Anthropometric and craniofacial patterns in mentally retarded males with emphasis on the fragile X syndrome.

PubMed

Butler, M G; Pratesi, R; Watson, M S; Breg, W R; Singh, D N

1993-09-01

Anthropometric and craniofacial profile patterns indicating the percent difference from the overall mean were developed on 34 physical parameters with 31 white, mentally retarded males (23 adults and 8 children) with the fra(X) syndrome matched for age with 31 white, mentally retarded males without a known cause of their retardation. The fra(X) syndrome males consistently showed larger dimensions for all anthropometric variables, with significant differences for height, sitting height, arm span, hand length, middle finger length, hand breadth, foot length, foot breadth, and testicular volume. A craniofacial pattern did emerge between the two groups of mentally retarded males, but with overlap of several variables. Significant differences were noted for head circumference, head breadth, lower face height, bizygomatic diameter, inner canthal distance, ear length and ear width, with the fra(X) syndrome males having larger head dimensions (head circumference, head breadth, head length, face height and lower face height), but smaller measurements for minimal frontal diameter, bizygomatic diameter, bigonial diameter, and inner canthal distance. Several significant correlations were found with the variables for both mentally retarded males with and without the fra(X) syndrome. In a combined anthropometric and craniofacial profile of 19 variables comparing 26 white fra(X) syndrome males (13 with high expression (> 30%) and 13 with low expression (< 30%), but matched for age), a relatively flat profile was observed with no significant differences for any of the variables. Generally, fra(X) syndrome males with increased fragile X chromosome expression have larger amplifications of the CGG trinucleotide repeat of the FMR-1 gene. No physical differences were detectable in our study between fra(X) males with high expression and apparently larger amplifications of the CGG trinucleotide repeats compared with those patients with low expression. Our research illustrates the use of

Anthropometric and craniofacial patterns in mentally retarded males with emphasis on the fragile X syndrome

PubMed Central

Butler, Merlin G.; Pratesi, Riccardo; Watson, Michael S.; Breg, W. Roy; Singh, Dharmdeo N.

2017-01-01

Anthropometric and craniofacial profile patterns indicating the percent difference from the overall mean were developed on 34 physical parameters with 31 white, mentally retarded males (23 adults and 8 children) with the fra(X) syndrome matched for age with 31 white, mentally retarded males without a known cause of their retardation. The fra(X) syndrome males consistently showed larger dimensions for all anthropometric variables, with significant differences for height, sitting height, arm span, hand length, middle finger length, hand breadth, foot length, foot breadth, and testicular volume. A craniofacial pattern did emerge between the two groups of mentally retarded males, but with overlap of several variables. Significant differences were noted for head circumference, head breadth, lower face height, bizygomatic diameter, inner canthal distance, ear length and ear width, with the fra(X) syndrome males having larger head dimensions (head circumference, head breadth, head length, face height and lower face height), but smaller measurements for minimal frontal diameter, bizygomatic diameter, bigonial diameter, and inner canthal distance. Several significant correlations were found with the variables for both mentally retarded males with and without the fra(X) syndrome. In a combined anthropometric and craniofacial profile of 19 variables comparing 26 white fra(X) syndrome males (13 with high expression (>30%) and 13 with low expression (< 30%), but matched for age), a relatively flat profile was observed with no significant differences for any of the variables. Generally, fra(X) syndrome males with increased fragile X chromosome expression have larger amplifications of the CGG trinucleotide repeat of the FMR-1 gene. No physical differences were detectable in our study between fra(X) males with high expression and apparently larger amplifications of the CGG trinucleotide repeats compared with those patients with low expression. Our research illustrates the use of

STRP linkage studies in a new family with X-linked mental retardation: Tight linkage to DXS458

DOE Office of Scientific and Technical Information (OSTI.GOV)

Lazzarini, A.; Stenroos, E.S.; Lehner, T.

1994-09-01

Isolated or non-specific mental retardation is defined as {open_quote}non-progressive intellectual handicap segregating in an X-linked manner without any consistent somatic or diagnostic features{close_quote}. The Human Gene Mapping Nomenclature Committee sequentially designates each newly reported MRX family, MRX1, MRX2,... etc. when a lod score of +2 is demonstrated between the MR locus and one or more X chromosome markers. A family, designated MRX20, was studied with nine short tandem repeat polymorphism (STRP) markers. Two-point lod scores above 3 were obtained with DXYS1 (Z = 3.02, {theta} = 0), DX3 (Z = 3.22, {theta} = 0), and DXS458 (Z = 3.31, {theta}more » = 0). A multipoint lod score of 3.66 was obtained with a peak between DXS3 and DXS458, 1.1cM distal to DXS3. A one-unit support interval is 16 cM between PGK1 and DXS458. This family represents the ninth of fourteen reported MRX families linked to markers in the region of DXYS1, perhaps reflecting a cluster of genes involved in brain function. The identification of genetic markers linked to the disease-causing gene has allowed gene carrier risk assessment for females in the family. Future research will concentrate on comparing the diversity of haplotypes containing the disease genes in different families, on physical mapping of the region, and on isolation of the MRX 20 gene.« less

Alpha Thalassemia/Mental Retardation Syndrome X-linked Gene Product ATRX Is Required for Proper Replication Restart and Cellular Resistance to Replication Stress*

PubMed Central

Leung, Justin Wai-Chung; Ghosal, Gargi; Wang, Wenqi; Shen, Xi; Wang, Jiadong; Li, Lei; Chen, Junjie

2013-01-01

Alpha thalassemia/mental retardation syndrome X-linked (ATRX) is a member of the SWI/SNF protein family of DNA-dependent ATPases. It functions as a chromatin remodeler and is classified as an SNF2-like helicase. Here, we showed somatic knock-out of ATRX displayed perturbed S-phase progression as well as hypersensitivity to replication stress. ATRX is recruited to sites of DNA damage, required for efficient checkpoint activation and faithful replication restart. In addition, we identified ATRX as a binding partner of MRE11-RAD50-NBS1 (MRN) complex. Together, these results suggest a non-canonical function of ATRX in guarding genomic stability. PMID:23329831

Fragile X Mental Retardation Protein (FMRP) controls diacylglycerol kinase activity in neurons.

PubMed

Tabet, Ricardos; Moutin, Enora; Becker, Jérôme A J; Heintz, Dimitri; Fouillen, Laetitia; Flatter, Eric; Krężel, Wojciech; Alunni, Violaine; Koebel, Pascale; Dembélé, Doulaye; Tassone, Flora; Bardoni, Barbara; Mandel, Jean-Louis; Vitale, Nicolas; Muller, Dominique; Le Merrer, Julie; Moine, Hervé

2016-06-28

Fragile X syndrome (FXS) is caused by the absence of the Fragile X Mental Retardation Protein (FMRP) in neurons. In the mouse, the lack of FMRP is associated with an excessive translation of hundreds of neuronal proteins, notably including postsynaptic proteins. This local protein synthesis deregulation is proposed to underlie the observed defects of glutamatergic synapse maturation and function and to affect preferentially the hundreds of mRNA species that were reported to bind to FMRP. How FMRP impacts synaptic protein translation and which mRNAs are most important for the pathology remain unclear. Here we show by cross-linking immunoprecipitation in cortical neurons that FMRP is mostly associated with one unique mRNA: diacylglycerol kinase kappa (Dgkκ), a master regulator that controls the switch between diacylglycerol and phosphatidic acid signaling pathways. The absence of FMRP in neurons abolishes group 1 metabotropic glutamate receptor-dependent DGK activity combined with a loss of Dgkκ expression. The reduction of Dgkκ in neurons is sufficient to cause dendritic spine abnormalities, synaptic plasticity alterations, and behavior disorders similar to those observed in the FXS mouse model. Overexpression of Dgkκ in neurons is able to rescue the dendritic spine defects of the Fragile X Mental Retardation 1 gene KO neurons. Together, these data suggest that Dgkκ deregulation contributes to FXS pathology and support a model where FMRP, by controlling the translation of Dgkκ, indirectly controls synaptic proteins translation and membrane properties by impacting lipid signaling in dendritic spine.

Further linkage evidence for localization of mutational sites for nonsyndromic types of X-linked mental retardation at pericentromeric region

DOE Office of Scientific and Technical Information (OSTI.GOV)

Robledo, R.; Melis, P.; Siniscalco, M.

We used several microsatellite markers scattered along the X chromosome to search for linkage relationships in a large Sardinian pedigree segregating for nonspecific X-linked mental retardation (MRX). Markers DXS573 and AR, located at chromosomal subregions Xp11.4-p11.22 and Xq11.2-q12, respectively, were found to segregate in full concordance with the disease, leading to a LOD score of 4.21 at zero recombination value. Recombination with the disease was found with markers MAOB and DXS454 located at Xp11.4-p11.3 and Xq21.1-q22, respectively; accordingly, markers distal to Xp11.4 and Xq22 also segregated independently of the disease. These findings provide strong linkage evidence in favor of themore » localization of one MRX mutational site in the pericentromeric region of the human X chromosome, justifying the assignment of a new symbol (MRX26) to our pedigree. Finally, on the basis of the recombinational events observed in the Xq21-q22 region, we have been able to refine the assignment of marker DXS456 to Xq21.33-q22. 26 refs., 3 figs., 1 tab.« less

Drosophila Fragile X Mental Retardation Protein Developmentally Regulates Activity-Dependent Axon Pruning

PubMed Central

Tessier, Charles R.; Broadie, Kendal

2014-01-01

Summary Fragile X Syndrome (FraX) is a broad-spectrum neurological disorder with symptoms ranging from hyperexcitability to mental retardation and autism. Loss of the fragile X mental retardation 1 (fmr1) gene product, the mRNA-binding translational regulator FMRP, causes structural over-elaboration of dendritic and axonal processes as well as functional alterations in synaptic plasticity at maturity. It is unclear, however, whether FraX is primarily a disease of development, a disease of plasticity or both; a distinction vital for engineering intervention strategies. To address this critical issue, we have used the Drosophila FraX model to investigate the developmental roles of Drosophila FMRP (dFMRP). dFMRP expression and regulation of chickadee/profilin coincides with a transient window of late brain development. During this time, dFMRP is positively regulated by sensory input activity, and required to limit axon growth and for efficient activity-dependent pruning of axon branches in the Mushroom Body learning/memory center. These results demonstrate that dFMRP has a primary role in activity-dependent neural circuit refinement in late brain development. PMID:18321984

The Fragile X Mental Retardation Protein, FMRP, Recognizes G-Quartets

ERIC Educational Resources Information Center

Darnell, Jennifer C.; Warren, Stephen T.; Darnell, Robert B.

2004-01-01

Fragile X mental retardation is a disease caused by the loss of function of a single RNA-binding protein, FMRP. Identifying the RNA targets recognized by FMRP is likely to reveal much about its functions in controlling some aspects of memory and behavior. Recent evidence suggests that one of the predominant RNA motifs recognized by the FMRP…

Fragile X mental retardation protein participates in non-coding RNA pathways.

PubMed

Li, En-Hui; Zhao, Xin; Zhang, Ce; Liu, Wei

2018-02-20

Fragile X syndrome is one of the most common forms of inherited intellectual disability. It is caused by mutations of the Fragile X mental retardation 1(FMR1) gene, resulting in either the loss or abnormal expression of the Fragile X mental retardation protein (FMRP). Recent research showed that FMRP participates in non-coding RNA pathways and plays various important roles in physiology, thereby extending our knowledge of the pathogenesis of the Fragile X syndrome. Initial studies showed that the Drosophila FMRP participates in siRNA and miRNA pathways by interacting with Dicer, Ago1 and Ago2, involved in neural activity and the fate determination of the germline stem cells. Subsequent studies showed that the Drosophila FMRP participates in piRNA pathway by interacting with Aub, Ago1 and Piwi in the maintenance of normal chromatin structures and genomic stability. More recent studies showed that FMRP is associated with lncRNA pathway, suggesting a potential role for the involvement in the clinical manifestations. In this review, we summarize the novel findings and explore the relationship between FMRP and non-coding RNA pathways, particularly the piRNA pathway, thereby providing critical insights on the molecular pathogenesis of Fragile X syndrome, and potential translational applications in clinical management of the disease.

Gene of a new X-linked syndrome with multiple congenital anomalies and severe mental retardation maps in Xp22-pter

DOE Office of Scientific and Technical Information (OSTI.GOV)

Wittwer, B.; Kircheisen, R.; Leutelt, J.

1994-09-01

We report on a family with 3 males presenting with a not yet described new X-chromosomal syndrome of multiple congenital anomalies and severe mental retardation. Two sisters have (with 3 different partners) 3 severely handicapped sons. In each case, oligohydramnios and intrauterine growth retardation were observed. Delivery was in the 34th, 31st, and 38th gestational week, respectively. Two of the patients had microcephaly (head circumference of the third case at birth is unknown). On physical examination, high and broad forehead, frontal bossing, downslanting palpebral fissures, long philtrum, thin upper lip, high arched palate, and deeply set anteverted ears were seen.more » One of the boys has microphthalmos and sclerocornea, while his cousin shows atrophy of the optic nerve. All three patients show a severe statomotor and mental retardation, they are most likely deaf and blind, have pathologic EEG, and seizures. Important additional findings are hydronephrosis, renal duplication, vesicorenal reflux, and agenesis of corpus callosum. The karyotype is normal (46,XY). We performed a segregation analysis in the family using more than 20 DNA polymorphisms distributed over the X chromosome. Linkage without recombination was found to KAL, DXS278, and DXS16 in Xp22. Analysis of multiple informative meioses suggested a location of the disease locus distal to DXS207. Recombinants were identified with all other marker loci from Xp22-Xpter.« less

The fragile X mental retardation protein has nucleic acid chaperone properties

PubMed Central

Gabus, Caroline; Mazroui, Rachid; Tremblay, Sandra; Khandjian, Edouard W.; Darlix, Jean-Luc

2004-01-01

The fragile X syndrome is the most common cause of inherited mental retardation resulting from the absence of the fragile X mental retardation protein (FMRP). FMRP contains two K-homology (KH) domains and one RGG box that are landmarks characteristic of RNA-binding proteins. In agreement with this, FMRP associates with messenger ribonucleoparticles (mRNPs) within actively translating ribosomes, and is thought to regulate translation of target mRNAs, including its own transcript. To investigate whether FMRP might chaperone nucleic acid folding and hybridization, we analysed the annealing and strand exchange activities of DNA oligonucleotides and the enhancement of ribozyme-directed RNA substrate cleavage by FMRP and deleted variants relative to canonical nucleic acid chaperones, such as the cellular YB-1/p50 protein and the retroviral nucleocapsid protein HIV-1 NCp7. FMRP was found to possess all the properties of a potent nucleic acid chaperone, requiring the KH motifs and RGG box for optimal activity. These findings suggest that FMRP may regulate translation by acting on RNA–RNA interactions and thus on the structural status of mRNAs. PMID:15096575

The fragile X mental retardation protein has nucleic acid chaperone properties.

PubMed

Gabus, Caroline; Mazroui, Rachid; Tremblay, Sandra; Khandjian, Edouard W; Darlix, Jean-Luc

2004-01-01

The fragile X syndrome is the most common cause of inherited mental retardation resulting from the absence of the fragile X mental retardation protein (FMRP). FMRP contains two K-homology (KH) domains and one RGG box that are landmarks characteristic of RNA-binding proteins. In agreement with this, FMRP associates with messenger ribonucleoparticles (mRNPs) within actively translating ribosomes, and is thought to regulate translation of target mRNAs, including its own transcript. To investigate whether FMRP might chaperone nucleic acid folding and hybridization, we analysed the annealing and strand exchange activities of DNA oligonucleotides and the enhancement of ribozyme-directed RNA substrate cleavage by FMRP and deleted variants relative to canonical nucleic acid chaperones, such as the cellular YB-1/p50 protein and the retroviral nucleocapsid protein HIV-1 NCp7. FMRP was found to possess all the properties of a potent nucleic acid chaperone, requiring the KH motifs and RGG box for optimal activity. These findings suggest that FMRP may regulate translation by acting on RNA-RNA interactions and thus on the structural status of mRNAs.

Functional consequences of mutations in CDKL5, an X-linked gene involved in infantile spasms and mental retardation.

PubMed

Bertani, Ilaria; Rusconi, Laura; Bolognese, Fabrizio; Forlani, Greta; Conca, Barbara; De Monte, Lucia; Badaracco, Gianfranco; Landsberger, Nicoletta; Kilstrup-Nielsen, Charlotte

2006-10-20

Mutations in the X-linked cyclin-dependent kinase-like 5 (CDKL5) gene have been identified in patients with Rett syndrome, West syndrome, and X-linked infantile spasms sharing the common features of generally intractable early seizures and mental retardation. Disease-causing mutations are distributed in both the catalytic domain and in the large COOH terminus. In this report, we examine the functional consequences of some Rett mutations of CDKL5 together with some synthetically designed derivatives useful to underline the functional domains of the protein. The mutated CDKL5 derivatives have been subjected to in vitro kinase assays and analyzed for phosphorylation of the TEY (Thr-Glu-Tyr) motif within the activation loop, their subcellular localization, and the capacity of CDKL5 to interact with itself. Whereas wild-type CDKL5 autophosphorylates and mediates the phosphorylation of the methyl-CpG-binding protein 2 (MeCP2) in vitro, Rett-mutated proteins show both impaired and increased catalytic activity suggesting that a tight regulation of CDKL5 is required for correct brain functions. Furthermore, we show that CDKL5 can self-associate and mediate the phosphorylation of its own TEY (Thr-Glu-Tyr) motif. Eventually, we show that the COOH terminus regulates CDKL5 properties; in particular, it negatively influences the catalytic activity and is required for its proper sub-nuclear localization. We propose a model in which CDKL5 phosphorylation is required for its entrance into the nucleus whereas a portion of the COOH-terminal domain is responsible for a stable residency in this cellular compartment probably through protein-protein interactions.

X-linked mental retardation syndrome: Three brothers with the Brooks-Wisniewski-Brown syndrome

DOE Office of Scientific and Technical Information (OSTI.GOV)

Morava, E.; Storcz, J.; Kosztolanyi, G.

1996-07-12

We report on 3 brothers with growth and mental retardation, bifrontal narrowness, short palpebral fissures, deeply set eyes with entropion, wide bulbous nose, small mouth, myopia, and spastic diplegia. The patients were born to normal and non-consanguineous parents. The similarity of our cases with those recently reported by Brooks et al. supports their suggestion that these patients are representative of a distinct entity. 8 refs., 3 figs., 1 tab.

Localization of a gene responsible for nonspecific mental retardation (MRX9) to the pericentromeric region of the X chromosome

DOE Office of Scientific and Technical Information (OSTI.GOV)

Willems, P.; Vits, L.; Buntinx, I.

1993-11-01

Nonspecific X-linked mental retardation (MRX) includes several distinct entities with mental retardation but without additional distinguishing features. The MRX family reported here has been classified previously as MRX9. In this study, the authors performed linkage analysis of MRX9 with a panel of 43 polymorphic DNA markers dispersed over chromosome X. Two-point linkage analysis revealed lod scores of 3.52 and 3.82 at 0% recombination for OATL1 and MAOA, both located in Xp11.2-p11.4. Lod scores for linkage with PGK1P1, DXS106, and DXS132, all located in Xq11-q13, were 3.83, 3.82, and 3.52, respectively, all at 0% recombination. Multipoint linkage analysis showed two peaksmore » with MAOA and DXS132/DXS106, respectively. Analysis of recombinational events indicated a position of the MRX9 gene between DXS164 and DXS453. These findings are compatible with a location of the MRX9 gene in the pericentromeric region of the X chromosome at Xp21-q13. 26 refs., 3 figs., 2 tabs.« less

THE MENTALLY RETARDED.

ERIC Educational Resources Information Center

JORDAN, THOMAS E.

THIS BOOK PROVIDES A GUIDE TO THE BASIC CONCEPTS AND ISSUES IN THE FIELD OF MENTAL RETARDATION. THERE ARE MANY SOURCES OR CAUSES OF MENTAL RETARDATION AND THE FOLLOWING TYPES ARE EXPLAINED--(1) GENETIC OR CHEMICAL DISORDERS, (2) BIRTH TRAUMA, (3) SUBSEQUENT ACCIDENTS OR DISEASE, AND (4) ENVIRONMENTAL INFLUENCES. IT IS NOTED THAT MOST CASES INVOLVE…

Realidades Acerca de la Deficiencia Mental = Facts about Mental Retardation.

ERIC Educational Resources Information Center

Texas State Dept. of Mental Health and Mental Retardation, Austin.

