis could be effective in treating the retarded but also that Freud had erred in contraindicated such treatment. However, Lacan in his oeuvre never settles on one fixed meaning for the term mental retardation but rather uses this turn of phrase in several manners and contexts, often in order to underscore some irony or to highlight certain structural aspects of mans relationship to knowledge. Therefore, this work focuses on the commentaries of Lacan, in which he carefully examines the diagnosis of mental retardation. Later, we examine three distinct analytical modes, whose authors, drawing upon the same source material (i.e., Lacans work), proposed three different definitions of this mental handicap. The first mode refers to the distinction between psychosis and mental retardation. The second mode reflects on mental retardation as a phenomenon, or defense, linked to a strategy of imaginarization this strategy in fact could be present in all three clinical structures (neurosis, psychosis, and perversion). The third mode relates mental retardation to inhibition. From this discussion and from the analysis of clinical cases, we indicate that so-called mental retardation can be a function of certain phenomena related to an imaginary inflation. On one hand, this treatment path seems interesting, because it allows us both to go beyond the concept of a deficit related to mental retardation and to consider the inclusion of a subject in this field. On the other hand, the real value of clinical treatment becomes apparent when such treatment is not reduced to a mere analysis of certain phenomena. In this sense, we must take into account: Mannonis challenge (1985), that doctors and psychoanalysts are not free from prejudice; Bruno´s consideration (1986), that the psychoanalytical treatment of the so-called mentally retarded in fact follows the guidelines of standard treatment; and Lacans speech, that we always say more than we want to say and know more than we believe we know