This document consists of two booklets, one in Spanish and one in English, both covering the same text: the characteristics of mentally retarded individuals, the prevalence of mentally retarded persons in Texas, causes of mental retardation, prevention possibilities, and services available to mentally retarded persons in Texas. A distinction is…

Muscle Fatigue during Intermittent Exercise in Individuals with Mental Retardation

ERIC Educational Resources Information Center

Zafeiridis, Andreas; Giagazoglou, Paraskevi; Dipla, Konstantina; Salonikidis, Konstantinos; Karra, Chrisanthi; Kellis, Eleftherios

2010-01-01

This study examined fatigue profile during intermittent exercise in 10 men with mild to moderate mental retardation (MR) and 10 men without mental retardation (C). They performed 4 x 30 s maximal knee extensions and flexions with 1-min rest on an isokinetic dynamometer. Peak torque of flexors (PTFL) and extensors (PTEX), total work (TW), and…

A computational approach to candidate gene prioritization for X-linked mental retardation using annotation-based binary filtering and motif-based linear discriminatory analysis

PubMed Central

2011-01-01

Background Several computational candidate gene selection and prioritization methods have recently been developed. These in silico selection and prioritization techniques are usually based on two central approaches - the examination of similarities to known disease genes and/or the evaluation of functional annotation of genes. Each of these approaches has its own caveats. Here we employ a previously described method of candidate gene prioritization based mainly on gene annotation, in accompaniment with a technique based on the evaluation of pertinent sequence motifs or signatures, in an attempt to refine the gene prioritization approach. We apply this approach to X-linked mental retardation (XLMR), a group of heterogeneous disorders for which some of the underlying genetics is known. Results The gene annotation-based binary filtering method yielded a ranked list of putative XLMR candidate genes with good plausibility of being associated with the development of mental retardation. In parallel, a motif finding approach based on linear discriminatory analysis (LDA) was employed to identify short sequence patterns that may discriminate XLMR from non-XLMR genes. High rates (>80%) of correct classification was achieved, suggesting that the identification of these motifs effectively captures genomic signals associated with XLMR vs. non-XLMR genes. The computational tools developed for the motif-based LDA is integrated into the freely available genomic analysis portal Galaxy (http://main.g2.bx.psu.edu/). Nine genes (APLN, ZC4H2, MAGED4, MAGED4B, RAP2C, FAM156A, FAM156B, TBL1X, and UXT) were highlighted as highly-ranked XLMR methods. Conclusions The combination of gene annotation information and sequence motif-orientated computational candidate gene prediction methods highlight an added benefit in generating a list of plausible candidate genes, as has been demonstrated for XLMR. Reviewers: This article was reviewed by Dr Barbara Bardoni (nominated by Prof Juergen Brosius

ATRX ADD domain links an atypical histone methylation recognition mechanism to human mental-retardation syndrome

DOE Office of Scientific and Technical Information (OSTI.GOV)

Iwase, Shigeki; Xiang, Bin; Ghosh, Sharmistha

ATR-X (alpha-thalassemia/mental retardation, X-linked) syndrome is a human congenital disorder that causes severe intellectual disabilities. Mutations in the ATRX gene, which encodes an ATP-dependent chromatin-remodeler, are responsible for the syndrome. Approximately 50% of the missense mutations in affected persons are clustered in a cysteine-rich domain termed ADD (ATRX-DNMT3-DNMT3L, ADD{sub ATRX}), whose function has remained elusive. Here we identify ADD{sub ATRX} as a previously unknown histone H3-binding module, whose binding is promoted by lysine 9 trimethylation (H3K9me3) but inhibited by lysine 4 trimethylation (H3K4me3). The cocrystal structure of ADD{sub ATRX} bound to H3{sub 1-15}K9me3 peptide reveals an atypical composite H3K9me3-binding pocket,more » which is distinct from the conventional trimethyllysine-binding aromatic cage. Notably, H3K9me3-pocket mutants and ATR-X syndrome mutants are defective in both H3K9me3 binding and localization at pericentromeric heterochromatin; thus, we have discovered a unique histone-recognition mechanism underlying the ATR-X etiology.« less

ATRX ADD Domain Links an Atypical Histone Methylation Recognition Mechanism to Human Mental-Retardation Syndrome

DOE Office of Scientific and Technical Information (OSTI.GOV)

S Iwase; B Xiang; S Ghosh

ATR-X (alpha-thalassemia/mental retardation, X-linked) syndrome is a human congenital disorder that causes severe intellectual disabilities. Mutations in the ATRX gene, which encodes an ATP-dependent chromatin-remodeler, are responsible for the syndrome. Approximately 50% of the missense mutations in affected persons are clustered in a cysteine-rich domain termed ADD (ATRX-DNMT3-DNMT3L, ADD{sub ATRX}), whose function has remained elusive. Here we identify ADD{sub ATRX} as a previously unknown histone H3-binding module, whose binding is promoted by lysine 9 trimethylation (H3K9me3) but inhibited by lysine 4 trimethylation (H3K4me3). The cocrystal structure of ADD{sub ATRX} bound to H3{sub 1-15}K9me3 peptide reveals an atypical composite H3K9me3-binding pocket,more » which is distinct from the conventional trimethyllysine-binding aromatic cage. Notably, H3K9me3-pocket mutants and ATR-X syndrome mutants are defective in both H3K9me3 binding and localization at pericentromeric heterochromatin; thus, we have discovered a unique histone-recognition mechanism underlying the ATR-X etiology.« less

Mental Retardation. Fact Sheet = El Retraso Mental. Hojas Informativas Sobre Discapacidades.

ERIC Educational Resources Information Center

National Information Center for Children and Youth with Disabilities, Washington, DC.

This fact sheet on mental retardation is written in both English and Spanish. It begins with a vignette of a 15-year-old boy with mental retardation. Mental retardation is briefly explained as are some causes of mental retardation. It notes that a diagnosis of mental retardation looks at two things: first, the ability of a person's brain to learn,…

The Fragile X Mental Retardation 1 Gene (FMR1): Historical Perspective, Phenotypes, Mechanism, Pathology, and Epidemiology

PubMed Central

Grigsby, Jim

2016-01-01

Objectives To provide an historical perspective and overview of the phenotypes, mechanism, pathology, and epidemiology of the fragile X-associated tremor/ataxia syndrome (FXTAS) for neuropsychologists. Methods Selective review of the literature on FXTAS. Results FXTAS is an X-linked neurodegenerative disorder of late onset. One of several phenotypes associated with different mutations of the fragile X mental retardation 1 gene (FMR1), FXTAS involves progressive action tremor, gait ataxia, and impaired executive functioning, among other features. It affects carriers of the FMR1 premutation, which may expand when passed from a mother to her children, in which case it is likely to cause fragile X syndrome (FXS), the most common inherited developmental disability. Conclusion This review briefly summarizes current knowledge of the mechanisms, epidemiology, and mode of transmission of FXTAS and FXS, as well as the neuropsychological, neurologic, neuropsychiatric, neuropathologic, and neuroradiologic phenotypes of FXTAS. Because it was only recently identified, FXTAS is not well known to most practitioners, and it remains largely misdiagnosed, despite the fact that its prevalence may be relatively high. PMID:27356167

The fragile X mental retardation 1 gene (FMR1): historical perspective, phenotypes, mechanism, pathology, and epidemiology.

PubMed

Grigsby, Jim

2016-08-01

To provide an historical perspective and overview of the phenotypes, mechanism, pathology, and epidemiology of the fragile X-associated tremor/ataxia syndrome (FXTAS) for neuropsychologists. Selective review of the literature on FXTAS. FXTAS is an X-linked neurodegenerative disorder of late onset. One of several phenotypes associated with different mutations of the fragile X mental retardation 1 gene (FMR1), FXTAS involves progressive action tremor, gait ataxia, and impaired executive functioning, among other features. It affects carriers of the FMR1 premutation, which may expand when passed from a mother to her children, in which case it is likely to cause fragile X syndrome (FXS), the most common inherited developmental disability. This review briefly summarizes current knowledge of the mechanisms, epidemiology, and mode of transmission of FXTAS and FXS, as well as the neuropsychological, neurologic, neuropsychiatric, neuropathologic, and neuroradiologic phenotypes of FXTAS. Because it was only recently identified, FXTAS is not well known to most practitioners, and it remains largely misdiagnosed, despite the fact that its prevalence may be relatively high.

The Coffin-Lowry syndrome: an inherited faciodigital mental retardation syndrome.

PubMed

Temtamy, S A; Miller, J D; Hussels-Maumenee, I

1975-05-01

Eight patients in three families had mental retardation, characteristic facies and hands, and skeletal changes; the clinical features suggested to us that they had a syndrome previously thought to represent two entities described by Lowry and associates and by Coffin and associates, respectively. New findings include skeletal, orodental, and dermatoglyphic abnormalities and histopathologic changes suggesting that the syndrome is a heritable disorder of connective tissue. Severe expression in males and transmission through mildly affected females suggest X-linked or sex-influenced autosomal dominant inheritance.

A Trial of Metformin in Individuals With Fragile X Syndrome

ClinicalTrials.gov

2018-06-05

Fragile X Syndrome; Fragile X Mental Retardation Syndrome; Mental Retardation, X Linked; Genetic Diseases, X-Linked; Trinucleotide Repeat Expansion; Fra(X) Syndrome; Intellectual Disability; FXS; Neurobehavioral Manifestations; Sex Chromosome Disorders

Mental Retardation: Prevention Strategies That Work.

ERIC Educational Resources Information Center

President's Committee on Mental Retardation, Washington, DC.

The report by the President's Committee on Mental Retardation reviews the current state of knowledge in the area of biological and environmental prevention of mental retardation and describes programs on the frontiers of research or service delivery. Section I examines programs that are effectively preventing mental retardation through biomedical…

AUTISTIC FEATURES IN CHILDREN WITH MENTAL RETARDATION

PubMed Central

Kar, Nilamadhab; Khanna, Rakesh; Kar, Gopal Chandra

1997-01-01

Most of the autistic disorder patients are also mentally retarded and many mentally retarded persons exhibit autistic symptoms. By using a standard instrument (Ritvo-Freeman Real Life Rating Scale) the autistic features of the mentally retarded children were studied. The study also examined the influence of age, sex and level of mental retardation on the occurrence of autistic symptoms. Children who came for consultation to child psychiatric unit were compared with those at a school for children with mental retardation receiving stimulation. Male children from child psychiatric unit had significantly higher scores than those from the school. Social and language impairment could be reliably identified and grouped. It was possible to diagnose the syndrome of autism in children with mental retardation in a significant number (9.6%)as compared to that was possible only clinically (1.9%). More number of children with severe/ profound mental retardation could be diagnosed as autistic. The autistic syndrome in children with mental retardation can be picked up more effectively by the use of structured instrument. PMID:21584097

Deletion of PTEN Produces Deficits in Conditioned Fear and Increases Fragile X Mental Retardation Protein

ERIC Educational Resources Information Center

Lugo, Joaquin N.; Smith, Gregory D.; Morrison, Jessica B.; White, Jessika

2013-01-01

The phosphatase and tensin homolog detected on chromosome 10 (PTEN) gene product modulates activation of the phosphatidylinositol 3-kinase (PI3K)/AKT pathway. The PI3K pathway has been found to be involved in the regulation of the fragile X mental retardation protein, which is important for long-term depression and in the formation of new…

Mental Retardation in Perspective.

ERIC Educational Resources Information Center

Horvath, Michael; And Others

This monograph presents a general introduction to the history, classification, and characteristics of mental retardation. It begins with a discussion of the history of mental retardation from ancient Greece and Rome to the present. The beginnings of special education are traced to the early 19th century in Europe. Major influences in treatment of…

The Mentally Retarded in Sweden.

ERIC Educational Resources Information Center

Grunewald, Karl

Described are residential and educational services provided for mentally retarded (MC) children and adults in Sweden. Normalization is the focus of the services which make maximum use of mental and physical capacities to reduce the handicap of mental retardation. Described are general principles, and four stages involving development of services…

Fragile X mental retardation protein controls ion channel expression and activity.

PubMed

Ferron, Laurent

2016-10-15

Fragile X-associated disorders are a family of genetic conditions resulting from the partial or complete loss of fragile X mental retardation protein (FMRP). Among these disorders is fragile X syndrome, the most common cause of inherited intellectual disability and autism. FMRP is an RNA-binding protein involved in the control of local translation, which has pleiotropic effects, in particular on synaptic function. Analysis of the brain FMRP transcriptome has revealed hundreds of potential mRNA targets encoding postsynaptic and presynaptic proteins, including a number of ion channels. FMRP has been confirmed to bind voltage-gated potassium channels (K v 3.1 and K v 4.2) mRNAs and regulates their expression in somatodendritic compartments of neurons. Recent studies have uncovered a number of additional roles for FMRP besides RNA regulation. FMRP was shown to directly interact with, and modulate, a number of ion channel complexes. The sodium-activated potassium (Slack) channel was the first ion channel shown to directly interact with FMRP; this interaction alters the single-channel properties of the Slack channel. FMRP was also shown to interact with the auxiliary β4 subunit of the calcium-activated potassium (BK) channel; this interaction increases calcium-dependent activation of the BK channel. More recently, FMRP was shown to directly interact with the voltage-gated calcium channel, Ca v 2.2, and reduce its trafficking to the plasma membrane. Studies performed on animal models of fragile X syndrome have revealed links between modifications of ion channel activity and changes in neuronal excitability, suggesting that these modifications could contribute to the phenotypes observed in patients with fragile X-associated disorders. © 2016 The Authors. The Journal of Physiology © 2016 The Physiological Society.

Educable Mentally Retarded, Level I.

ERIC Educational Resources Information Center

Suo, Minnie Alice; Willemin, Helen

Intended for teachers of special classes of educable mentally retarded children aged 6 to 8 (mental age = 3.5 to 4.9), the guide stresses skills necessary to the development of physical, personal and social, and vocational competency. An introduction defines philosophy and goals, outlines the educable mentally retarded program and the readiness…

Molecular investigation of mental retardation locus gene PRSS12 by linkage analysis

PubMed Central

Ali, Zafar; Babar, Masroor Ellahi; Ahmad, Jamil; Yousaf, Muhammad Zubair; Asif, Muhammad; Shah, Sajjad Ali

2011-01-01

The present study was carried out to determine the prevalence of families having mental retardation in Pakistani population. We enrolled seven mentally retarded (MR) families with two or more affected individuals. Family history was taken to minimize the chances of other abnormalities. Pedigrees were drawn using the Cyrillic software (version 2.1). The structure of pedigrees shows that all the marriages are consanguineous and the families have recessive mode of inheritance. All the families were studied by linkage analysis to mental retardation locus (MRT1)/gene PRSS12. Three STR markers (D4S191, D4S2392, and D4S3024) in vicinity of mental retardation (MR) locus (MRT1)/gene PRSS12 were amplified on all the sample of each family by PCR. The PCR products were then genotyped on non denaturing polyacrylamide gel electrophoresis (PAGE). The Haplotype were constructed to determine the pattern of inheritance and also to determine that a family was linked or unlinked to gene PRSS12. One out of the seven families was potentially linked to gene PRSS12, while the other six families remain unlinked. PMID:22090715

Molecular investigation of mental retardation locus gene PRSS12 by linkage analysis.

PubMed

Ali, Zafar; Babar, Masroor Ellahi; Ahmad, Jamil; Yousaf, Muhammad Zubair; Asif, Muhammad; Shah, Sajjad Ali

2011-05-01

The present study was carried out to determine the prevalence of families having mental retardation in Pakistani population. We enrolled seven mentally retarded (MR) families with two or more affected individuals. Family history was taken to minimize the chances of other abnormalities. Pedigrees were drawn using the Cyrillic software (version 2.1). The structure of pedigrees shows that all the marriages are consanguineous and the families have recessive mode of inheritance. All the families were studied by linkage analysis to mental retardation locus (MRT1)/gene PRSS12. Three STR markers (D4S191, D4S2392, and D4S3024) in vicinity of mental retardation (MR) locus (MRT1)/gene PRSS12 were amplified on all the sample of each family by PCR. The PCR products were then genotyped on non denaturing polyacrylamide gel electrophoresis (PAGE). The Haplotype were constructed to determine the pattern of inheritance and also to determine that a family was linked or unlinked to gene PRSS12. One out of the seven families was potentially linked to gene PRSS12, while the other six families remain unlinked.

Fragile X mental retardation protein controls synaptic vesicle exocytosis by modulating N-type calcium channel density

NASA Astrophysics Data System (ADS)

Ferron, Laurent; Nieto-Rostro, Manuela; Cassidy, John S.; Dolphin, Annette C.

2014-04-01

Fragile X syndrome (FXS), the most common heritable form of mental retardation, is characterized by synaptic dysfunction. Synaptic transmission depends critically on presynaptic calcium entry via voltage-gated calcium (CaV) channels. Here we show that the functional expression of neuronal N-type CaV channels (CaV2.2) is regulated by fragile X mental retardation protein (FMRP). We find that FMRP knockdown in dorsal root ganglion neurons increases CaV channel density in somata and in presynaptic terminals. We then show that FMRP controls CaV2.2 surface expression by targeting the channels to the proteasome for degradation. The interaction between FMRP and CaV2.2 occurs between the carboxy-terminal domain of FMRP and domains of CaV2.2 known to interact with the neurotransmitter release machinery. Finally, we show that FMRP controls synaptic exocytosis via CaV2.2 channels. Our data indicate that FMRP is a potent regulator of presynaptic activity, and its loss is likely to contribute to synaptic dysfunction in FXS.

Mental Retardation; Its Social Context and Social Consequences.

ERIC Educational Resources Information Center

Farber, Bernard

Concerned with mental retardation as a social product, the following topics are discussed: mental retardation as a social phenomenon, the concept of the retarded as surplus population, labeling and incompetence in relation to life chances, mental retardation as deviance and as incompetence, and findings on the prevalence of retardation in the…

PERSPECTIVES IN MENTAL RETARDATION.

ERIC Educational Resources Information Center

JORDAN, THOMAS E.

THIRTY-THREE ARTICLES ILLUSTRATE THE EDITOR'S FORMULATION OF ISSUES CONCERNING THE PROBLEM OF MENTAL RETARDATION--(1) THE TRIPARTITE (ONTOLOGICAL, FUNCTIONAL, AND EDOLOGICAL) NATURE OF RETARDATION, (2) THE NECESSITY TO MOVE BEYOND A SINGLE DISCIPLINE IN DEALING WITH RETARDATION, (3) THE NUMBER OF AGENCIES NOW INVOLVED IN STUDY AND CARE, AND (4)…

THE PATHOLOGY OF MENTAL RETARDATION.

ERIC Educational Resources Information Center

CROME, L.; STERN, J.

DATA FROM RECENT COMPREHENSIVE STUDIES OF THE PATHOLOGY OF MENTAL RETARDATION ARE ASSEMBLED, INCLUDING MATERIAL ON ETIOLOGY, MORPHOLOGY, BIOCHEMISTRY, AND LABORATORY DIAGNOSIS. AREAS COVERED ARE (1) GENETIC CAUSES OF MENTAL RETARDATION, (2) DISORDERS OF GESTATION, (3) BIRTH INJURY, (4) GENERAL CONSIDERATIONS OF POSTNATAL CAUSES OF MENTAL…

People with Mental Retardation Are Dying, Legally.

ERIC Educational Resources Information Center

Keyes, Denis; And Others

1997-01-01

Criticizes the institution of the death penalty for convicted criminals with mental retardation. Examples are given of cases in which juries were not told of the defendant's mental retardation before sentencing, and a list of defendants with mental retardation that have been executed since 1976 is provided. (CR)

Reduced Cortical Thickness in Mental Retardation

PubMed Central

Wang, Chao; Wang, Jiaojian; Zhang, Yun; Yu, Chunshui; Jiang, Tianzi

2011-01-01

Mental retardation is a developmental disorder associated with impaired cognitive functioning and deficits in adaptive behaviors. Many studies have addressed white matter abnormalities in patients with mental retardation, while the changes of the cerebral cortex have been studied to a lesser extent. Quantitative analysis of cortical integrity using cortical thickness measurement may provide new insights into the gray matter pathology. In this study, cortical thickness was compared between 13 patients with mental retardation and 26 demographically matched healthy controls. We found that patients with mental retardation had significantly reduced cortical thickness in multiple brain regions compared with healthy controls. These regions include the bilateral lingual gyrus, the bilateral fusiform gyrus, the bilateral parahippocampal gyrus, the bilateral temporal pole, the left inferior temporal gyrus, the right lateral orbitofrontal cortex and the right precentral gyrus. The observed cortical thickness reductions might be the anatomical substrates for the impaired cognitive functioning and deficits in adaptive behaviors in patients with mental retardation. Cortical thickness measurement might provide a sensitive prospective surrogate marker for clinical trials of neuroprotective medications. PMID:22216343

ECT IN MENTALLY RETARDED SUBJECTS WITH PSYCHIATRIC ILLNESS

PubMed Central

Chopra, V.K; Sinha, V.K.

2002-01-01

The mentally retarded subjects show a much higher prevalence of full range of psychiatric disorders than the non-retarded population. Whereas the role of psychotropic drugs in such patients is well discussed, the reports on the use of ECT are scarce. Many psychiatrists dealing with the mentally retarded psychiatric patients are reluctant to consider ECT due to lack of adequate experience. We report five mentally retarded patients with different psychiatric disorders who were successfully treated with ECT after failing adequate trials of pharmacotherapy. No disproportionately higher adverse events on account of mental retardation were observed. ECT need to be favorably considered in mentally retarded persons with psychiatric morbidity especially when treatment with psychotropic drugs either fail or is intolerable. PMID:21206883

Fragile X mental retardation protein: A paradigm for translational control by RNA-binding proteins.

PubMed

Chen, Eileen; Joseph, Simpson

2015-07-01

Translational control is a common mechanism used to regulate gene expression and occur in bacteria to mammals. Typically in translational control, an RNA-binding protein binds to a unique sequence in the mRNA to regulate protein synthesis by the ribosomes. Alternatively, a protein may bind to or modify a translation factor to globally regulate protein synthesis by the cell. Here, we review translational control by the fragile X mental retardation protein (FMRP), the absence of which causes the neurological disease, fragile X syndrome (FXS). Copyright © 2015 Elsevier B.V. and Société française de biochimie et biologie Moléculaire (SFBBM). All rights reserved.

Mutations in the Small GTPase Gene RAB39B Are Responsible for X-linked Mental Retardation Associated with Autism, Epilepsy, and Macrocephaly

PubMed Central

Giannandrea, Maila; Bianchi, Veronica; Mignogna, Maria Lidia; Sirri, Alessandra; Carrabino, Salvatore; D'Elia, Errico; Vecellio, Matteo; Russo, Silvia; Cogliati, Francesca; Larizza, Lidia; Ropers, Hans-Hilger; Tzschach, Andreas; Kalscheuer, Vera; Oehl-Jaschkowitz, Barbara; Skinner, Cindy; Schwartz, Charles E.; Gecz, Jozef; Van Esch, Hilde; Raynaud, Martine; Chelly, Jamel; de Brouwer, Arjan P.M.; Toniolo, Daniela; D'Adamo, Patrizia

2010-01-01

Human Mental Retardation (MR) is a common and highly heterogeneous pediatric disorder affecting around 3% of the general population; at least 215 X-linked MR (XLMR) conditions have been described, and mutations have been identified in 83 different genes, encoding proteins with a variety of function, such as chromatin remodeling, synaptic function, and intracellular trafficking. The small GTPases of the RAB family, which play an essential role in intracellular vesicular trafficking, have been shown to be involved in MR. We report here the identification of mutations in the small GTPase RAB39B gene in two male patients. One mutation in family X (D-23) introduced a stop codon seven amino acids after the start codon (c.21C > A; p.Y7X). A second mutation, in the MRX72 family, altered the 5′ splice site (c.215+1G > A) and normal splicing. Neither instance produced a protein. Mutations segregate with the disease in the families, and in some family members intellectual disabilities were associated with autism spectrum disorder, epileptic seizures, and macrocephaly. We show that RAB39B, a novel RAB GTPase of unknown function, is a neuronal-specific protein that is localized to the Golgi compartment. Its downregulation leads to an alteration in the number and morphology of neurite growth cones and a significant reduction in presynaptic buttons, suggesting that RAB39B is required for synapse formation and maintenance. Our results demonstrate developmental and functional neuronal alteration as a consequence of downregulation of RAB39B and emphasize the critical role of vesicular trafficking in the development of neurons and human intellectual abilities. PMID:20159109

Innovations in Vocational Rehabilitation and Mental Retardation.

ERIC Educational Resources Information Center

Ayers, George E., Ed.

Conference proceedings of the Vocational Rehabilitation Subdivision Meetings held at the American Association on Mental Deficiency contain discussions of innovative aspects of vocational rehabilitation and mental retardation. In the area of training rehabilitation counselors, George Baroff describes the Mental Retardation Training Institute in…

Teaching the Educable Mentally Retarded.

ERIC Educational Resources Information Center

Love, Harold D.

The text discusses the behavior, evaluation, and education of mentally retarded children. Harold D. Love presents an overview of the retarded, a description of intelligence and personality tests, and a historical survey of retardation; Virginia Cantrell reviews the educational philosophies and methods of Itard, Seguin, and Montessori. Shirley K.…

Learning and Behavioral Deficits Associated with the Absence of the Fragile X Mental Retardation Protein: What a Fly and Mouse Model Can Teach Us

ERIC Educational Resources Information Center

Santos, Ana Rita; Kanellopoulos, Alexandros K.; Bagni, Claudia

2014-01-01

The Fragile X syndrome (FXS) is the most frequent form of inherited mental disability and is considered a monogenic cause of autism spectrum disorder. FXS is caused by a triplet expansion that inhibits the expression of the "FMR1" gene. The gene product, the Fragile X Mental Retardation Protein (FMRP), regulates mRNA metabolism in brain…

The amino-terminal structure of human fragile X mental retardation protein obtained using precipitant-immobilized imprinted polymers

NASA Astrophysics Data System (ADS)

Hu, Yufeng; Chen, Zhenhang; Fu, Yanjun; He, Qingzhong; Jiang, Lun; Zheng, Jiangge; Gao, Yina; Mei, Pinchao; Chen, Zhongzhou; Ren, Xueqin

2015-03-01

Flexibility is an intrinsic property of proteins and essential for their biological functions. However, because of structural flexibility, obtaining high-quality crystals of proteins with heterogeneous conformations remain challenging. Here, we show a novel approach to immobilize traditional precipitants onto molecularly imprinted polymers (MIPs) to facilitate protein crystallization, especially for flexible proteins. By applying this method, high-quality crystals of the flexible N-terminus of human fragile X mental retardation protein are obtained, whose absence causes the most common inherited mental retardation. A novel KH domain and an intermolecular disulfide bond are discovered, and several types of dimers are found in solution, thus providing insights into the function of this protein. Furthermore, the precipitant-immobilized MIPs (piMIPs) successfully facilitate flexible protein crystal formation for five model proteins with increased diffraction resolution. This highlights the potential of piMIPs for the crystallization of flexible proteins.

A BIBLIOGRAPHY ON MENTAL RETARDATION. NUMBER I.

ERIC Educational Resources Information Center

GELHART, ROBERT P.

INCLUDING BOOKS, JOURNAL ARTICLES, THESES, RESEARCH REPORTS, MONOGRAPHS, AND GOVERNMENT PUBLICATIONS, THE BIBLIOGRAPHY LISTS 105 ITEMS ON MENTAL RETARDATION. PUBLICATION DATES RANGE FROM 1931 THROUGH 1966 (IN PRESS). THE ITEMS CITED COVER CONCEPTS OF MENTAL RETARDATION, EDUCATIONAL PERFORMANCE OF THE MENTALLY HANDICAPPED, AND PROGRAMS FOR THEM. A…

A National Mental Retardation Manpower Model.

ERIC Educational Resources Information Center

National Inst. on Mental Retardation, Toronto (Ontario).

The report of the Task Force established by the Canadian National Institute on Mental Retardation concerns a comprehensive planning and organization program for training personnel, especially associate professionals, in the field of mental retardation to provide a more effective utilization of manpower. Summarized are results of surveys of…

Regulation of GABAA receptors by fragile X mental retardation protein

PubMed Central

Liu, Baosong; Li, Lijun; Chen, Juan; Wang, Zefen; Li, Zhiqiang; Wan, Qi

2013-01-01

Fragile X syndrome (FXS) is caused by the loss of fragile X mental retardation protein (FMRP). The deficiency of GABAA receptors (GABAARs) is implicated in FXS. However, the underlying mechanisms remain unclear. To investigate the effect of FMRP on GABAARs, we transfected FMRP cDNAs in rat cortical neurons. We measured the protein expression of GABAARs and phosphatase PTEN, and recorded GABAAR-mediated whole-cell currents in the transfected neurons. We show that the transfection of FMRP cDNAs causes increased protein expression of GABAARs in cortical neurons, but GABAAR-mediated whole-cell currents are not potentiated by FMRP transfection. These results suggest the possibility that intracellular signaling antagonizing GABAAR activity may play a role in inhibiting GABAAR function in FMRP-transfected neurons. We further show that FMRP transfection results in an enhanced protein expression of PTEN, which contributes to the inhibition of GABAAR function in FMRP-transfected neurons. These results indicate that GABAARs are regulated by FMRP through both an up-regulation of GABAAR expression and a PTEN enhancement-induced inhibition of GABAAR function, suggesting that an abnormal regulation of GABAAR and PTEN by the loss of FMRP underlies the pathogenesis of FXS. PMID:24044036

Noncitizen: Plight of the Mentally Retarded

ERIC Educational Resources Information Center

Skarnulis, Ed

1974-01-01

Mentally retarded citizens have been denied their human and civil rights, not only by the public, but by professionals--including social workers. The author claims that most programs for the mentally retarded are, at best, dehumanizing. Professionals have an ethical obligation to refuse to refer children to such programs. (Author)

Suspected X-linked facial dysmorphia and growth retardation in related Labrador retriever puppies.

PubMed

Dierks, C; Hoffmann, H; Heinrich, F; Hellige, M; Hewicker-Trautwein, M; Distl, O

2017-02-01

Seven male Labrador retriever puppies from four different litters were identified with a brachycephalic-like face and skull, associated with low birth weight, severe growth retardation, and reduced abilities to crawl and suckle, which were not compatible with survival. Excessive doming of the cranium, brachygnathia superior and inferior, and an abnormally opened fontanelle were found in all affected puppies by computed tomography and at post-mortem examination. Pedigree analysis supported an X-linked recessive mode of inheritance. Copyright © 2017 Elsevier Ltd. All rights reserved.

Direct versus indirect molecular diagnosis of fragile X mental retardation in 40 German families at risk.

PubMed

Knobloch, O; Pelz, F; Wick, U; Nelson, D L; Zoll, B

1993-03-01

In order to test whether the direct molecular diagnostic approach for fragile X mental retardation (Martin-Bell syndrome, MBS) really makes diagnosis of this disease more precise, we evaluated the results of direct diagnosis in 40 German families at risk together with the results of an earlier study with closely linked flanking markers in the same families. Of 84 men analysed, 43 showed clinical signs. In 39 of these affected men the disease could be confirmed by direct diagnosis. Compared to cytogenetic data, one man was false negative and two were false positive. Two men, whose status could not be determined by means of RFLP data, proved to be normal transmitting males (NTMs). However, the possibility of being an NTM had to be rejected in one case on RFLP data. Fragile X syndrome could be confirmed in 10 of the 13 women with clinical signs. Compared to cytogenetic data there were three cases of false negative results and one of false positive. All 36 obligate carrier women were detected by the direct approach. In addition, 22 women were newly identified as normal transmitting females (NTFs), among them one woman who could not be identified by cytogenetic means or by analysis with closely linked markers. These findings are discussed in view of the relative reliability of the three diagnostic approaches to MBS. Special attention is drawn to the significance of false negative and false positive results in direct diagnosis.

Fragile X mental retardation protein stimulates ribonucleoprotein assembly of influenza A virus

NASA Astrophysics Data System (ADS)

Zhou, Zhuo; Cao, Mengmeng; Guo, Yang; Zhao, Lili; Wang, Jingfeng; Jia, Xue; Li, Jianguo; Wang, Conghui; Gabriel, Gülsah; Xue, Qinghua; Yi, Yonghong; Cui, Sheng; Jin, Qi; Wang, Jianwei; Deng, Tao

2014-02-01

The ribonucleoprotein (RNP) of the influenza A virus is responsible for the transcription and replication of viral RNA in the nucleus. These processes require interplay between host factors and RNP components. Here, we report that the Fragile X mental retardation protein (FMRP) targets influenza virus RNA synthesis machinery and facilitates virus replication both in cell culture and in mice. We demonstrate that FMRP transiently associates with viral RNP and stimulates viral RNP assembly through RNA-mediated interaction with the nucleoprotein. Furthermore, the KH2 domain of FMRP mediates its association with the nucleoprotein. A point mutation (I304N) in the KH2 domain, identified from a Fragile X syndrome patient, disrupts the FMRP-nucleoprotein association and abolishes the ability of FMRP to participate in viral RNP assembly. We conclude that FMRP is a critical host factor used by influenza viruses to facilitate viral RNP assembly. Our observation reveals a mechanism of influenza virus RNA synthesis and provides insights into FMRP functions.

Alterations of amino acids and monoamine metabolism in male Fmr1 knockout mice: a putative animal model of the human fragile X mental retardation syndrome.

PubMed

Gruss, M; Braun, K

2001-01-01

The Fragile X syndrome, a common form of mental retardation in humans, is caused by silencing the fragile X mental retardation (FMR1) gene leading to the absence of the encoded fragile X mental retardation protein 1 (FMRP). We describe morphological and behavioral abnormalities for both affected humans and Fmr1 knockout mice, a putative animal model for the human Fragile X syndrome. The aim of the present study was to identify possible neurochemical abnormalities in Fmr1 knockout mice, with particular focus on neurotransmission. Significant region-specific differences of basal neurotransmitter and metabolite levels were found between wildtype and Fmr1 knockout animals, predominantly in juveniles (post-natal days 28 to 31). Adults (postnatal days 209 to 221) showed only few abnormalities as compared with the wildtype. In juvenile knockout mice, aspartate and taurine were especially increased in cortical regions, striatum, hippocampus, cerebellum, and brainstem. In addition, juveniles showed an altered balance between excitatory and inhibitory amino acids in the caudal cortex, hippocampus, and brainstem. We detected very few differences in monoamine turnover in both age stages. The results presented here provide the first evidence that lack of FMRP expression in FMRP knockout mice is accompanied by age-dependent, region-specific alterations in neurotransmission.

Identifying Depression in Students with Mental Retardation.

ERIC Educational Resources Information Center

Stough, Laura M.; Baker, Lynn

1999-01-01

Offers guidelines to teachers for identifying depression in students with mental retardation. Discusses prevalence and symptoms of depression, causes of depression, difficulty of diagnosis in students with mental retardation, detecting symptoms in the classroom, treatment of depression, and psychological services. Inserts list ideas for helping…

Towards a National Mental Retardation Manpower Model for Canada.

ERIC Educational Resources Information Center

Linton, Thomas E.

The stated need for developing a national mental retardation manpower model for Canada is not the manpower shortages in mental retardation, but the unsound conceptual and functional approaches to the socialization and education of the mentally retarded. The report is divided into the four major areas investigated by a task force. First, the…

Drosophila fragile X mental retardation protein and metabotropic glutamate receptor A convergently regulate the synaptic ratio of ionotropic glutamate receptor subclasses.

PubMed

Pan, Luyuan; Broadie, Kendal S

2007-11-07

A current hypothesis proposes that fragile X mental retardation protein (FMRP), an RNA-binding translational regulator, acts downstream of glutamatergic transmission, via metabotropic glutamate receptor (mGluR) G(q)-dependent signaling, to modulate protein synthesis critical for trafficking ionotropic glutamate receptors (iGluRs) at synapses. However, direct evidence linking FMRP and mGluR function with iGluR synaptic expression is limited. In this study, we use the Drosophila fragile X model to test this hypothesis at the well characterized glutamatergic neuromuscular junction (NMJ). Two iGluR classes reside at this synapse, each containing common GluRIIC (III), IID and IIE subunits, and variable GluRIIA (A-class) or GluRIIB (B-class) subunits. In Drosophila fragile X mental retardation 1 (dfmr1) null mutants, A-class GluRs accumulate and B-class GluRs are lost, whereas total GluR levels do not change, resulting in a striking change in GluR subclass ratio at individual synapses. The sole Drosophila mGluR, DmGluRA, is also expressed at the NMJ. In dmGluRA null mutants, both iGluR classes increase, resulting in an increase in total synaptic GluR content at individual synapses. Targeted postsynaptic dmGluRA overexpression causes the exact opposite GluR phenotype to the dfmr1 null, confirming postsynaptic GluR subtype-specific regulation. In dfmr1; dmGluRA double null mutants, there is an additive increase in A-class GluRs, and a similar additive impact on B-class GluRs, toward normal levels in the double mutants. These results show that both dFMRP and DmGluRA differentially regulate the abundance of different GluR subclasses in a convergent mechanism within individual postsynaptic domains.

Severe Mental Retardation: From Theory to Practice.

ERIC Educational Resources Information Center

Bricker, Diane, Ed.; Filler, John, Ed.

Fourteen papers examine current issues and practices in the education of students with severe mental retardation (SMR). Papers touch upon the broad context of education for SMR students, programs for the SMR population, and critical issues. The following papers are presented: "The Severely Mentally Retarded Individual: Philosophical and…

38 CFR 4.127 - Mental retardation and personality disorders.

Code of Federal Regulations, 2010 CFR

2010-07-01

... AFFAIRS SCHEDULE FOR RATING DISABILITIES Disability Ratings Mental Disorders § 4.127 Mental retardation and personality disorders. Mental retardation and personality disorders are not diseases or injuries... from them may not be service-connected. However, disability resulting from a mental disorder that is...

Referential first mention in narratives by mildly mentally retarded adults.

PubMed

Kernan, K T; Sabsay, S

1987-01-01

Referential first mentions in narrative reports of a short film by 40 mildly mentally retarded adults and 20 nonretarded adults were compared. The mentally retarded sample included equal numbers of male and female, and black and white speakers. The mentally retarded speakers made significantly fewer first mentions and significantly more errors in the form of the first mentions than did nonretarded speakers. A pattern of better performance by black males than by other mentally retarded speakers was found. It is suggested that task difficulty and incomplete mastery of the use of definite and indefinite forms for encoding old and new information, rather than some global type of egocentrism, accounted for the poorer performance by mentally retarded speakers.

Conjunctive visual search in individuals with and without mental retardation.

PubMed

Carlin, Michael; Chrysler, Christina; Sullivan, Kate

2007-01-01

A comprehensive understanding of the basic visual and cognitive abilities of individuals with mental retardation is critical for understanding the basis of mental retardation and for the design of remediation programs. We assessed visual search abilities in individuals with mild mental retardation and in MA- and CA-matched comparison groups. Our goal was to determine the effect of decreasing target-distracter disparities on visual search efficiency. Results showed that search rates for the group with mental retardation and the MA-matched comparisons were more negatively affected by decreasing disparities than were those of the CA-matched group. The group with mental retardation and the MA-matched group performed similarly on all tasks. Implications for theory and application are discussed.

Mapping Self-Reports of Working Memory Deficits to Executive Dysfunction in Fragile X Mental Retardation 1 ("FMR1") Gene Premutation Carriers Asymptomatic for FXTAS

ERIC Educational Resources Information Center

Kogan, Cary S.; Cornish, Kim M.

2010-01-01

Fragile X Syndrome is a neurodevelopmental disorder that is caused by the silencing of a single gene on the X chromosome, the Fragile X Mental Retardation 1 ("FMR1") gene. In recent years, the premutation ("carrier") status has received considerable attention and there is now an emerging consensus that despite intellectual functioning being within…

Strongyloidiasis in an institution for mentally retarded adults.

PubMed

Braun, T I; Fekete, T; Lynch, A

1988-03-01

A serologic and epidemiologic survey was undertaken to explain the high incidence of eosinophilia (14.1%) in nine of 64 residents of a facility for mentally retarded adults in Pennsylvania. Seven residents had antibodies to Strongyloides stercoralis as determined by an IgG enzyme-linked immunosorbent assay system (10.9%) and five (71.4%) of this subset had persistent eosinophilia, suggesting a strong association. Sex, race, age, ward, degree of retardation, and medication were not associated with eosinophilia. Stool examination revealed S stercoralis larvae in two residents with S stercoralis antibodies and eosinophilia. Follow-up studies after thiabendazole treatment of the residents with S stercoralis antibodies showed resolution of eosinophilia and disappearance of S stercoralis antibodies in most. Eosinophilia in an adult institutional setting may be associated with strongyloidiasis, even in a nonendemic area. The enzyme-linked immunosorbent assay for antibody to S stercoralis may be useful in diagnosing or monitoring therapy of strongyloidiasis.

Arm Tremor, Tardive Dyskinesia, and Mental Retardation.

ERIC Educational Resources Information Center

van Emmerik, R. E. A.; And Others

1993-01-01

The arm tremor of adults (n=32) diagnosed as having mental retardation and/or tardive dyskinesia was examined through an analysis of the acceleration properties of several arm postures. The degree of arm acceleration was increased in all subjects compared to a control group without mental retardation. Effects of neuroleptic medication were noted.…

Mental Illness in Persons with Mental Retardation: ARC Facts.