Os objetivos deste estudo foram estimar a prevalência da deficiência intelectual aos 7-8 anos de idade em uma coorte de nascimentos, através de investigação genética clínica e laboratorial e, também, investigar a etiologia da mesma e os fatores associados. Os participantes faziam parte de uma coorte acompanhada desde o nascimento e foram incluídos neste estudo por apresentar, em acompanhamentos anteriores, suspeita de atraso no desenvolvimento segundo o Teste de Rastreamento de Battelle, QI abaixo de 70 segundo a escala WPPSI e/ou problemas no comportamento observados durante entrevista. Das 4231 crianças da Coorte de 2004 de Pelotas, 214 foram selecionadas para a avaliação genética que constou de: anamnese, exame físico e dismorfológico e coleta de sangue e urina quando indicado. Criou-se um banco de dados incluindo variáves desta avaliação e dos acompanhamentos anteriores da Coorte, tais como: variáveis da gestação e do nascimento, sociodemográficas e relativas à saúde e estimulação da criança. Os dados foram processados no pacote estatístico Stata 13.0 e foi utilizada análise de variância (ANOVA). Foi considerada como tendo deficiência intelectual a criança que, além de apresentar um QI abaixo de 70, apresentava também problemas no comportamento adaptativo. Cento e setenta crianças das duzentas e quatorze selecionadas no início do estudo foram diagnosticadas com deficiência intelectual e classificadas em cinco grupos etiológicos. A maior parte das crianças (44,4%) foi classificada como tendo deficiência intelectual devida a causas não-biológicas, ou seja, ligada a fatores ambientais. O segundo maior grupo (16,6%) foi o grupo de crianças com deficiência intelectual genética que incluiu crianças com síndrome de Down, microdeleções e patologias autossômicas dominantes e patologias multifatoriais. A seguir, crianças com sequelas neonatais (13,3%) e deficiência intelectual associada a outras doenças (13,3%), como epilepsia e TDAH. O menor grupo foi o idiopático, constituído por crianças que, mesmo após investigação clínica e laboratorial, permaneceram sem diagnóstico definido. A prevalência de deficiência intelectual foi de 4,5% e a prevalência de deficiência intelectual genética de 0,66%. Apesar de algumas limitações como a identificação e seleção dos casos aos 4 anos para uma avaliação aos 7-8 anos, é importante considerar que, por ser um estudo de base populacional, com alta taxa de acompanhamento (92,0%), isto minimiza o viés de seleção. O fato dos dados serem colhidos no momento ou em um curto intervalo de tempo, considerando os diversos acompanhamentos, minimiza o viés de memória. Fora do mundo desenvolvido, são raros os estudos de coorte que avaliaram deficiência intelectual, seus fatores de risco e sua etiologia. Grande parte destes estudos, mesmo os conduzidos em países de renda alta, avaliaram a prevalência, mas não a etiologia. Os dados sugerem que boa parte destes casos poderia ser prevenida, principalmente considerando uma etiologia não-biológica, caso existissem, além do rastreamento de problemas no desenvolvimento, estratégias de intervenção educacional e de saúde. / The aims of this study were to estimate the prevalence and etiology of intellectual disability at 7-8 years of age in a birth cohort through clinical and laboratory investigation and associated factors. Participants were part of a cohort followed from birth and were included in this study due to suspected developmental delay according to the Battelle Screening Test, IQ below 70 according to WPPSI scale and / or behavior problems observed during the interview in previous follow-ups. Of the 4231 children in the 2004 Pelotas birth cohort, 214 were selected for genetic evaluation which included anamnesis, physical and dysmorphological examination and collection of blood and urine when indicated. A dataset including variables from this evaluation and the previous cohort of follow-ups such as variables of pregnancy and birth, social demographic and health-related and stimulation of the child. Data were analyzed using Stata version 13.0. Analysis of variance (ANOVA) was performed. To be considered as having intellectual disability the child that presenting an IQ below 70 and problems in adaptive behavior. One hundred and seventy children from two hundred fourteen selected at baseline were diagnosed with intellectual disability and they were classified into five etiologic groups. Most children (44.4 %) were classified as having intellectual disability due to no biological causes, i.e., linked to environmental factors. The second largest group (16.6%) was the group of children with genetic intellectual disability which included children with Down syndrome, microdeletions and autosomal dominant and multifactorial diseases. Children with neonatal sequelae accounted for 13.3% and intellectual disability associated with other diseases such as epilepsy and ADHD also accounted for 13.3%. The smallest group was idiopathic composed of children who even after clinical and laboratory investigation remained without a definite diagnosis. The prevalence of intellectual disability was 4.5 % and the prevalence of genetic intellectual disability 0.66 %. Despite some limitations such as the identification and selection of cases to four years for an assessment at 7-8 years it is important to consider that it is a population-based study with high follow-up rate (92.0 %) which minimizes selection and information bias. As data were collected in time or in a short period of time considering the several follow-ups minimize recall bias. Outside the developed world few cohort studies assessed intellectual disabilities, their risk factors and etiology. Most of these studies even those conducted in high-income countries assessed the prevalence but not the etiology. The data suggest that part of these cases could be prevented specially considering the non-biological etiology if there were screening of developmental delay and intervention strategies on health and educational bases.

The study reports on analyses of data collected from the Inventory for Client and Agency Planning (ICAP) for 5,859 children with Autism (n = 511), Traumatic Brain Injury (TBI, n = 522), or Mental Retardation (MR, n = 4826) whose legal guardians applied for support services through the Utah Department of Services for People with Disabilities (DSPD). Results indicate that the least to most frequent problem behaviors were (a) destructive to property, (b) hurtful to self, (c) hurtful to others, (d) socially offensive, (e) unusual habits, (f) withdrawal, (g) uncooperative, and (h) disruptive behaviors. The degree of severity varied from problem to problem, with uncooperative behaviors rated as most severe. Males displayed higher frequency and severity for all problem behaviors, except hurtful to self.

This is a report of a descriptive study in which a random sample of fifty out of four hundred returned questionnaires from parents with a mentally retarded child were analyzed. The foci of analysis were: (1) parental attitudes toward the child; (2) the changes experienced by the family as affected by birth order and sex of the mentally retarded child; (3) how the diagnosis was accomplished; and (4) how in their opinion services during this diagnostic period could be improved. The data revealed that: (1) protectiveness toward the child is the predominant parental response; (2) negative changes are probably twice as high with an only child who is retarded; (3) male retardates are somewhat more disruptive than are female; (4) diagnosis must be individualized; however, generalizations which can be made include: understandable language, patience, empathy, and above all honesty.