ERIC Educational Resources Information Center

Weber, Linda R.; Wimmer, Sharon

This brief factsheet presents information on mental illness in mentally retarded persons. The most prevalent disorders found in this population are schizophrenia, organic brain syndrome, adjustment disorders, personality disorders, depression, and behavioral problems. Few standardized methods of assessment exist for the diagnosis of mental illness…

Reactions to the Labels "Institutionalized" and "Mentally Retarded" by Retarded and Nonretarded Persons.

ERIC Educational Resources Information Center

Gibbons, Frederick X.; Gibbons, Barbara N.

The effects of labels, "mentally retarded" and "institutionalized" on the evaluations and causal attributions of nonretarded persons, and on the social distance preferences of EMR persons, were assessed. In addition, each group was asked to predict the likelihood of a labeled (mentally retarded) or a nonlabeled target person achieving success at a…

Body Awareness in Children with Mental Retardation

ERIC Educational Resources Information Center

Simons, Johan; Dedroog, Inge

2009-01-01

The body awareness of 124 toddlers with mental retardation and of 124 children developing normally matched to them on age and gender was examined. Twenty-nine of the children with mental retardation were diagnosed as Down syndrome (DS). The "Pointing and Naming" Test of Berges and Lezine [Berges, J., & Lezine, I. (1978). "Test d'imitation de…

Conjunctive Visual Search in Individuals with and without Mental Retardation

ERIC Educational Resources Information Center

Carlin, Michael; Chrysler, Christina; Sullivan, Kate

2007-01-01

A comprehensive understanding of the basic visual and cognitive abilities of individuals with mental retardation is critical for understanding the basis of mental retardation and for the design of remediation programs. We assessed visual search abilities in individuals with mild mental retardation and in MA- and CA-matched comparison groups. Our…

The Van Hiele geometry thinking levels of mild mental retardation students

NASA Astrophysics Data System (ADS)

Shomad, Z. A.; Kusmayadi, T. A.; Riyadi

2017-12-01

This research is to investigate the level of mild mental retardation geometry students thinking. This research focuses on the geometry thinking level based on Van Hiele theory. This study uses qualitative methods with case study strategy. Data obtained from observation and tests result. The subjects are 12 mental retardation students. The result show that ability of mild mental retardation students with each other is different but have same level of level thinking geometry. The geometry thinking level of mental retardation students was identified in level 1 of the Van Hiele theory. Based on the level thinking geometry of mental retardation students simplify geometry thinking teachers in selecting appropriate learning methods, choose the materials in accordance with ability, and can modify the material following the geometry thinking level of mental retardation students.

Poverty and Mental Retardation: A Causal Relationship.

ERIC Educational Resources Information Center

Hurley, Rodger L.

The incidence of mental retardation among the poor and the reasons for such high prevalence are the focus of the text which is based largely on the state of New Jersey. Mental retardation is viewed as a social pathology which thrives in the ghetto; the effects of poverty and racial prejudice are explored as are the assessment of intelligence and…

Expression of fragile X mental retardation protein and Fmr1 mRNA during folliculogenesis in the rat.

PubMed

Ferder, Ianina; Parborell, Fernanda; Sundblad, Victoria; Chiauzzi, Violeta; Gómez, Karina; Charreau, Eduardo H; Tesone, Marta; Dain, Liliana

2013-04-01

Fragile X mental retardation protein (FMRP) belongs to a small family of RNA-binding proteins. Its absence or inactivity is responsible for fragile X syndrome, the most common cause of inherited mental retardation. Despite its ubiquitous expression, FMRP function and expression remain almost understudied in non-neuronal tissues, though previous studies on germline development during oogenesis may suggest a special function of this protein also in ovarian tissue. In addition, the well-documented association of FMR1 premutation state with fragile X-related premature ovarian insufficiency adds interest to the role of FMRP in ovarian physiology. The aim of the present work was to investigate the expression of Fmr1 mRNA and its protein, FMRP, at different stages of rat follicular development. By immunohistochemical studies we demonstrated FMRP expression in granulosa, theca and germ cells in all stages of follicular development. In addition, changes in Fmr1 expression, both at the protein and mRNA levels, were observed. FMRP levels increased upon follicular development while preantral and early antral follicles presented similar levels of Fmr1 transcripts with decreased expression in preovulatory follicles. These observations suggest that Fmr1 expression in the ovary is regulated at different and perhaps independent levels. In addition, our results show expression of at least four different isoforms of FMRP during all stages of follicular growth with expression patterns that differ from those observed in brain and testis. Our study shows a regulated expression of Fmr1, both at mRNA and protein levels, during rat follicular development.

Evaluation of Community Residential Programs for Mentally Retarded Persons.

ERIC Educational Resources Information Center

Minnesota State Office of the Legislative Auditor, St. Paul. Program Evaluation Div.

The report evaluates how Minnesota plans, regulates, and finances residential services for mentally retarded persons. The first chapter reviews descriptive findings about the mentally retarded population in the state and notes that despite a decline in the number of state hospital residents, the total number of retarded persons in long-term care…

High-Throughput Screening to Identify Compounds That Increase Fragile X Mental Retardation Protein Expression in Neural Stem Cells Differentiated From Fragile X Syndrome Patient-Derived Induced Pluripotent Stem Cells.

PubMed

Kumari, Daman; Swaroop, Manju; Southall, Noel; Huang, Wenwei; Zheng, Wei; Usdin, Karen

2015-07-01

: Fragile X syndrome (FXS), the most common form of inherited cognitive disability, is caused by a deficiency of the fragile X mental retardation protein (FMRP). In most patients, the absence of FMRP is due to an aberrant transcriptional silencing of the fragile X mental retardation 1 (FMR1) gene. FXS has no cure, and the available treatments only provide symptomatic relief. Given that FMR1 gene silencing in FXS patient cells can be partially reversed by treatment with compounds that target repressive epigenetic marks, restoring FMRP expression could be one approach for the treatment of FXS. We describe a homogeneous and highly sensitive time-resolved fluorescence resonance energy transfer assay for FMRP detection in a 1,536-well plate format. Using neural stem cells differentiated from an FXS patient-derived induced pluripotent stem cell (iPSC) line that does not express any FMRP, we screened a collection of approximately 5,000 known tool compounds and approved drugs using this FMRP assay and identified 6 compounds that modestly increase FMR1 gene expression in FXS patient cells. Although none of these compounds resulted in clinically relevant levels of FMR1 mRNA, our data provide proof of principle that this assay combined with FXS patient-derived neural stem cells can be used in a high-throughput format to identify better lead compounds for FXS drug development. In this study, a specific and sensitive fluorescence resonance energy transfer-based assay for fragile X mental retardation protein detection was developed and optimized for high-throughput screening (HTS) of compound libraries using fragile X syndrome (FXS) patient-derived neural stem cells. The data suggest that this HTS format will be useful for the identification of better lead compounds for developing new therapeutics for FXS. This assay can also be adapted for FMRP detection in clinical and research settings. ©AlphaMed Press.

The Fragile X Mental Retardation Syndrome 20 Years After the FMR1 Gene Discovery: an Expanding Universe of Knowledge

PubMed Central

Rousseau, François; Labelle, Yves; Bussières, Johanne; Lindsay, Carmen

2011-01-01

The fragile X mental retardation (FXMR) syndrome is one of the most frequent causes of mental retardation. Affected individuals display a wide range of additional characteristic features including behavioural and physical phenotypes, and the extent to which individuals are affected is highly variable. For these reasons, elucidation of the pathophysiology of this disease has been an important challenge to the scientific community. 1991 marks the year of the discovery of both the FMR1 gene mutations involved in this disease, and of their dynamic nature. Although a mouse model for the disease has been available for 16 years and extensive research has been performed on the FMR1 protein (FMRP), we still understand little about how the disease develops, and no treatment has yet been shown to be effective. In this review, we summarise current knowledge on FXMR with an emphasis on the technical challenges of molecular diagnostics, on its prevalence and dynamics among populations, and on the potential of screening for FMR1 mutations. PMID:21912443

The fragile x mental retardation syndrome 20 years after the FMR1 gene discovery: an expanding universe of knowledge.

PubMed

Rousseau, François; Labelle, Yves; Bussières, Johanne; Lindsay, Carmen

2011-08-01

The fragile X mental retardation (FXMR) syndrome is one of the most frequent causes of mental retardation. Affected individuals display a wide range of additional characteristic features including behavioural and physical phenotypes, and the extent to which individuals are affected is highly variable. For these reasons, elucidation of the pathophysiology of this disease has been an important challenge to the scientific community. 1991 marks the year of the discovery of both the FMR1 gene mutations involved in this disease, and of their dynamic nature. Although a mouse model for the disease has been available for 16 years and extensive research has been performed on the FMR1 protein (FMRP), we still understand little about how the disease develops, and no treatment has yet been shown to be effective. In this review, we summarise current knowledge on FXMR with an emphasis on the technical challenges of molecular diagnostics, on its prevalence and dynamics among populations, and on the potential of screening for FMR1 mutations.

The difficulties of teacher in teaching geometry for mental retardation students

NASA Astrophysics Data System (ADS)

Shomad, Z. A.; Kusmayadi, T. A.; Riyadi

2018-03-01

The purpose of this research is to find out the problems faced by teachers in teaching materials on mental retardation students. It focused on the difficulties faced by the teacher in teaching geometry. A qualitative method with field study approach used in this study. The subjects in this research are the teacher and mild mental retardation students. There are six teachers and six students involve as the subject which is chosen by purposive sampling. The data of this research is the observation and interview against teachers and mental retardation students. The data was analyzed qualitatively with Miles and Huberman steps. The results of this research show that mental retardation students have less attention to the materials, less special books or learning media props, difficult in the set the students, and the difficulty in choosing the material that suits the student needs and the condition of mental retardation students. There's not much pay attention to the children with special need, particularly mental retardation student. Thus, this study can help analyze the difficulties teachers so that learning math for mental retardation students more optimal.

Socioeconomic Status and the Prevalence of Mental Retardation in Bangladesh.

ERIC Educational Resources Information Center

Islam, Shaheen; And Others

1993-01-01

A population-based study of the prevalence of mental retardation among children (ages 2-9) in Bangladesh screened 10,000 children and found the prevalence rates of 5.9 per thousand for severe and 14.4 per thousand for mild mental retardation. Only the prevalence of mild mental retardation was strongly associated with low socioeconomic status.…

Epilepsy and Mental Retardation Limited to Females: An Under-Recognized Disorder

ERIC Educational Resources Information Center

Scheffer, Ingrid E.; Turner, Samantha J.; Dibbens, Leanne M.; Bayly, Marta A.; Friend, Kathryn; Hodgson, Bree; Burrows, Linda; Shaw, Marie; Wei, Chen; Ullmann, Reinhard; Ropers, Hans-Hilger; Szepetowski, Pierre; Haan, Eric; Mazarib, Aziz; Afawi, Zaid; Neufeld, Miriam Y.; Andrews, P. Ian; Wallace, Geoffrey; Kivity, Sara; Lev, Dorit; Lerman-Sagie, Tally; Derry, Christopher P.; Korczyn, Amos D.; Gecz, Jozef; Mulley, John C.; Berkovic, Samuel F.

2008-01-01

Epilepsy and Mental Retardation limited to Females (EFMR) which links to Xq22 has been reported in only one family. We aimed to determine if there was a distinctive phenotype that would enhance recognition of this disorder. We ascertained four unrelated families (two Australian, two Israeli) where seizures in females were transmitted through…

The fragile X mental retardation protein regulates tumor invasiveness-related pathways in melanoma cells.

PubMed

Zalfa, Francesca; Panasiti, Vincenzo; Carotti, Simone; Zingariello, Maria; Perrone, Giuseppe; Sancillo, Laura; Pacini, Laura; Luciani, Flavie; Roberti, Vincenzo; D'Amico, Silvia; Coppola, Rosa; Abate, Simona Osella; Rana, Rosa Alba; De Luca, Anastasia; Fiers, Mark; Melocchi, Valentina; Bianchi, Fabrizio; Farace, Maria Giulia; Achsel, Tilmann; Marine, Jean-Christophe; Morini, Sergio; Bagni, Claudia

2017-11-16

The fragile X mental retardation protein (FMRP) is lacking or mutated in patients with the fragile X syndrome (FXS), the most frequent form of inherited intellectual disability. FMRP affects metastasis formation in a mouse model for breast cancer. Here we show that FMRP is overexpressed in human melanoma with high Breslow thickness and high Clark level. Furthermore, meta-analysis of the TCGA melanoma data revealed that high levels of FMRP expression correlate significantly with metastatic tumor tissues, risk of relapsing and disease-free survival. Reduction of FMRP in metastatic melanoma cell lines impinges on cell migration, invasion and adhesion. Next-generation sequencing in human melanoma cells revealed that FMRP regulates a large number of mRNAs involved in relevant processes of melanoma progression. Our findings suggest an association between FMRP levels and the invasive phenotype in melanoma and might open new avenues towards the discovery of novel therapeutic targets.

The fragile X mental retardation protein regulates tumor invasiveness-related pathways in melanoma cells

PubMed Central

Zalfa, Francesca; Panasiti, Vincenzo; Carotti, Simone; Zingariello, Maria; Perrone, Giuseppe; Sancillo, Laura; Pacini, Laura; Luciani, Flavie; Roberti, Vincenzo; D'Amico, Silvia; Coppola, Rosa; Abate, Simona Osella; Rana, Rosa Alba; De Luca, Anastasia; Fiers, Mark; Melocchi, Valentina; Bianchi, Fabrizio; Farace, Maria Giulia; Achsel, Tilmann; Marine, Jean-Christophe; Morini, Sergio; Bagni, Claudia

2017-01-01

The fragile X mental retardation protein (FMRP) is lacking or mutated in patients with the fragile X syndrome (FXS), the most frequent form of inherited intellectual disability. FMRP affects metastasis formation in a mouse model for breast cancer. Here we show that FMRP is overexpressed in human melanoma with high Breslow thickness and high Clark level. Furthermore, meta-analysis of the TCGA melanoma data revealed that high levels of FMRP expression correlate significantly with metastatic tumor tissues, risk of relapsing and disease-free survival. Reduction of FMRP in metastatic melanoma cell lines impinges on cell migration, invasion and adhesion. Next-generation sequencing in human melanoma cells revealed that FMRP regulates a large number of mRNAs involved in relevant processes of melanoma progression. Our findings suggest an association between FMRP levels and the invasive phenotype in melanoma and might open new avenues towards the discovery of novel therapeutic targets. PMID:29144507

Structural Studies of the Tandem Tudor Domains of Fragile X Mental Retardation Related Proteins FXR1 and FXR2

DOE Office of Scientific and Technical Information (OSTI.GOV)

Adams-Cioaba, Melanie A.; Guo, Yahong; Bian, ChuanBing

Expansion of the CGG trinucleotide repeat in the 5'-untranslated region of the FMR1, fragile X mental retardation 1, gene results in suppression of protein expression for this gene and is the underlying cause of Fragile X syndrome. In unaffected individuals, the FMRP protein, together with two additional paralogues (Fragile X Mental Retardation Syndrome-related Protein 1 and 2), associates with mRNA to form a ribonucleoprotein complex in the nucleus that is transported to dendrites and spines of neuronal cells. It is thought that the fragile X family of proteins contributes to the regulation of protein synthesis at sites where mRNAs aremore » locally translated in response to stimuli. Here, we report the X-ray crystal structures of the non-canonical nuclear localization signals of the FXR1 and FXR2 autosomal paralogues of FMRP, which were determined at 2.50 and 1.92 {angstrom}, respectively. The nuclear localization signals of the FXR1 and FXR2 comprise tandem Tudor domain architectures, closely resembling that of UHRF1, which is proposed to bind methylated histone H3K9. The FMRP, FXR1 and FXR2 proteins comprise a small family of highly conserved proteins that appear to be important in translational regulation, particularly in neuronal cells. The crystal structures of the N-terminal tandem Tudor domains of FXR1 and FXR2 revealed a conserved architecture with that of FMRP. Biochemical analysis of the tandem Tudor doamins reveals their ability to preferentially recognize trimethylated peptides in a sequence-specific manner.« less

Programs for Preventing the Causes of Mental Retardation.

ERIC Educational Resources Information Center

Oliphant, Peter S.; And Others

This monograph, which reports findings from the New Jersey Governor's Council on the Prevention of Mental Retardation, discusses the scope of mental retardation (MR), its causes, identification of people at risk, and prevention methods. The Council cites several cost-effective prevention programs, such as vaccination programs and prenatal care…

Mental Health and Mental Retardation Services in Nevada.

ERIC Educational Resources Information Center

Kakalik, J. S.; And Others

Summarized are the findings and recommendations of a 2-year study of all major mental health, and mental retardation, alcohol, and drug abuse services and programs in Nevada. Fourteen chapters are given to the following topics (sample subtopics are in parentheses): description of the survey (scope of the project); summary and recommendations…

Mental Retardation in the Caribbean: Needs, Resources, Approaches.

ERIC Educational Resources Information Center

Thorburn, Marigold J., Ed.

Presented are conference reports including an opening address on the economic benefits of programs for the mentally retarded (MR), and eight papers discussing the problem of mental retardation in the Caribbean. Two papers on preschool age children, respectively, consider the identification and assessment of MR children in the Caribbean and present…

Carbamazepine-Induced Hyponatremia in Patients with Mental Retardation.

ERIC Educational Resources Information Center

Kastner, Ted; And Others

1992-01-01

This study of 40 patients with mental retardation receiving carbamazepine found hyponatremia in only 5 percent of these patients and found a statistically, but not clinically, significant decrease in serum sodium levels in patients receiving anticonvulsant polytherapy. Results support the use of this drug with patients with mental retardation and…

Low Elevated Lead Levels and Mental Retardation.

ERIC Educational Resources Information Center

Marlowe, Mike; And Others

The relationship between low elevated lead absorption and mild mental retardation was investigated in 40 rural children (preschool to grade 12) without demonstrable cause for their retardation. Trace mineral analysis of hair samples from Ss and a control group (N=20) indicated the mean hair lead concentrations for the retarded Ss were considerably…

Political Philosophy and the Mentally Retarded.

ERIC Educational Resources Information Center

Stanovich, Keith E.

The effects of Social Darwinism, eugenics, and contemporary political conservatism on the status of advocacy efforts for the mentally retarded are reviewed. Provided are historical sketches of Social Darwinism, which viewed the retarded as members of an inferior race, and eugenics, which argued for sterilization of the "genetically…

The Mentally Retarded Offender and Corrections.

ERIC Educational Resources Information Center

Santamour, Miles; West, Bernadette

The booklet provides an overview of the issues involved in correctional rehabilitation for the mentally retarded offender. Reviewed are clinical and legal definitions of criminal behavior and retardation, and discussed are such issues as law enforcement and court proceedings problems, pros and cons of special facilities, labeling, normalization,…

Mental Retardation Activities of the Department of Health, Education, and Welfare.

ERIC Educational Resources Information Center

Department of Health, Education, and Welfare, Washington, DC. Office of Mental Retardation Coordination.

Briefly described are eight mental retardation programs of the Department of Health, Education, and Welfare. Coordination is thought to be the most cricial factor in successful administration of the mental retardation programs. The mental retardation activities of the Department are arranged according to categories of preventive services, basic…

"Idiopathic" mental retardation and new chromosomal abnormalities

PubMed Central

2010-01-01

Mental retardation is a heterogeneous condition, affecting 1-3% of general population. In the last few years, several emerging clinical entities have been described, due to the advent of newest genetic techniques, such as array Comparative Genomic Hybridization. The detection of cryptic microdeletion/microduplication abnormalities has allowed genotype-phenotype correlations, delineating recognizable syndromic conditions that are herein reviewed. With the aim to provide to Paediatricians a combined clinical and genetic approach to the child with cognitive impairment, a practical diagnostic algorithm is also illustrated. The use of microarray platforms has further reduced the percentage of "idiopathic" forms of mental retardation, previously accounted for about half of total cases. We discussed the putative pathways at the basis of remaining "pure idiopathic" forms of mental retardation, highlighting possible environmental and epigenetic mechanisms as causes of altered cognition. PMID:20152051

Fragile X Mental Retardation Protein Regulates the Levels of Scaffold Proteins and Glutamate Receptors in Postsynaptic Densities*

PubMed Central

Schütt, Janin; Falley, Katrin; Richter, Dietmar; Kreienkamp, Hans-Jürgen; Kindler, Stefan

2009-01-01

Functional absence of fragile X mental retardation protein (FMRP) causes the fragile X syndrome, a hereditary form of mental retardation characterized by a change in dendritic spine morphology. The RNA-binding protein FMRP has been implicated in regulating postsynaptic protein synthesis. Here we have analyzed whether the abundance of scaffold proteins and neurotransmitter receptor subunits in postsynaptic densities (PSDs) is altered in the neocortex and hippocampus of FMRP-deficient mice. Whereas the levels of several PSD components are unchanged, concentrations of Shank1 and SAPAP scaffold proteins and various glutamate receptor subunits are altered in both adult and juvenile knock-out mice. With the exception of slightly increased hippocampal SAPAP2 mRNA levels in adult animals, altered postsynaptic protein concentrations do not correlate with similar changes in total and synaptic levels of corresponding mRNAs. Thus, loss of FMRP in neurons appears to mainly affect the translation and not the abundance of particular brain transcripts. Semi-quantitative analysis of RNA levels in FMRP immunoprecipitates showed that in the mouse brain mRNAs encoding PSD components, such as Shank1, SAPAP1–3, PSD-95, and the glutamate receptor subunits NR1 and NR2B, are associated with FMRP. Luciferase reporter assays performed in primary cortical neurons from knock-out and wild-type mice indicate that FMRP silences translation of Shank1 mRNAs via their 3′-untranslated region. Activation of metabotropic glutamate receptors relieves translational suppression. As Shank1 controls dendritic spine morphology, our data suggest that dysregulation of Shank1 synthesis may significantly contribute to the abnormal spine development and function observed in brains of fragile X syndrome patients. PMID:19640847

Epidemiology of Mental Retardation.

ERIC Educational Resources Information Center

Heber, Rick

Prevalence data on mental retardation is presented including international estimates on general prevalence, age directions, geographical variations within the United States, racial and ethnic variations, economic class distributions, family variations, and population distribution in institutions. Statistics are also provided in areas of specific…

Genetic Analysis of a Kindred With X-linked Mental Handicap and Retinitis Pigmentosa

PubMed Central

Aldred, M. A.; Dry, K. L.; Knight-Jones, E. B.; Hardwick, L. J.; Teague, P. W.; Lester, D. H.; Brown, J.; Spowart, G.; Carothers, A. D.; Raeburn, J. A.; Bird, A. C.; Fielder, A. R.; Wright, A. F.

1994-01-01

A kindred is described in which X-linked nonspecific mental handicap segregates together with retinitis pigmentosa. Carrier females are mentally normal but may show signs of the X-linked retinitis pigmentosa carrier state and become symptomatic in their later years. Analysis of polymorphic DNA markers at nine loci on the short arm of the X chromosome shows that no crossing-over occurs between the disease and Xp11 markers DXS255, TIMP, DXS426, MAOA, and DXS228. The 90% confidence limits show that the locus is in the Xp21-q21 region. Haplotype analysis is consistent with the causal gene being located proximal to the Xp21 loci DXS538 and 5'-dystrophin on the short arm of the X chromosome. The posterior probability of linkage to the RP2 region of the X chromosome short arm (Xp11.4-p11.23) is .727, suggesting the possibility of a contiguous-gene-deletion syndrome. No cytogenetic abnormality has been identified. PMID:7977353

Physical activity of adults with mental retardation: review and research needs.

PubMed

Temple, Viviene A; Frey, Georgia C; Stanish, Heidi I

2006-01-01

To characterize physical activity levels of adults with mental retardation and identify limitations in published research. Key word searches for "mental retardation," "intellectual disability," "learning disability," or "developmental disability" combined with "physical activity" or "habitual exercise" identified articles from MEDLINE, Academic Search Elite, Psych Articles, Health Source, and SPORT Discus. This produced a total of 801 citations. Published English-language literature that quantitatively measured physical activity levels of adults with mental retardation was included in this review. Fourteen articles met this criterion. Characteristics of participants, study design, outcome measures, methods of analyses, and findings in terms of percentages, step counts, and accelerometer output were extracted. Data were synthesized to identify the percentage of adults with mental retardation who met published health-related physical activity criteria and compare them with adults without mental retardation and to examine study limitations. The studies with the greatest rigor indicate that one-third of adults or fewer with mental retardation were sufficiently active to achieve health benefits. However, data are insufficient to determine whether adults with mental retardation are less active than the general community. Future research would be enhanced by including appropriately powered representative samples, by including comparison groups, by validating physical activity questionnaires, and by determining the accuracy of proxy respondents.

Mental Retardation: Definition, Classification, and Systems of Supports. 10th Edition.

ERIC Educational Resources Information Center

Luckasson, Ruth; Borthwick-Duffy, Sharon; Buntinx, Wil H. E.; Coulter, David L.; Craig, Ellis M.; Reeve, Alya; Schalock, Robert L.; Snell, Martha E.; Spitalnik, Deborah M.; Spreat, Scott; Tasse, Marc J.

This manual, the 10th edition of a regularly published definition and classification work on mental retardation, presents five key assumptions upon which the definition of mental retardation is based and a theoretical model of five essential dimensions that explain mental retardation and how to use the companion system. These dimensions include…

Mental Retardation Activities of the Department of Health, Education, and Welfare.

ERIC Educational Resources Information Center

1970

Introduced by discussions of the coordination of mental retardation programs, a summary of activities, and a list of the Secretary's Committee on Mental Retardation for 1969, the pamphlet reports the activities of the U.S. Department of Health, Education, and Welfare in mental retardation. Activities described include those of the Health Services…

Characteristics of Those Persons with Mental Retardation Registered with the Mental Retardation Division.

ERIC Educational Resources Information Center

Cocks, Errol; Ng, Pin Chee

1983-01-01

The paper discusses an analysis of a computerized data bank on the mentally retarded population in Victoria, Australia. Prevalence rates, severity of handicap, age, sex, and residence type are reviewed and implications for community vocational and residential services for adults are noted. (CL)

ARX polyalanine expansions are highly implicated in familial cases of mental retardation with infantile epilepsy and/or hand dystonia.

PubMed

Cossée, Mireille; Faivre, Laurence; Philippe, Christophe; Hichri, Heifa; de Saint-Martin, Anne; Laugel, Vincent; Bahi-Buisson, Nadia; Lemaitre, Jean-François; Leheup, Bruno; Delobel, Bruno; Demeer, Bénédicte; Poirier, Karine; Biancalana, Valérie; Pinoit, Jean-Michel; Julia, Sophie; Chelly, Jamel; Devys, Didier; Mandel, Jean-Louis

2011-01-01

Mutations in the ARX gene cause both nonsyndromic and several forms of syndromic mental retardation (MR). Two polyalanine (polyA) expansions of ARX are recurrent mutations. The most common one, the c.428_451dup, is associated with a wide spectrum of phenotypes, ranging from the most severe West syndrome to Partington syndrome (MR and hand dystonia), and even nonsyndromic X-linked mental retardation (NS-XLMR). Studies of patients not selected for specific clinical signs showed that the c.428_451dup is relatively frequent in families harboring X-linked MR (7.5%), but less common in familial cases compatible with X-linked NR (1%), and very rare in sporadic cases (0.1%). The c.333_334ins(GCG)7 expansion is less frequent and mainly associated with West syndrome. We screened for both ARX polyA expansions in 98 unrelated patients selected for the presence of NR associated with different types of epilepsy and/or with hand dystonia. We also studied two families with an initial diagnosis of NS-XLMR, one of which was identified as showing linkage to the ARX locus. The c.428_451dup was identified in three patients and the c.333_334ins(GCG)7 in one; all of the patients were from families with two affected brothers. We also found the c.428_451dup in the family linked to ARX, and clinical re-evaluation showed subtle, previously undetected signs. Our study illustrates that ARX polyA expansions are primarily associated with syndromic MR and shows a higher yield (18% in our cohort) when these mutations are screened in familial cases of MR with epilepsy and/or dystonia. Copyright © 2010 Wiley-Liss, Inc.

Stimulating Physical Development of Mentally Retarded Children. Mental Retardation Training Program Technical Report Series 70-3.

ERIC Educational Resources Information Center

Stein, Julian U.; And Others

Four papers presented at an all-day workshop at Ohio State University focus on stimulating the physical development of mentally retarded children. Noted in the introduction is importance of cooperation between university training programs and facilities serving the mentally handicapped. Julian Stein discusses the physical and motor development of…

On BC1 RNA and the fragile X mental retardation protein

PubMed Central

Iacoangeli, Anna; Rozhdestvensky, Timofey S.; Dolzhanskaya, Natalia; Tournier, Barthélémy; Schütt, Janin; Brosius, Jürgen; Denman, Robert B.; Khandjian, Edouard W.; Kindler, Stefan; Tiedge, Henri

2008-01-01

The fragile X mental retardation protein (FMRP), the functional absence of which causes fragile X syndrome, is an RNA-binding protein that has been implicated in the regulation of local protein synthesis at the synapse. The mechanism of FMRP's interaction with its target mRNAs, however, has remained controversial. In one model, it has been proposed that BC1 RNA, a small non-protein-coding RNA that localizes to synaptodendritic domains, operates as a requisite adaptor by specifically binding to both FMRP and, via direct base-pairing, to FMRP target mRNAs. Other models posit that FMRP interacts with its target mRNAs directly, i.e., in a BC1-independent manner. Here five laboratories independently set out to test the BC1–FMRP model. We report that specific BC1–FMRP interactions could be documented neither in vitro nor in vivo. Interactions between BC1 RNA and FMRP target mRNAs were determined to be of a nonspecific nature. Significantly, the association of FMRP with bona fide target mRNAs was independent of the presence of BC1 RNA in vivo. The combined experimental evidence is discordant with a proposed scenario in which BC1 RNA acts as a bridge between FMRP and its target mRNAs and rather supports a model in which BC1 RNA and FMRP are translational repressors that operate independently. PMID:18184799

38 CFR 4.127 - Mental retardation and personality disorders.

Code of Federal Regulations, 2014 CFR

2014-07-01

... personality disorders. 4.127 Section 4.127 Pensions, Bonuses, and Veterans' Relief DEPARTMENT OF VETERANS... and personality disorders. Mental retardation and personality disorders are not diseases or injuries... superimposed upon mental retardation or a personality disorder may be service-connected. (Authority: 38 U.S.C...

38 CFR 4.127 - Mental retardation and personality disorders.

Code of Federal Regulations, 2012 CFR

2012-07-01

... personality disorders. 4.127 Section 4.127 Pensions, Bonuses, and Veterans' Relief DEPARTMENT OF VETERANS... and personality disorders. Mental retardation and personality disorders are not diseases or injuries... superimposed upon mental retardation or a personality disorder may be service-connected. (Authority: 38 U.S.C...

38 CFR 4.127 - Mental retardation and personality disorders.

Code of Federal Regulations, 2013 CFR

2013-07-01

... personality disorders. 4.127 Section 4.127 Pensions, Bonuses, and Veterans' Relief DEPARTMENT OF VETERANS... and personality disorders. Mental retardation and personality disorders are not diseases or injuries... superimposed upon mental retardation or a personality disorder may be service-connected. (Authority: 38 U.S.C...

38 CFR 4.127 - Mental retardation and personality disorders.

Code of Federal Regulations, 2011 CFR

2011-07-01

... personality disorders. 4.127 Section 4.127 Pensions, Bonuses, and Veterans' Relief DEPARTMENT OF VETERANS... and personality disorders. Mental retardation and personality disorders are not diseases or injuries... superimposed upon mental retardation or a personality disorder may be service-connected. (Authority: 38 U.S.C...

Subcortical laminar heterotopia and lissencephaly in two families: a single X linked dominant gene.

PubMed Central

Pinard, J M; Motte, J; Chiron, C; Brian, R; Andermann, E; Dulac, O

1994-01-01

Neuronal migration disorders can now be recognised by MRI. This paper reports two families in which the mothers had subcortical laminar heterotopia and four of their children had either similar heterotopia (two girls) or severe pachygyria or lissencephaly (two boys). Laminar heterotopia was more evident on MRI T2 weighted images. The patients had mild to severe epilepsy and mental retardation depending on the extent of cortical abnormalities. In these families, subcortical laminar heterotopia, pachygyria, and lissencephaly seem to share the same X linked or autosomal dominant gene. No chromosomal abnormalities, especially of chromosome 17, could be identified. For appropriate genetic counselling of the family of a child with lissencephaly or subcortical laminar heterotopia, MRI should be performed in parents or siblings with mental retardation or epilepsy. Images PMID:8057113

Research on mental retardation: an agenda for the future.

PubMed

Verdugo, M A

2000-06-01

This article summarizes the results of a study carried out on 12 scientific journals that deal with research on mental retardation. The purpose was to analyze the type of research currently being published. Data shows that, although most of research on mental retardation from 1991 to June, 1999 agrees with the multidimensional system proposed by the AAMR in 1992, research still tends to focus on a psychopathological model when considering people with mental retardation. We conclude by offering several suggestions on the need for a collaborative approach between researchers and professionals and the benefits of developing a supportive culture for research.

Psychometric properties of ADHD rating scales among children with mental retardation.

PubMed

Miller, Michael L; Fee, Virginia E; Jones, Christie J

2004-01-01

The validity of hyperactivity rating scales in children with mental retardation was evaluated. Forty-eight children with mental retardation were rated by parents, teachers and teaching assistants on rating scales measuring Attention Deficit/Hyperactivity Disorder (ADHD) as part of a related investigation. In addition, direct observations were conducted using the Abikoff Classroom Observation Code. The concurrent validity of each scale was examined. Scales completed by both teachers and teaching assistants were found to provide valid information for the assessment of ADHD in mentally retarded children. Results provided the best support for the ABC-C in the assessment of ADHD in mentally retarded children.

Mental Health and Mental Retardation Services in Nevada. Executive Summary.

ERIC Educational Resources Information Center

Kakalik, J. S.; And Others

Summarized are the findings and recommendations of a 2-year study of all major services and service delivery systems in Nevada for persons with mental health disorders, mentally retarded persons, and abusers of alcohol and other drugs. Considered are the following areas of basic service needs: prevention of the mentally handicapping conditions,…

Current Issues in Mental Retardation and Human Development: Selected Papers from the 1970 Staff Development Conferences of the President's Committee on Mental Retardation (Washington, D.C., 1971).

ERIC Educational Resources Information Center

Stedman, Donald J., Ed.

Six papers discuss some of the current issues in the field of mental retardation and human development. Epidemiology of mental retardation from a sociological and clinical point of view is analyzed by Jane R. Mercer, based on studies of mental retardation in the community in Pomona, California. The role of genetics and intra-uterine diagnosis of…

Nutrition and Mental Retardation. An Annotated Bibliography, 1964-1970.

ERIC Educational Resources Information Center

Springer, Ninfa Saturnino

This annotated bibliography is primarily organized for nutritionists. It presents selected articles published from 1964 to the present. All aspects of nutrition in mental retardation are covered excepting inborn errors of metabolism. Sections are included on: (1) nutrition, birthweight, and mental retardation; (2) nutrition, growth, and mental…

Mental retardation, congenital heart defect, cleft palate, short stature, and facial anomalies: A new X-linked multiple congenital anomalies/mental retardation syndrome: Clinical description and molecular studies

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hamel, B.C.J.; Mariman, E.C.M.; Beersum, S.E.C. van

1994-07-15

We report on two brothers and their two maternal uncles with severe mental retardation, congenital heart defect, cleft or highly arched palate, short stature and craniofacial anomalies consisting of microcephaly, abnormal ears, bulbous nose, broad nasal bridge, malar hypoplasia, and micro-gnathia. Three of the four patients died at an early age. The mother of the two brothers had an atrial septal defect. She is assumed to be manifesting carrier of a mutant gene, which is expressed in her two sons and two brothers. By multipoint linkage analysis it is found that the most likely location of the responsible gene ismore » the pericentromeric region Xp21.3-q21.3 with DMD and DXS3 as flanking markers. Maximum information is obtained with marker DXS453 (Z = 1.20 at {theta} = 0.0). 24 refs., 12 figs., 1 tab.« less

Sustained Attention of Adults with Mental Retardation.

ERIC Educational Resources Information Center

Tomporowski, Phillip D.; Allison, Pamela

1988-01-01

The sustained attention of 23 young adults with mild mental retardation and nonretarded subjects was assessed. Findings suggested that the sustained attention of the retarded differs from that of the nonretarded on those vigilance tasks that place demands on memory abilities. (Author/DB)

Theories on Criminality and Mental Retardation Project CAMIO, Volume 2.

ERIC Educational Resources Information Center

Haskins, Jimmy R.; Friel, Charles M.

This historical review of theories on criminality and mental retardation is part of Project CAMIO (Correctional Administration and the Mentally Incompetent Offender), a Texas study to determine the incidence of criminal incarceration of the mentally retarded (MR) and to identify laws, procedures, and practices which affect the prosecution and…

THE REASONING METHODS AND REASONING ABILITY IN NORMAL AND MENTALLY RETARDED GIRLS AND THE REASONING ABILITY OF NORMAL AND MENTALLY RETARDED BOYS AND GIRLS.

ERIC Educational Resources Information Center

CAPOBIANCO, RUDOLPH J.; AND OTHERS

A STUDY WAS MADE TO ESTABLISH AND ANALYZE THE METHODS OF SOLVING INDUCTIVE REASONING PROBLEMS BY MENTALLY RETARDED CHILDREN. THE MAJOR OBJECTIVES WERE--(1) TO EXPLORE AND DESCRIBE REASONING IN MENTALLY RETARDED CHILDREN, (2) TO COMPARE THEIR METHODS WITH THOSE UTILIZED BY NORMAL CHILDREN OF APPROXIMATELY THE SAME MENTAL AGE, (3) TO EXPLORE THE…

Detection and Quantification of the Fragile X Mental Retardation Protein 1 (FMRP).

PubMed

LaFauci, Giuseppe; Adayev, Tatyana; Kascsak, Richard; Brown, W Ted

2016-12-09

The final product of FMR1 gene transcription, Fragile X Mental Retardation Protein 1 (FMRP), is an RNA binding protein that acts as a repressor of translation. FMRP is expressed in several tissues and plays important roles in neurogenesis, synaptic plasticity, and ovarian functions and has been implicated in a number of neuropsychological disorders. The loss of FMRP causes Fragile X Syndrome (FXS). In most cases, FXS is due to large expansions of a CGG repeat in FMR1 -normally containing 6-54 repeats-to over 200 CGGs and identified as full mutation (FM). Hypermethylation of the repeat induces FMR1 silencing and lack of FMRP expression in FM male. Mosaic FM males express low levels of FMRP and present a less severe phenotype that inversely correlates with FMRP levels. Carriers of pre-mutations (55-200 CGG) show increased mRNA, and normal to reduced FMRP levels. Alternative splicing of FMR1 mRNA results in 24 FMRP predicted isoforms whose expression are tissues and developmentally regulated. Here, we summarize the approaches used by several laboratories including our own to (a) detect and estimate the amount of FMRP in different tissues, developmental stages and various pathologies; and (b) to accurately quantifying FMRP for a direct diagnosis of FXS in adults and newborns.

Cognitive Representation of Motion in Individuals with Mental Retardation.

ERIC Educational Resources Information Center

Conners, Frances A.; Wyatt, Beverly S.; Dulaney, Cynthia L.

1998-01-01

Fifteen adolescents with and 15 without mental retardation were compared on their tendency to show the representational momentum effect when viewing a stimulus array that implied motion. Participants with mental retardation showed the representational momentum effects as did the others, although the magnitude of the memory shift was smaller.…

Genetic analysis of a kindred with X-linked mental handicap and retinitis pigmentosa

DOE Office of Scientific and Technical Information (OSTI.GOV)

Aldred, M.A.; Dry, K.L.; Hardwick, L.J.

1994-11-01

A kindred is described in which X-linked nonspecific mental handicap segregates together with retinitis pigmentosa. Carrier females are mentally normal but may show signs of the X-linked retinitis pigmentosa carrier state and become symptomatic in their later years. Analysis of polymorphic DNA markers at nine loci on the short arm of the X chromosome shows that no crossing-over occurs between the disease and Xp11 markers DXS255, TIMP, DXS426, MAOA, and DXS228. The 90% confidence limits show that the locus is in the Xp21-q21 region. Haplotype analysis is consistent with the causal gene being located proximal to the Xp21 loci DXS538more » and 5{prime}-dystrophin on the short arm of the X chromosome. The posterior probability of linkage to the RP2 region of the X chromosome short arm (Xp11.4-p11.23) is .727, suggesting the possibility of a contiguous-gene-deletion syndrome. No cytogenetic abnormality has been identified. 33 refs., 2 figs., 2 tabs.« less

Perceptual-Motor Attributes of Mentally Retarded Youth.

ERIC Educational Resources Information Center

Cratty, Bryant J.