The purposes of this study were: (1) to compare nine groups of subjects composed of intellectually normal children, mentally retarded adolescents, and mentally retarded adults on a three dimensional concept formation task; (2) to determine the effects of discrimination training on the sortings of the nine groups on the experimental task. The 207 subjects of this study were divided into nine groups. Seven of t he groups, consisting of high average and low average grade 3, superior high average, and low average grade 6, and high and low adolescent retardates were chosen on the bases of school grade level (3, 6, and adolescent retarded) and IQ level (low average, high average, superior, low and high adolescent, and low and high adult retarded) with each group composed of 21 subjects, except the two adolescent groups which were composed of 30 subjects each. The remaining two groups, high and low adult retardates, were chosen on the bases of chronological age (between 20-35) and IQ level (high and low mildly retarded), with both groups composed of 30 subjects. One-third of the subjects in each group were given special discrimination training with the task objects. The experimental task required each subject to place 27 objects in three trays which could be moved back and forth. The trays were stacked one on another vertically but separated by one-sixteenth of one inch. Each tray was divided into nine boxes. The objects were of three kinds: sphere, cube, and tetrahedron; three sizes: 1, 1 1/2, and 1 3/4 inches; and three shades of blue: dark, medium, and light. Each subject was directed to place the objects in the three dimensional matrix as he desired. The discrimination subjects of each group performed the same task, but they received special orientation training with the trays and objects. One task object, a medium sized, medium blue cube was pre-placed in the center box of the middle tray for an anchor point for each subject to use for his sortings. The results of this study indicate the following: The nine diverse groups included in this study did not show statistically significant differences in their grouping of identical color shades, identical forms and identical sizes in the three dimensional matrix when each element The nine groups did not significantly (statistically) differ in their use of the left to right direction in their grouping of identical color, identical form, and identical size horizontally as well as vertically. This lack of significance also applied to the use of the front to back direction in sorting color, form and size differences both horizontally and vertically. Discrimination training did not significantly affect the performance of the nine groups on any of the dimensions measured in this study. Adult and adolescent retarded groups showed noticeable effects from discrimination training by increasing their responses to size likenesses in their horizontal sortings. In general, normal subjects increased their groupings of identical elements more than retarded subjects, hut the findings indicate that IQ and chronological age did not significantly (statistically) affect discrimination training in these nine groups. Neither chronological age nor IQ significantly (statistically) affected the subjects' concrete tendency to place the largest size objects into the top tray which was most accessible for sorting.

Work, leisure and retirement are fundamental aspects of life for individuals with an intellectual disability, just as with the general population. Many educational efforts have taught knowledge and skills to persons with an intellectual disability to improve their functioning in the work and leisure domains. More recently, retirement concerns have become particularly salient because so many individuals now live much longer. The present study looked at using a conceptual approach to improve education in these three domains. It employed the principles that instruction works much better when it proceeds from an individual=s existing concepts and that instruction should teach useful concepts that an individual can apply to improve his or her real world functioning. This conceptual approach has not been used much with the education of persons with an intellectual disability. In Study 1, sixty adults with an intellectual disability were interviewed to determine their existing concepts of work, leisure and retirement and their work and leisure histories. Most had solid concepts of work and leisure, but with some gaps, particularly in notions of volunteer work and occupational status. Most reported satisfactory work and leisure lives. Most had a relatively poor concept of retirement at best and had done little or no retirement planning. These data suggested some key targets for an educational program to improve their knowledge and functioning in these domains. In Study 2, these data were used to develop an instructional program that focussed on gaps in knowledge of volunteer work, banking, budgeting and participation in satisfying leisure activities and in retirement planning. This instructional program was delivered over eight weeks to a class consisting of nine adults with an intellectual disability, with some success. This general conceptual approach can be usefully applied to teaching in other important domains with persons with an intellectual disability. They can be taught key concepts which they can use to live their lives more purposely and independently.