To evaluate six perceptual-motor attributes of trainable and educable mentally retarded children, a battery of tests was constructed which included body perception, gross agility, balance, locomotor ability, throwing, and tracking; 83 retarded subjects provided reliability data, and their scores, with those of 120 additional subjects, provided…

[Behavioral disorders and substance abuse in adolescents with mental retardation].

PubMed

Papachristou, Ec; Anagnostopoulos, Dk

2014-01-01

The percentage of people with mental retardation in the general population is estimated at about 2.3%, with adolescence (15-20 years) constituting the development period during which a peak in rates of mental retardation is observed. The increased prevalence of adolescence may be explained from the fact that the specified requirements of the school initially, and society later, inevitably lead to comparative evaluation of the teen with mental retardation in relation to peers, thus making mental retardation more apparent. Adolescents with mental retardation face a number of physical and psychological needs which are not often distinguishable and as a consequence undergo the deterioration of their already burdened quality of life. In particular, mental health problems occur 3 to 4 times more often in adolescents with mental retardation compared with adolescents of the general population. This review presents the most recent epidemiological findings regarding the correlation between behavioral disorders, substance use and the possible comorbidity in adolescents with intellectual disability, both at community level and residential care level. Epidemiological data indicate that behavioral disorders are among the most common types of psychopathology in mentally retarded adolescents with the severity and symptoms varying depending on the personal characteristics of each adolescent. Regarding substance use, the available data show that the rates of substance use (alcohol, smoking, illicit drugs) are lower in this specific population group but the differences over the last years tend to be eliminated. Finally, according to the few surveys that were examined referring to the comorbidity of behavioral disorders and substance use in adolescents with intellectual disability, the results were contradictory. Specifically, while behavioral disorders continued to be one of the most common types of psychopathology, the related substances disorders indicated lower rates compared to

Physical Trauma as an Etiological Agent in Mental Retardation.

ERIC Educational Resources Information Center

Angle, Carol R., Ed.; Bering, Edgar A., Jr., Ed.

The conference on Physical Trauma as a Cause of Mental Retardation dealt with two major areas of etiological concern - postnatal and perinatal trauma. Following two introductory statements on the problem of and issues related to mental retardation (MR) after early trauma to the brain, five papers on the epidemiology of head trauma cover…

Mutations in CUL4B, which encodes a ubiquitin E3 ligase subunit, cause an X-linked mental retardation syndrome associated with aggressive outbursts, seizures, relative macrocephaly, central obesity, hypogonadism, pes cavus, and tremor.

PubMed

Tarpey, Patrick S; Raymond, F Lucy; O'Meara, Sarah; Edkins, Sarah; Teague, Jon; Butler, Adam; Dicks, Ed; Stevens, Claire; Tofts, Calli; Avis, Tim; Barthorpe, Syd; Buck, Gemma; Cole, Jennifer; Gray, Kristian; Halliday, Kelly; Harrison, Rachel; Hills, Katy; Jenkinson, Andrew; Jones, David; Menzies, Andrew; Mironenko, Tatiana; Perry, Janet; Raine, Keiran; Richardson, David; Shepherd, Rebecca; Small, Alexandra; Varian, Jennifer; West, Sofie; Widaa, Sara; Mallya, Uma; Moon, Jenny; Luo, Ying; Holder, Susan; Smithson, Sarah F; Hurst, Jane A; Clayton-Smith, Jill; Kerr, Bronwyn; Boyle, Jackie; Shaw, Marie; Vandeleur, Lucianne; Rodriguez, Jayson; Slaugh, Rachel; Easton, Douglas F; Wooster, Richard; Bobrow, Martin; Srivastava, Anand K; Stevenson, Roger E; Schwartz, Charles E; Turner, Gillian; Gecz, Jozef; Futreal, P Andrew; Stratton, Michael R; Partington, Michael

2007-02-01

We have identified three truncating, two splice-site, and three missense variants at conserved amino acids in the CUL4B gene on Xq24 in 8 of 250 families with X-linked mental retardation (XLMR). During affected subjects' adolescence, a syndrome emerged with delayed puberty, hypogonadism, relative macrocephaly, moderate short stature, central obesity, unprovoked aggressive outbursts, fine intention tremor, pes cavus, and abnormalities of the toes. This syndrome was first described by Cazebas et al., in a family that was included in our study and that carried a CUL4B missense variant. CUL4B is a ubiquitin E3 ligase subunit implicated in the regulation of several biological processes, and CUL4B is the first XLMR gene that encodes an E3 ubiquitin ligase. The relatively high frequency of CUL4B mutations in this series indicates that it is one of the most commonly mutated genes underlying XLMR and suggests that its introduction into clinical diagnostics should be a high priority.

Hansels and Gretels: Studies of Children in Institutions for the Mentally Retarded.

ERIC Educational Resources Information Center

Braginsky, Dorothea D.; Braginsky, Benjamin M.

Data from research with mildly mentally retarded children conducted in state training schools contradicts prevalent conceptions and theories of mental retardation and supports a new way of conceptualizing retardation, in which retardates are not seen as qualitatively different from others nor as defective or less than human organisms. Investigated…

Presidential Address 1985: Inalienable Rights of Persons with Mental Retardation.

ERIC Educational Resources Information Center

Walker, B. R.

1985-01-01

The address reviews the establishment of rights for mentally retarded persons, cautions about the need to safeguard those rights, discusses the central role of individual program plans, and cites the importance of a continuum of services for mentally retarded persons. (CL)

Weight Survey on Adult Persons with Mental Retardation Living in the Community

ERIC Educational Resources Information Center

Hove, Oddbjorn

2004-01-01

Prevalence of underweight and obesity were investigated in 282 mentally retarded persons living on the West Coast of Norway. Data collected in this survey suggest that people with severe mental retardation were more likely to be underweight and people with mild mental retardation were more likely to be obese. Compared to persons of average…

DIA1R is an X-linked gene related to Deleted In Autism-1.

PubMed

Aziz, Azhari; Harrop, Sean P; Bishop, Naomi E

2011-01-17

Autism spectrum disorders (ASDS) are frequently occurring disorders diagnosed by deficits in three core functional areas: social skills, communication, and behaviours and/or interests. Mental retardation frequently accompanies the most severe forms of ASDs, while overall ASDs are more commonly diagnosed in males. Most ASDs have a genetic origin and one gene recently implicated in the etiology of autism is the Deleted-In-Autism-1 (DIA1) gene. Using a bioinformatics-based approach, we have identified a human gene closely related to DIA1, we term DIA1R (DIA1-Related). While DIA1 is autosomal (chromosome 3, position 3q24), DIA1R localizes to the X chromosome at position Xp11.3 and is known to escape X-inactivation. The gene products are of similar size, with DIA1 encoding 430, and DIA1R 433, residues. At the amino acid level, DIA1 and DIA1R are 62% similar overall (28% identical), and both encode signal peptides for targeting to the secretory pathway. Both genes are ubiquitously expressed, including in fetal and adult brain tissue. Examination of published literature revealed point mutations in DIA1R are associated with X-linked mental retardation (XLMR) and DIA1R deletion is associated with syndromes with ASD-like traits and/or XLMR. Together, these results support a model where the DIA1 and DIA1R gene products regulate molecular traffic through the cellular secretory pathway or affect the function of secreted factors, and functional deficits cause disorders with ASD-like symptoms and/or mental retardation.

The Texas Plan to Combat Mental Retardation.

ERIC Educational Resources Information Center

Governor's Advisory Committee on Mental Retardation Planning, Austin, TX.

The Texas state plan of action against mental retardation is presented. Aspects considered include the evolution of the plan, the role of the health services, medical aspects of retardation, education and training, vocational rehabilitation and employment, and social welfare. Also surveyed are the following: residential and day care, manpower,…

Identification of Important Community Living Skills for Adults with Mental Retardation.

ERIC Educational Resources Information Center

Lovett, David L.; Harris, Mary B.

1987-01-01

Attempted to identify skills important for successful community living of adults with mental retardation. Individuals (N=73) who had significant contact with adults with mental retardation completed questionnaire on academic, leisure, personal, social, and vocational skills frequently taught to persons with mental handicaps. Subjects considered…

Sterilization of the Mentally Ill and the Mentally Retarded.

ERIC Educational Resources Information Center

National Association of State Mental Health Program Directors, Washington, DC.

Reported were the results of a survey on the sterilization of the mentally ill and the mentally retarded. Thirty-three states responded to the survey. It was found that 17 states have a sterilization statute, but the existence of the statute was explained not to mean that the procedure was used. Sixteen states responded that they did not have a…

Orthopaedic Problems of the Mentally Retarded

ERIC Educational Resources Information Center

McSweeney, Anthony

1972-01-01

Problems encountered by orthopedic surgeons treating the mentally retarded are identified, and cooperation among pediatricians, psychiatrists, psychologists, social workers, physiotherapists, occupational therapists, and orthopedic surgeons is recommended. (GW)

Problems of Psychology of Mentally Retarded Children.

ERIC Educational Resources Information Center

Academy of Pedagogical Sciences of the USSR, Moscow. Inst. of Defectology.

Presented are 18 papers on problems in the psychology of mentally retarded children. Seven of the papers are in English, two in French, and nine in Russian. The English papers are concerned with the following topics: peculiarities of psychic functions in oligophrenic (retarded) children with pronounced underdevelopment of frontal lobes of cerebral…

Newborn Screening To Prevent Mental Retardation. The Arc Q & A.

ERIC Educational Resources Information Center

Arc, Arlington, TX.

This information fact sheet on screening newborns to prevent mental retardation defines newborn screening and outlines how screening is performed. It discusses the six most common disorders resulting in mental retardation for which states most commonly screen. These include phenylketonuria, congenital hypothyroidism, galactosemia, maple syrup…

The self-esteem and anxiety of children with and without mentally retarded siblings.

PubMed

Saban, Fatma; Arıkan, Duygu

2013-11-01

The study was carried out with the aim of determining the factors affecting and to evaluate anxiety situations and self-esteem of children with and without mentally retarded siblings. The sampling included 227 healthy children: 108 of them have mental retarded sibling and 119 of them do not have mental retarded sibling. The context of this study consisted of 15-18 year of age healthy children with mentally retarded siblings and 15-18 year of aged healthy children having at least one sibling between the dates February 15(st) and June 26(st) 2010. Personal Information Form, Rosenberg Self-Esteem Scale and Trait Anxiety Scale were used. It was found out that trait anxiety of 17-18 aged of children with mental retarded sibling (47.04 ± 7.3) was higher than that of the children without mental retarded siblings (44.05 ± 11.23) (P < 0.05). It was observed that self-esteem of children with mentally retarded sibling was not affected from the handicap of their siblings (P > 0.05). Trait anxiety score averages of children with mentally retarded sibling and experience some difficulties due to his or her siblings's handicap (47.00 ± 7.76) were found higher than those of those of the children without any problem with the environment (42.61 ± 7.48) (P < 0.05). Although the average score of trait anxiety and self-esteem in both groups were not significant different, score of trait anxiety for children with mentally disabled siblings was higher in comparison. It was concluded that anxiety of children with and without mentally retarded siblings increased as self-esteem of these children decreased.

Validity of the Schizophrenia Diagnosis of the Psychopathology Instrument for Mentally Retarded Adults (PIMRA): A Comparison of Schizophrenic Patients with and without Mental Retardation.

ERIC Educational Resources Information Center

Linaker, Olav M.; Helle, Jon

1994-01-01

This study found that the schizophrenia subscale of the Psychopathology Instrument for Mentally Retarded Adults was a valid quantitative measure of schizophrenia if one item was removed from the scale. Comparison with a nonretarded population indicated that mentally retarded patients had less delusions and more incoherence and flat affect. They…

Mental retardation in a boy with an interstitial deletion at Xp22.3 involving STS, KAL1, and OA1: Implication for the MRX locus

DOE Office of Scientific and Technical Information (OSTI.GOV)

Muroya, Koji; Ogata, Tsutomu; Natsuo, Nobutake

Although genotype-phenotype correlations in male patients with various types of nullisomy for Xp22.3 have assigned a locus for X-linked mental retardation (MRX) to an approximately 3-Mb region between DXS31 and STS, the precise location has not been determined. In this paper, we describe a 14 7/12 year old Japanese boy with mental retardation and an interstitial deletion at Xp22.3 involving STS, KAL1, and OA1, and compare the deletion map with that of previously reported three familial male patients with low-normal intelligence and a similar interstitial deletion at Xp22.3. The results suggest that the MRX gene is further localized to themore » roughly 1.5-Mb region between DXS1060 and DXS1139. 31 refs., 4 figs.« less

Behavioral Treatment of Aggression in the Mentally Retarded: A Review.

ERIC Educational Resources Information Center

Feldstein, Jerome H.

The paper reviews 34 behavioral treatment studies (1967-1983) examining reduction of aggressive behavior in mentally retarded people. Research reviewed was limited to treatment of physically aggressive responses such as hits, kicks, bites, chokes, scratches, and throwing objects by persons designated as mentally retarded. Among results reported…

Detection and Quantification of the Fragile X Mental Retardation Protein 1 (FMRP)

PubMed Central

LaFauci, Giuseppe; Adayev, Tatyana; Kascsak, Richard; Brown, W. Ted

2016-01-01

The final product of FMR1 gene transcription, Fragile X Mental Retardation Protein 1 (FMRP), is an RNA binding protein that acts as a repressor of translation. FMRP is expressed in several tissues and plays important roles in neurogenesis, synaptic plasticity, and ovarian functions and has been implicated in a number of neuropsychological disorders. The loss of FMRP causes Fragile X Syndrome (FXS). In most cases, FXS is due to large expansions of a CGG repeat in FMR1—normally containing 6–54 repeats—to over 200 CGGs and identified as full mutation (FM). Hypermethylation of the repeat induces FMR1 silencing and lack of FMRP expression in FM male. Mosaic FM males express low levels of FMRP and present a less severe phenotype that inversely correlates with FMRP levels. Carriers of pre-mutations (55–200 CGG) show increased mRNA, and normal to reduced FMRP levels. Alternative splicing of FMR1 mRNA results in 24 FMRP predicted isoforms whose expression are tissues and developmentally regulated. Here, we summarize the approaches used by several laboratories including our own to (a) detect and estimate the amount of FMRP in different tissues, developmental stages and various pathologies; and (b) to accurately quantifying FMRP for a direct diagnosis of FXS in adults and newborns. PMID:27941672

Prospects for Prevention and Amelioration of Mental Retardation: A Guest Editorial

ERIC Educational Resources Information Center

Clarke, A. D. B.; Clarke, Ann M.

1977-01-01

In a guest editorial, the view of the President's Committee on Mental Retardation (1972), that the incidence of mental retardation can be decreased by 50 percent by the year 2000, is critically examined. (BB)

Mental retardation in Nance-Horan syndrome: clinical and neuropsychological assessment in four families.

PubMed

Toutain, A; Ayrault, A D; Moraine, C

1997-08-22

Nance-Horan syndrome (NHS) is a rare X-linked condition comprising congenital cataract with microcornea, distinctive dental, and evocative facial anomalies. Intellectual handicap was mentioned in seven published NHS patients. We performed a clinical study focused on psychomotor development, intellectual abilities, and behavior in 13 affected males in four NHS families, and present the results of a neuropsychological evaluation in 7 of them. Our study confirms that mental retardation (MR) can be a major component of the NHS. Combining our data with those from the literature leads to a frequency of MR in NHS of around 30%. In most cases, MR is mild or moderate (80%) and not associated with motor delay. Conversely, a profound mental handicap associated with autistic traits may be observed. MR has intra- and inter-familial variability but does not appear to be expressed in carriers. Awareness of MR in NHS may be of importance in the management of the patients, especially in terms of education. Cloning and characterization of the gene and analysis of mutations will be an important step towards understanding the molecular basis of mental deficiency in NHS, and in delineation from the other XLMR conditions at Xp22.

Mental Retardation: Diagnosis and Treatment.

ERIC Educational Resources Information Center

Poser, Charles M., Ed.

A collection of writings by 17 authors, the text includes the following discussions: general principles of diagnosis and management of mental retardation, neurologic evaluation of the infant and child, psychological evaluation, educational information, and treatment of pseudoretardation, communicative disorders, and metabolic and endocrine causes.…

Type and Frequency of Social Interaction among Workers with and without Mental Retardation.

ERIC Educational Resources Information Center

Ferguson, Brad; And Others

1993-01-01

The social/verbal interactions of six employees with moderate or severe mental retardation and six workers without mental retardation were observed in restaurant settings. Among findings was that interactions between workers with mental retardation and the job coach correlated negatively with the workers' initiation of interactions with co-workers…

Fragile X Mental Retardation Protein Is Required to Maintain Visual Conditioning-Induced Behavioral Plasticity by Limiting Local Protein Synthesis

PubMed Central

Liu, Han-Hsuan

2016-01-01

Fragile X mental retardation protein (FMRP) is thought to regulate neuronal plasticity by limiting dendritic protein synthesis, but direct demonstration of a requirement for FMRP control of local protein synthesis during behavioral plasticity is lacking. Here we tested whether FMRP knockdown in Xenopus optic tectum affects local protein synthesis in vivo and whether FMRP knockdown affects protein synthesis-dependent visual avoidance behavioral plasticity. We tagged newly synthesized proteins by incorporation of the noncanonical amino acid azidohomoalanine and visualized them with fluorescent noncanonical amino acid tagging (FUNCAT). Visual conditioning and FMRP knockdown produce similar increases in FUNCAT in tectal neuropil. Induction of visual conditioning-dependent behavioral plasticity occurs normally in FMRP knockdown animals, but plasticity degrades over 24 h. These results indicate that FMRP affects visual conditioning-induced local protein synthesis and is required to maintain the visual conditioning-induced behavioral plasticity. SIGNIFICANCE STATEMENT Fragile X syndrome (FXS) is the most common form of inherited intellectual disability. Exaggerated dendritic protein synthesis resulting from loss of fragile X mental retardation protein (FMRP) is thought to underlie cognitive deficits in FXS, but no direct evidence has demonstrated that FMRP-regulated dendritic protein synthesis affects behavioral plasticity in intact animals. Xenopus tadpoles exhibit a visual avoidance behavior that improves with visual conditioning in a protein synthesis-dependent manner. We showed that FMRP knockdown and visual conditioning dramatically increase protein synthesis in neuronal processes. Furthermore, induction of visual conditioning-dependent behavioral plasticity occurs normally after FMRP knockdown, but performance rapidly deteriorated in the absence of FMRP. These studies show that FMRP negatively regulates local protein synthesis and is required to maintain visual

Decision Making in Leisure. Empowerment for People with Mental Retardation.

ERIC Educational Resources Information Center

Bullock, Charles C.; Mahon, Michael J.

1992-01-01

People with mental retardation have been overlooked in recreation/leisure and decision making, which are integral to full community participation. They must be provided with leisure education and decision-making skills. The article describes the Decision Making in Leisure model, explaining its use with individuals with mental retardation. (SM)

Competence and Adjustment of Siblings of Children with Mental Retardation.

ERIC Educational Resources Information Center

Hannah, Mary E.; Midlarsky, Elizabeth

1999-01-01

This study compared the adjustment and competence of 100 children and adolescents, half of whom were siblings of individuals with mental retardation. Although there were no overall differences for internalizing disorders, externalizing disorders, self-esteem, and competence, boys with a mentally retarded sibling had more difficulty in school…

The self-esteem and anxiety of children with and without mentally retarded siblings

PubMed Central

Saban, Fatma; Arıkan, Duygu

2013-01-01

Background: The study was carried out with the aim of determining the factors affecting and to evaluate anxiety situations and self-esteem of children with and without mentally retarded siblings. Materials and Methods: The sampling included 227 healthy children: 108 of them have mental retarded sibling and 119 of them do not have mental retarded sibling. The context of this study consisted of 15-18 year of age healthy children with mentally retarded siblings and 15-18 year of aged healthy children having at least one sibling between the dates February 15st and June 26st 2010. Personal Information Form, Rosenberg Self-Esteem Scale and Trait Anxiety Scale were used. Results: It was found out that trait anxiety of 17-18 aged of children with mental retarded sibling (47.04 ± 7.3) was higher than that of the children without mental retarded siblings (44.05 ± 11.23) (P < 0.05). It was observed that self-esteem of children with mentally retarded sibling was not affected from the handicap of their siblings (P > 0.05). Trait anxiety score averages of children with mentally retarded sibling and experience some difficulties due to his or her siblings's handicap (47.00 ± 7.76) were found higher than those of those of the children without any problem with the environment (42.61 ± 7.48) (P < 0.05). Conclusion: Although the average score of trait anxiety and self-esteem in both groups were not significant different, score of trait anxiety for children with mentally disabled siblings was higher in comparison. It was concluded that anxiety of children with and without mentally retarded siblings increased as self-esteem of these children decreased. PMID:24523782

Editorial: X-chromosome-linked Kallmann's syndrome: Pathology at the molecular level

DOE Office of Scientific and Technical Information (OSTI.GOV)

Prager, D.; Braunstein, G.D.

Kallmann's syndrome or olfactogenital dysplasia refers to a disorder characterized by hypogonadotropic hypogonadism and anosmia or hyposmia which can occur sporadically or in a familial setting. Originally described in 1856, the first familial cases were reported by Kallmann et al., in 1944. Based on segregation analysis of multiple families, three modes of transmission have been documented: X-linked, autosomal dominant with variable penetrance, and autosomal recessive. Kallmann's syndrome occurs in less than 1 in 10,000 male births, with a 5-fold excess of affected males to females, suggesting that the X-linked form is the most frequent. By genetic linkage analysis the X-linkedmore » form of Kallmann's syndrome was localized to Xp22.3. This was confirmed by the description of patients with contiguous gene syndromes due to deletions of various portions of the distal short arm of the X-chromosome. Such patients present with complex phenotypes characterized by a combination of Kallmann's syndrome with X-linked icthyosis due to steroid sulfatase deficiency, chondrodysplasia punctata, short stature, and mental retardation. DNA analysis has identified and mapped the genes responsible for these disorders. 10 refs., 1 fig., 1 tab.« less

A Proposal for the Unification of Vermont's Mental Retardation Service System.

ERIC Educational Resources Information Center

Vermont State Dept. of Mental Health, Montpelier. Div. of Mental Retardation.

This planning document proposes that services to Vermonters with mental retardation be unified around the community mental retardation service system, in order to provide community placements for residents of the Brandon Training School (BTS) who are under court order for discharge between 1989 and 1993. The Department of Mental Health plans to…

Elevated progranulin contributes to synaptic and learning deficit due to loss of fragile X mental retardation protein.

PubMed

Zhang, Kun; Li, Yu-Jiao; Guo, Yanyan; Zheng, Kai-Yin; Yang, Qi; Yang, Le; Wang, Xin-Shang; Song, Qian; Chen, Tao; Zhuo, Min; Zhao, Ming-Gao

2017-12-01

Fragile X syndrome is an inheritable form of intellectual disability caused by loss of fragile X mental retardation protein (FMRP, encoded by the FMR1 gene). Absence of FMRP caused overexpression of progranulin (PGRN, encoded by GRN), a putative tumour necrosis factor receptor ligand. In the present study, we found that progranulin mRNA and protein were upregulated in the medial prefrontal cortex of Fmr1 knock-out mice. In Fmr1 knock-out mice, elevated progranulin caused insufficient dendritic spine pruning and late-phase long-term potentiation in the medial prefrontal cortex of Fmr1 knock-out mice. Partial progranulin knock-down restored spine morphology and reversed behavioural deficits, including impaired fear memory, hyperactivity, and motor inflexibility in Fmr1 knock-out mice. Progranulin increased levels of phosphorylated glutamate ionotropic receptor GluA1 and nuclear factor kappa B in cultured wild-type neurons. Tumour necrosis factor receptor 2 antibody perfusion blocked the effects of progranulin on GluA1 phosphorylation; this result indicates that tumour necrosis factor receptor 2 is required for progranulin-mediated GluA1 phosphorylation and late-phase long-term potentiation expression. However, high basal level of progranulin in Fmr1 knock-out mice prevented further facilitation of synaptic plasticity by exogenous progranulin. Partial downregulation of progranulin or tumour necrosis factor receptor 2/nuclear factor kappa B signalling restored synaptic plasticity and memory deficits in Fmr1 knock-out mice. These findings suggest that elevated PGRN is linked to cognitive deficits of fragile X syndrome, and the progranulin/tumour necrosis factor receptor 2 signalling pathway may be a putative therapeutic target for improving cognitive deficits in fragile X syndrome. © The Author (2017). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Psychopharmacology and Mental Retardation: A 10 Year Review (1990- 1999).

ERIC Educational Resources Information Center

Matson, Johnny L.; Bamburg, Jay W.; Mayville, Erik A.; Pinkston, Jim; Bielecki, Joanne; Kuhn, David; Smalls, Yemonja; Logan, James R.

2000-01-01

Review of the literature on psychopharmacology and mental retardation from 1990-1999 found most studies had major methodological flaws. Also, most drug administrations were not based in science, were not evaluated appropriately, and generally did not follow best practices for treatment of persons with mental retardation. A table lists the studies…

HANDBOOK OF MENTAL RETARDATION SYNDROMES.

ERIC Educational Resources Information Center

CARTER, CHARLES H.

THE CLINICAL SYNDROMES WHICH CONTRIBUTE TO THE PRODUCTION OF MENTAL RETARDATION ARE DESCRIBED BY SIGNS, SYMPTOMS, AND ETIOLOGY. SYNDROMES TREATED ARE (1) PRENATAL AND POSTNATAL INFECTIONS, (2) PRENATAL INTOXICATION AND ALLERGIC REACTIONS, (3) PRENATAL TRAUMA, PHYSICAL AGENTS, OR INTOXICATION, (4) BIRTH INJURIES, (5) POSTNATAL POISONS AND ALLERGIC…

PUBLIC PROVISION FOR THE MENTALLY RETARDED IN THE UNITED STATES.

ERIC Educational Resources Information Center

BEST, HARRY

WRITTEN FROM THE STANDPOINT OF THE SOCIOLOGIST OR SOCIAL SCIENTIST, THIS BOOK REPORTS DATA OBTAINED FROM STATISTICAL RESEARCH ON MENTAL RETARDATES. ITS CHIEF PURPOSE IS THE SCIENTIFIC STUDY OF THE MENTALLY RETARDED AND PROVISIONS MADE FOR THEM IN THE UNITED STATES. DISCUSSION OF THE GENERAL CONDITION COVERS DEFINITION AND CLASSIFICATION, ETIOLOGY,…

Sterilization of Persons with Mental Retardation.

ERIC Educational Resources Information Center

Elkins, Thomas E.; Andersen, H. Frank

1992-01-01

This article examines the historical, legal, and ethical concerns regarding sterilization for persons with mental retardation and offers guidelines to help counsel individuals with disabilities or their families regarding decision making about sterilization. (DB)

Temporal Requirements of the Fragile X Mental Retardation Protein in Modulating Circadian Clock Circuit Synaptic Architecture

PubMed Central

Gatto, Cheryl L.; Broadie, Kendal

2009-01-01

Loss of fragile X mental retardation 1 (FMR1) gene function is the most common cause of inherited mental retardation and autism spectrum disorders, characterized by attention disorder, hyperactivity and disruption of circadian activity cycles. Pursuit of effective intervention strategies requires determining when the FMR1 product (FMRP) is required in the regulation of neuronal circuitry controlling these behaviors. In the well-characterized Drosophila disease model, loss of the highly conserved dFMRP causes circadian arrhythmicity and conspicuous abnormalities in the circadian clock circuitry. Here, a novel Sholl Analysis was used to quantify over-elaborated synaptic architecture in dfmr1-null small ventrolateral neurons (sLNvs), a key subset of clock neurons. The transgenic Gene-Switch system was employed to drive conditional neuronal dFMRP expression in the dfmr1-null mutant background in order to dissect temporal requirements within the clock circuit. Introduction of dFMRP during early brain development, including the stages of neurogenesis, neuronal fate specification and early pathfinding, provided no rescue of dfmr1 mutant phenotypes. Similarly, restoring normal dFMRP expression in the adult failed to restore circadian circuit architecture. In sharp contrast, supplying dFMRP during a transient window of very late brain development, wherein synaptogenesis and substantial subsequent synaptic reorganization (e.g. use-dependent pruning) occur, provided strong morphological rescue to reestablish normal sLNvs synaptic arbors. We conclude that dFMRP plays a developmentally restricted role in sculpting synaptic architecture in these neurons that cannot be compensated for by later reintroduction of the protein at maturity. PMID:19738924

Mental Retardation. Selected Articles from the Rehabilitation Record.

ERIC Educational Resources Information Center

Rehabilitation Services Administration (DHEW), Washington, DC.

Presented are six articles on residential living, vocational education, employment recreation, deinstitutionalization, and workshop experience of mentally retarded children and adults. K. Grunewald discusses the planning of housing for five- to eight person groups of retarded children and adults in varying kinds of residential facilities in Sweden…

Genetic Counseling in Mental Retardation.

ERIC Educational Resources Information Center

Bowen, Peter

The task of the genetic counselor who identifies genetic causes of mental retardation and assists families to understand risk of recurrence is described. Considered are chromosomal genetic disorders such as Down's syndrome, inherited disorders such as Tay-Sachs disease, identification by testing the amniotic fluid cells (amniocentresis) in time…

Nonretarded and Mentally Retarded Children's Control over Syntax Structures.

ERIC Educational Resources Information Center

McLeavey, Breda C.; And Others

1982-01-01

Ten nonretarded preschoolers and 10 mildly mentally retarded children (mean age 8 years) were compared for control over various syntactic structures. The retarded children evidenced difficulties similar to those of younger nonretarded children. (Author/SB)

Iconic Memory Deficit of Mildly Mentally Retarded Individuals.

ERIC Educational Resources Information Center

Hornstein, Henry A.; Mosley, James L.

1987-01-01

Ten mildly retarded young adult males and nonretarded subjects matched for chronological age or mental age were required to recognize both verbal and nonverbal stimuli presented tachistoscopically. Results of a backward visual masking paradigm varying stimulus onset asynchrony (SOA) indicated the retarded subjects performed poorer at the longest…

DIRECTORY OF RESIDENTIAL CAMPS SERVING THE MENTALLY RETARDED.

ERIC Educational Resources Information Center

National Association for Retarded Children, New York, NY.

PREPARED BY THE NATIONAL ASSOCIATION FOR RETARDED CHILDREN FROM A QUESTIONNAIRE SENT TO STATE AND LOCAL ORGANIZATIONS, THE DIRECTORY (1967) LISTS RESIDENT CAMPS SERVING THE MENTALLY RETARDED THROUGHOUT THE UNITED STATES. CAMPS ARE ENTERED ALPHABETICALLY ACCORDING TO GEOGRAPHIC LOCATION WITHIN EACH STATE. TYPES OF CHILDREN SERVED INCLUDE MENTALLY…

Detection of Malingered Mental Retardation

ERIC Educational Resources Information Center

Shandera, Anne L.; Berry, David T. R.; Clark, Jessica A.; Schipper, Lindsey J.; Graue, Lili O.; Harp, Jordan P.

2010-01-01

In a cross-validation of results from L. O. Graue et al. (2007), standard psychological assessment instruments, as well as tests of neurocognitive and psychiatric feigning, were administered under standard instructions to 24 participants diagnosed with mild mental retardation (MR) and 10 demographically matched community volunteers (CVH). A 2nd…

Primacy Effects in Short-Term Memory with the Mentally Retarded.

ERIC Educational Resources Information Center

Detterman, Douglas K.

This paper reports on two experiments conducted in an attempt to extend findings by Ellis which suggest a rehearsal deficit in mentally retarded subjects. In experiment one, mentally retarded subjects saw nine stimuli in a serial position probe task for either two, four, or six seconds each. Performances for the two and four second-per-item rates…

Everyday technology use among people with mental retardation: relevance, perceived difficulty, and influencing factors.

PubMed

Hällgren, Monica; Nygård, Louise; Kottorp, Anders

2014-05-01

While the development and possibilities of technology today are commonly regarded to be unlimited, knowledge regarding the technological needs of people with mental retardation is fairly limited. The aim of this study was to enhance knowledge of perceived relevance and difficulty in using everyday technology (ET) such as stoves, cell phones, and elevators in adults with mental retardation. 120 participants with different levels of mental retardation were interviewed with the Everyday Technology Use Questionnaire (ETUQ) about their use of such technologies in their everyday life. Analyses of variance, post hoc tests, and regression analyses were used to explore the data. Participants with moderate and severe mental retardation differed in mean perceived difficulty from those with mild mental retardation, suggesting that increased perceived difficulty in ET use is related to the level of mental retardation. Differences between groups were also found in the proportion of items that were relevant for each person. The variables Level of Mental Retardation, Additional Disabilities, and Proportional Relevance of ET Items could together predict 67.2% of the variation in perceived difficulty in technology use. The findings also indicate that age, housing, gender, and geographical district do not covariate with perceived difficulty in ET use.

Localization to Xq22 and clinical update of a family with X-linked recessive mental retardation with progression sensorineural deafness, progressive tapeto-retinal degeneration and dystonia

DOE Office of Scientific and Technical Information (OSTI.GOV)

Tranebjaerg, L.; Schwartz, C.; Huggins, K.

1994-07-15

In a reinvestigation of a six-generation Norwegian family, originally reported with non-syndromic X-linked recessive deafness by Mohr and Mageroy, we have demonstrated several syndromic manifestations. The 10 clinically characterized affected males range in age from 14-61 years, and show progressive mental deterioration and visual disability. Ophthalmological and electrophysiological studies showed myopia, decreased visual acuity, combined cone-rod dystrophy as well as central areolar dystrophy by means of ERG. Brain CT-scans showed cortical and central atrophy without predilection to specific areas. Linkage analysis, using X-chromosomal RFLPs and CA-repeats, yielded a maximum LOD score of 4.37 with linkage to DXS17. DXS17 is localizedmore » to Xq22. One recombinant with COL4A5 (deficient in Alport syndrome) was observed. Results from the studies of this family will be important in reclassification of non-syndromic X-linked deafness since the family now represents syndromic deafness and XLMR with a specific phenotype.« less

The Myth of Mental Retardation: Paradigm Shifts, Disaggregation, and Developmental Disabilities.

ERIC Educational Resources Information Center

Smith, J. David

2002-01-01

This commentary discusses whether the American Association on Mental Retardation should change its name. It argues that the term "mental retardation" should become an historical artifact of the evolution of our thinking about children and adults with developmental disabilities. The plurality of the term "developmental…

Fragile X Mental Retardation Protein Is Required to Maintain Visual Conditioning-Induced Behavioral Plasticity by Limiting Local Protein Synthesis.

PubMed

Liu, Han-Hsuan; Cline, Hollis T

2016-07-06

Fragile X mental retardation protein (FMRP) is thought to regulate neuronal plasticity by limiting dendritic protein synthesis, but direct demonstration of a requirement for FMRP control of local protein synthesis during behavioral plasticity is lacking. Here we tested whether FMRP knockdown in Xenopus optic tectum affects local protein synthesis in vivo and whether FMRP knockdown affects protein synthesis-dependent visual avoidance behavioral plasticity. We tagged newly synthesized proteins by incorporation of the noncanonical amino acid azidohomoalanine and visualized them with fluorescent noncanonical amino acid tagging (FUNCAT). Visual conditioning and FMRP knockdown produce similar increases in FUNCAT in tectal neuropil. Induction of visual conditioning-dependent behavioral plasticity occurs normally in FMRP knockdown animals, but plasticity degrades over 24 h. These results indicate that FMRP affects visual conditioning-induced local protein synthesis and is required to maintain the visual conditioning-induced behavioral plasticity. Fragile X syndrome (FXS) is the most common form of inherited intellectual disability. Exaggerated dendritic protein synthesis resulting from loss of fragile X mental retardation protein (FMRP) is thought to underlie cognitive deficits in FXS, but no direct evidence has demonstrated that FMRP-regulated dendritic protein synthesis affects behavioral plasticity in intact animals. Xenopus tadpoles exhibit a visual avoidance behavior that improves with visual conditioning in a protein synthesis-dependent manner. We showed that FMRP knockdown and visual conditioning dramatically increase protein synthesis in neuronal processes. Furthermore, induction of visual conditioning-dependent behavioral plasticity occurs normally after FMRP knockdown, but performance rapidly deteriorated in the absence of FMRP. These studies show that FMRP negatively regulates local protein synthesis and is required to maintain visual conditioning

Transportation and the Mentally Retarded.

ERIC Educational Resources Information Center

President's Committee on Mental Retardation, Washington, DC.

Reported were the results of a contract that involved identification, description, and categorization of the nature of transportation problems for the mentally retarded by means of analysis of existing studies, two surveys, and an inventory of specialized programs and systems operating in the United States. One major problem was found to be…

Prefrontal Cortex Dysfunction in Fragile X Mice Depends on the Continued Absence of Fragile X Mental Retardation Protein in the Adult Brain.

PubMed

Siegel, Jennifer J; Chitwood, Raymond A; Ding, James M; Payne, Clayton; Taylor, William; Gray, Richard; Zemelman, Boris V; Johnston, Daniel

2017-08-02

Fragile X Syndrome (FX) is generally considered a developmental disorder, arising from a mutation that disrupts the transcription of Fragile X Mental Retardation Protein (FMRP). However, FMRP regulates the transcription of other proteins and participates in an unknown number of protein-protein interactions throughout life. In addition to known developmental issues, it is thus likely that some dysfunction is also due to the ongoing absence of FMRP. Dissociating dysfunction due to developmental dysregulation from dysfunction due to the continued absence of FMRP is necessary to understand the different roles of FMRP and to treat patients effectively throughout life. We show here that FX model mice display substantial deficits in a PFC-dependent task. We then use conditional knock-out mice to eliminate FMRP only in the PFC alone of adult mice. We observe an increase in the proportion of nonlearners and a delay in the onset of learning in both FX and conditional knock-out mice. The results suggest that these deficits (1) are due to the absence of FMRP in the PFC alone and (2) are not the result of developmental dysregulation. Furthermore, PFC-associated deficits are rescued by initiating production of FMRP in adult conditional restoration mice, suggesting that PFC dysfunction may persist as long as FMRP is absent and therefore can be rescued after development. The data suggest that it is possible to dissociate the roles of FMRP in neural function from developmental dysregulation, and that PFC function can be restored in the adult FX brain. SIGNIFICANCE STATEMENT The absence of Fragile X Mental Retardation Protein (FMRP) from birth results in developmental disabilities and lifelong impairments. We show here that in mouse models PFC dysfunction in Fragile X Syndrome (FX) can be attributed to the continued absence of FMRP from the PFC, independent of FMRP status during development. Furthermore, initiation of FMRP production in the PFC of adult FX animals rescues PFC

A locus for axonal motor-sensory neuropathy with deafness and mental retardation maps to Xq24-q26

DOE Office of Scientific and Technical Information (OSTI.GOV)

Priest, J.M.; Nouri, N.; Keats, B.J.B.

1995-09-20

DNA markers on the X chromosome were used to map the locus for an unusual form of X-linked recessive hereditary motor and sensory neuropathy with associated deafness and mental retardation in a three-generation family that was originally reported by Towchock et al. This family included seven affected males, three obligate carrier females, and four unaffected males. The patients were severely affected within the first few years of life with distal weakness, muscle atrophy, sensory loss, areflexia, pes cavus, and hammer toes. Five of the seven affected males showed associated deafness, and three of these five individuals also presented with mentalmore » retardation or social development delay. Motor nerve conduction velocitites in affected males were normal to mildly delayed, and sensory conduction was markedly abnormal. Heterozygous females were asymptomatic. Close linkage to the Xg blood group locus (Xp22) and the PGK locus (Xq13) was previously excluded in this family, while weak linkage of the disease gene to DXYS1 (Xq21.3) was suggested. Our current linkage studies and haplotype analysis of 19 microsatellite markers on the long arm of the X chromosome demonstrate that DXS425 (Xq24) and HPRT (Xq26.1) are flanking markers and that the disease gene is closely linked to the markers DSX1122, DXS994, DXS737, DXS100, DXS1206, and DXS1047. 27 refs., 1 fig., 2 tabs.« less

Retraso Mental. Traduccion de ERIC EC Digest #528. (Mental Retardation. Translation of ERIC EC Digest #528).

ERIC Educational Resources Information Center

Hawkins-Shepard, Charlotte

This fact sheet presents basic information on mental retardation for Spanish-speaking educators and others. First, definitions from the Individuals with Disabilities Education Act (IDEA) and the American Association on Mental Retardation (AAMR) are presented. The fact sheet then analyzes how the new AAMR definitions differ from earlier ones,…

Epilepsy, Mental Retardation, and Anticonvulsant Therapy.

ERIC Educational Resources Information Center

Kaufman, Kenneth Roland; Katz-Garris, Lynda

1979-01-01

Inappropriate or inadequately documented medication for patients in mental retardation institutions is a major medical and economic problem. Within a 127-patient ward, 41 patients were treated with anticonvulsants. Of these patients, 24 had no documented indications for usage. (Author)

Mental Retardation: Past, Present and Future

ERIC Educational Resources Information Center

Crissey, Marie Skodak

1975-01-01

Notes that two developments had major impacts on policies towards the mentally retarded between the 1880s and the 1920s: (1) the swing toward the eugenics-heredity-genetics movement, and (2) the development of individual intelligence testing. (Author/JM)

Tracking the Fragile X Mental Retardation Protein in a Highly Ordered Neuronal RiboNucleoParticles Population: A Link between Stalled Polyribosomes and RNA Granules.

PubMed

El Fatimy, Rachid; Davidovic, Laetitia; Tremblay, Sandra; Jaglin, Xavier; Dury, Alain; Robert, Claude; De Koninck, Paul; Khandjian, Edouard W

2016-07-01

Local translation at the synapse plays key roles in neuron development and activity-dependent synaptic plasticity. mRNAs are translocated from the neuronal soma to the distant synapses as compacted ribonucleoparticles referred to as RNA granules. These contain many RNA-binding proteins, including the Fragile X Mental Retardation Protein (FMRP), the absence of which results in Fragile X Syndrome, the most common inherited form of intellectual disability and the leading genetic cause of autism. Using FMRP as a tracer, we purified a specific population of RNA granules from mouse brain homogenates. Protein composition analyses revealed a strong relationship between polyribosomes and RNA granules. However, the latter have distinct architectural and structural properties, since they are detected as close compact structures as observed by electron microscopy, and converging evidence point to the possibility that these structures emerge from stalled polyribosomes. Time-lapse video microscopy indicated that single granules merge to form cargoes that are transported from the soma to distal locations. Transcriptomic analyses showed that a subset of mRNAs involved in cytoskeleton remodelling and neural development is selectively enriched in RNA granules. One third of the putative mRNA targets described for FMRP appear to be transported in granules and FMRP is more abundant in granules than in polyribosomes. This observation supports a primary role for FMRP in granules biology. Our findings open new avenues for the study of RNA granule dysfunctions in animal models of nervous system disorders, such as Fragile X syndrome.

Teacher Report versus Adaptive Behavior Scale in Assessment of Mental Retardation.

ERIC Educational Resources Information Center

Al-Ansari, Ahmed

1993-01-01

This study assessed the degree of agreement between teacher report and an adapted Adaptive Behavior Scale in the identification of mental retardation and associated learning difficulties in 257 young Bahraini school children. Findings indicated that the instrument is sensitive in identification of children with mental retardation and exhibits high…

A 4q35.2 subtelomeric deletion identified in a screen of patients with co-morbid psychiatric illness and mental retardation

PubMed Central

Pickard, Ben S; Hollox, Edward J; Malloy, M Pat; Porteous, David J; Blackwood, Douglas HR; Armour, John AL; Muir, Walter J

2004-01-01

Background Cryptic structural abnormalities within the subtelomeric regions of chromosomes have been the focus of much recent research because of their discovery in a percentage of people with mental retardation (UK terminology: learning disability). These studies focused on subjects (largely children) with various severities of intellectual impairment with or without additional physical clinical features such as dysmorphisms. However it is well established that prevalence of schizophrenia is around three times greater in those with mild mental retardation. The rates of bipolar disorder and major depressive disorder have also been reported as increased in people with mental retardation. We describe here a screen for telomeric abnormalities in a cohort of 69 patients in which mental retardation co-exists with severe psychiatric illness. Methods We have applied two techniques, subtelomeric fluorescence in situ hybridisation (FISH) and multiplex amplifiable probe hybridisation (MAPH) to detect abnormalities in the patient group. Results A subtelomeric deletion was discovered involving loss of 4q in a patient with co-morbid schizoaffective disorder and mental retardation. Conclusion The precise region of loss has been defined allowing us to identify genes that may contribute to the clinical phenotype through hemizygosity. Interestingly, the region of 4q loss exactly matches that linked to bipolar affective disorder in a large multiply affected Australian kindred. PMID:15310400

Roles of fragile X mental retardation protein in dopaminergic stimulation-induced synapse-associated protein synthesis and subsequent alpha-amino-3-hydroxyl-5-methyl-4-isoxazole-4-propionate (AMPA) receptor internalization.

PubMed

Wang, Hansen; Kim, Susan S; Zhuo, Min

2010-07-09

Fragile X syndrome, the most common form of inherited mental retardation, is caused by the absence of the RNA-binding protein fragile X mental retardation protein (FMRP). FMRP regulates local protein synthesis in dendritic spines. Dopamine (DA) is involved in the modulation of synaptic plasticity. Activation of DA receptors can regulate higher brain functions in a protein synthesis-dependent manner. Our recent study has shown that FMRP acts as a key messenger for DA modulation in forebrain neurons. Here, we demonstrate that FMRP is critical for DA D1 receptor-mediated synthesis of synapse-associated protein 90/PSD-95-associated protein 3 (SAPAP3) in the prefrontal cortex (PFC). DA D1 receptor stimulation induced dynamic changes of FMRP phosphorylation. The changes in FMRP phosphorylation temporally correspond with the expression of SAPAP3 after D1 receptor stimulation. Protein phosphatase 2A, ribosomal protein S6 kinase, and mammalian target of rapamycin are the key signaling molecules for FMRP linking DA D1 receptors to SAPAP3. Knockdown of SAPAP3 did not affect surface expression of alpha-amino-3-hydroxyl-5-methyl-4-isoxazole-4-propionate (AMPA) GluR1 receptors induced by D1 receptor activation but impaired their subsequent internalization in cultured PFC neurons; the subsequent internalization of GluR1 was also impaired in Fmr1 knock-out PFC neurons, suggesting that FMRP may be involved in subsequent internalization of GluR1 through regulating the abundance of SAPAP3 after DA D1 receptor stimulation. Our study thus provides further insights into FMRP involvement in DA modulation and may help to reveal the molecular mechanisms underlying impaired learning and memory in fragile X syndrome.

People with Mental Retardation in the Criminal Justice System. ARC Q&A #101-47.

ERIC Educational Resources Information Center

Reynolds, Leigh Ann

This fact sheet uses a question-and-answer format to summarize issues related to people with mental retardation in the criminal justice system. Questions and answers address the following topics: the number of people with mental retardation in the criminal justice system (2 to 10 percent of the prison population is mentally retarded); whether…

Assessment of Seizures and Related Symptomatology in Persons with Mental Retardation

ERIC Educational Resources Information Center

Mayville, Erik A.; Matson, Johnny L.

2004-01-01

Seizures can be debilitating across a number of physical, social, occupational, and personal variables. Given the deficits in all of these areas frequently present in persons with mental retardation, effective assessment and subsequent treatment of seizures is a primary goal for individuals with both mental retardation and epilepsy. To thoroughly…

Who Is Interested in Careers in Mental Retardation and Developmental Disabilities?

ERIC Educational Resources Information Center

Kobe, Frank H.; Hammer, David

1993-01-01

Assessment of career interests in 80 undergraduate students enrolled in a course and practicum on the psychology of mental retardation found no significant changes in career interest from pretest to posttest. Students with prior exposure to individuals with mental retardation (especially as volunteers) reported high levels of career interest.…

Pharmacotherapy in Mental Retardation and Autism.

ERIC Educational Resources Information Center

Handen, Benjamin L.

1993-01-01

Reviews studies examining effects of pharmacological interventions for children with mental retardation and autism. Discusses information regarding stimulants, neuroleptics, anticonvulsants, antianxiety drugs, and antidepressant drugs as measured by their effects on laboratory and clinical measures of activity level, self-injurious behavior, and…

Mental Retardation: The Known and the Unknown. Information as of February 1, 1975.

ERIC Educational Resources Information Center

President's Committee on Mental Retardation, Washington, DC.

The report of the President's Committee on Mental Retardation deals with three goal areas: minimum occurrence of disability, adequate and humane service systems, and assurance of full citizenship for the retarded. Considered in an initial section are the background, definition, prevalence, and incidence of mental retardation. Reviewed is research…

Equally unequal: gender discrimination in the workplace among adults with mental retardation.

PubMed

Julius, Elona; Wolfson, Hagit; Yalon-Chamovitz, Shira

2003-01-01

Gender discrimination in the work place has been widely reported. Women are usually discriminated against both with respect to level of occupation and salary. The current study explored the correlation between gender and employment among adults with mental retardation, specifically, whether gender discrimination in the work place is as prominent among people with mental retardation as in the general population. Level of occupation and salary earned were studied in 227 adults with mild and moderate mental retardation residing in institutions, hostels, and sheltered homes in Israel. The findings suggest a correlation between gender and employment similar to that in the general population. Women were found to be employed mainly in sheltered workshops and lower levels of occupation, and to earn significantly less than the men. However, closer examination of each work place revealed that within each level of occupation there were no significant gender differences in salary. The finding suggests that while women with mental retardation earn lower salaries than men, this is mainly the result of their lower level of occupation. Rehabilitation efforts should therefore be directed toward ensuring higher levels of occupation as well as community employment among women with mental retardation.

Mental Retardation: The Search for Cures. Research Monograph Number 7.

ERIC Educational Resources Information Center

Menolascino, Frank J.; Neman, Ronald

The booklet describes the Association for Retarded Citizens' (ARC's) goal of coordinating efforts to seek a cure for mental retardation. Cures are defined as any intervention that would significantly increase intellectual functioning and adaptive behavior beyond the upper level of retardation. It is explained that because of the variety of causes…

[Schooling of patients exhibiting Autism Spectrum Disorders without mental retardation].

PubMed

Grimm, D; Assouline, B; Piero, A

2015-12-01

Autism Spectrum Disorders belong to Pervasive Development Disorders. Although access to education is recommended by the French National High Authority for Health (HAS), the practice remains limited and the reasons for the low education rate of these children have still not been sufficiently explored in the literature. The main objective of this study was to analyze the links between Autism Spectrum Disorder without mental retardation, psychiatric comorbidity and education. The secondary objective was to analyze the cognitive and contextual factors that could limit educational inclusion. Eighty-three autistic patients (3-18years old; 73 males and 10 females) with childhood autism, atypical autism or Asperger's syndrome (criteria from the International Classification of Diseases-10) without mental retardation and in education were assessed at the Alpine Centre for Early Diagnosis of Autism. The sample included 45 subjects with childhood autism, 12 subjects with atypical autism and 26 subjects with Asperger's syndrome. The diagnosis was based on the Autism Diagnostic Interview Revised (ADI-R), in accordance with the recommendations of the HAS, the Autism Diagnostic Observation Schedule (ADOS) and the Wechsler Intelligence Scale for Children, 4th edition (WISC-IV). Our results showed that childhood autism and atypical autism were mainly found in nursery and primary school, whereas Asperger's syndrome was mainly found in secondary school (Chi(2)=18.23; df=6; P<.006). Individuals with childhood autism and atypical autism were more likely to receive the support of a special educational assistant (Chi(2)=15.61; df=2; P<.000) and underwent a higher number of consultations and treatment episodes than those with Asperger's syndrome (Chi(2)=27.83; df=14; P<.015). The cognitive profiles obtained with the WISC-IV also differed: the Verbal Comprehension Index (VCI) and Working Memory Index (WMI) were higher for Asperger's syndrome than for childhood autism and atypical autism

Fragile X Mental Retardation Protein Restricts Small Dye Iontophoresis Entry into Central Neurons

PubMed Central

2017-01-01

Fragile X mental retardation protein (FMRP) loss causes Fragile X syndrome (FXS), a major disorder characterized by autism, intellectual disability, hyperactivity, and seizures. FMRP is both an RNA- and channel-binding regulator, with critical roles in neural circuit formation and function. However, it remains unclear how these FMRP activities relate to each other and how dysfunction in their absence underlies FXS neurological symptoms. In testing circuit level defects in the Drosophila FXS model, we discovered a completely unexpected and highly robust neuronal dye iontophoresis phenotype in the well mapped giant fiber (GF) circuit. Controlled dye injection into the GF interneuron results in a dramatic increase in dye uptake in neurons lacking FMRP. Transgenic wild-type FMRP reintroduction rescues the mutant defect, demonstrating a specific FMRP requirement. This phenotype affects only small dyes, but is independent of dye charge polarity. Surprisingly, the elevated dye iontophoresis persists in shaking B mutants that eliminate gap junctions and dye coupling among GF circuit neurons. We therefore used a wide range of manipulations to investigate the dye uptake defect, including timed injection series, pharmacology and ion replacement, and optogenetic activity studies. The results show that FMRP strongly limits the rate of dye entry via a cytosolic mechanism. This study reveals an unexpected new phenotype in a physical property of central neurons lacking FMRP that could underlie aspects of FXS disruption of neural function. SIGNIFICANCE STATEMENT FXS is a leading heritable cause of intellectual disability and autism spectrum disorders. Although researchers established the causal link with FMRP loss >;25 years ago, studies continue to reveal diverse FMRP functions. The Drosophila FXS model is key to discovering new FMRP roles, because of its genetic malleability and individually identified neuron maps. Taking advantage of a well characterized Drosophila neural

Fragile X Mental Retardation Protein Restricts Small Dye Iontophoresis Entry into Central Neurons.

PubMed

Kennedy, Tyler; Broadie, Kendal

2017-10-11

Fragile X mental retardation protein (FMRP) loss causes Fragile X syndrome (FXS), a major disorder characterized by autism, intellectual disability, hyperactivity, and seizures. FMRP is both an RNA- and channel-binding regulator, with critical roles in neural circuit formation and function. However, it remains unclear how these FMRP activities relate to each other and how dysfunction in their absence underlies FXS neurological symptoms. In testing circuit level defects in the Drosophila FXS model, we discovered a completely unexpected and highly robust neuronal dye iontophoresis phenotype in the well mapped giant fiber (GF) circuit. Controlled dye injection into the GF interneuron results in a dramatic increase in dye uptake in neurons lacking FMRP. Transgenic wild-type FMRP reintroduction rescues the mutant defect, demonstrating a specific FMRP requirement. This phenotype affects only small dyes, but is independent of dye charge polarity. Surprisingly, the elevated dye iontophoresis persists in shaking B mutants that eliminate gap junctions and dye coupling among GF circuit neurons. We therefore used a wide range of manipulations to investigate the dye uptake defect, including timed injection series, pharmacology and ion replacement, and optogenetic activity studies. The results show that FMRP strongly limits the rate of dye entry via a cytosolic mechanism. This study reveals an unexpected new phenotype in a physical property of central neurons lacking FMRP that could underlie aspects of FXS disruption of neural function. SIGNIFICANCE STATEMENT FXS is a leading heritable cause of intellectual disability and autism spectrum disorders. Although researchers established the causal link with FMRP loss >;25 years ago, studies continue to reveal diverse FMRP functions. The Drosophila FXS model is key to discovering new FMRP roles, because of its genetic malleability and individually identified neuron maps. Taking advantage of a well characterized Drosophila neural

Science and students with mental retardation: An analysis of curriculum features and learner characteristics

NASA Astrophysics Data System (ADS)

Scruggs, Thomas E.; Mastropieri, Margo A.

Although much research has been conducted on the learning characteristics of individuals with mental retardation, science learning of such individuals has received far less attention. In this investigation, students with mental retardation were observed over a 2-year period, in order to determine how the characteristics of mental retardation manifested themselves in the context of inquiry-oriented, hands-on science curriculum. Analysis of all relevant data sources, including observations and field notes, videotape and audiotape recordings, student products, and interviews, suggested that several characteristics commonly attributed to students with mild mental retardation were observed to interact with the science curriculum. These characteristics included attention, semantic memory, logical reasoning, and outerdirectedness. However, teachers were skilled at adapting instruction to meet the special needs of these learners. Implications for teaching science to students with mental retardation are provided.

Genetic studies in a patient with X-linked retinoschisis coexisting with developmental delay and sensorineural hearing loss.

PubMed

Sudha, Dhandayuthapani; Patric, Irene Rosita Pia; Ganapathy, Aparna; Agarwal, Smitha; Krishna, Shuba; Neriyanuri, Srividya; Sripriya, Sarangapani; Sen, Parveen; Chidambaram, Subbulakshmi; Arunachalam, Jayamuruga Pandian

2017-01-01

In this study, we present a juvenile retinoschisis patient with developmental delay, sensorineural hearing loss, and reduced axial tone. X-linked juvenile retinoschisis (XLRS) is a retinal dystrophy, most often not associated with systemic anomalies and also not showing any locus heterogeneity. Therefore it was of interest to understand the genetic basis of the condition in this patient. RS1 gene screening for XLRS was performed by Sanger sequencing. Whole genome SNP 6.0 array analysis was carried out to investigate gross chromosomal aberrations that could result in systemic phenotype. In addition, targeted next generation sequencing (NGS) was employed to determine any possible involvement of X-linked syndromic and non-syndromic mental retardation genes. This NGS panel consisted of 550 genes implicated in several other rare inherited diseases. RS1 gene screening revealed a pathogenic hemizygous splice site mutation (c.78+1G>T), inherited from the mother. SNP 6.0 array analysis did not indicate any significant chromosomal aberrations that could be disease-associated. Targeted resequencing did not identify any mutations in the X-linked mental retardation genes. However, variations in three other genes (NSD1, LARGE, and POLG) were detected, which were all inherited from the patient's unaffected father. Taken together, RS1 mutation was found to segregate with retinoschisis phenotype while none of the other identified variations were co-segregating with the systemic defects. Hereby, we infer that the multisystemic defects harbored by the patient are a rare coexistence of XLRS, developmental delay, sensorineural hearing loss, and reduced axial tone reported for the first time in the literature.

Sexual self-esteem in mothers of normal and mentally-retarded children.

PubMed

Tavakolizadeh, Jahanshir; Amiri, Mostafa; Nejad, Fahimeh Rastgoo

2017-06-01

Sexual self-esteem is negatively influenced by the stressful experiences in lifetime. This study compared the sexual self-esteem and its components in mothers with normal and mentally-retarded children in Qaen city, in 2014. A total of 120 mothers were selected and assigned into two groups of 60 samples based on convenient sampling method and randomized multiple stage sampling. Both groups completed sexual self-esteem questionnaire. The data were analyzed employing t-test through SPSS software version15. The results showed that the rate of sexual self-esteem in mothers of mentally-retarded children decreased significantly compared with that of mothers with normal children (p<0.05). Moreover, the mean scores of all components of sexual self-esteem including skill and experience, attractiveness, control, moral judgment, and adaptiveness in mothers of mentally-retarded children were significantly less than those of mothers with normal children (p <0.05). Therefore, it is recommended that self-esteem, especially the sexual one, be taught to mothers of mentally-retarded children by specialists.

Mental Retardation Activities of the U.S. Department of Health, Education, and Welfare.

ERIC Educational Resources Information Center

Department of Health, Education, and Welfare, Washington, DC. Secretary's Committee on Mental Retardation.

The coordination of mental retardation programs in the U.S. Department of Health, Education, and Welfare (DHEW) and 1968 mental retardation legislation are reviewed. Departmental activities summarized include those of the Health Services and Mental Health Administration, National Institutes of Health, Office of Education, Social and Rehabilitation…

Perception of Contingency and Mental Retardation.

ERIC Educational Resources Information Center

DeVellis, Robert F.; McCauley, Charley

1979-01-01

It is argued that the general learning difficulties exhibited by mentally retarded persons are similar in many respects to the learning difficulties of nonretarded persons who are in a state of learned helplessness (M. Seligman, 1975) or who are external in locus of control orientation. (Author)

Trends in Deinstitutionalization of the Mentally Retarded

ERIC Educational Resources Information Center

Conroy, James W.

1977-01-01

A review of national trends in deinstitutionalization of the mentally retarded indicated limited progress toward depopulation of institutions and showed that much of the national effort has been directed toward interinstitutional relocation. (Author/CL)

Isolation of anonymous DNA sequences from within a submicroscopic X chromosomal deletion in a patient with choroideremia, deafness, and mental retardation

DOE Office of Scientific and Technical Information (OSTI.GOV)

Nussbaum, R.L.; Lesko, J.G.; Lewis, R.A.

1987-09-01

Choroideremia, an X-chromosome linked retinal dystrophy of unknown pathogenesis, causes progressive nightblindness and eventual central blindness in affected males by the third to fourth decade of life. Choroideremia has been mapped to Xq13-21 by tight linkage to restriction fragment length polymorphism loci. The authors have recently identified two families in which choroideremia is inherited with mental retardation and deafness. In family XL-62, an interstitial deletion Xq21 is visible by cytogenetic analysis and two linked anonymous DNA markers, DXYS1 and DXS72, are deleted. In the second family, XL-45, an interstitial deletion was suspected on phenotypic grounds but could not be confirmedmore » by high-resolution cytogenetic analysis. They used phenol-enhanced reassociation of 48,XXXX DNA in competition with excess XL-45 DNA to generate a library of cloned DNA enriched for sequences that might be deleted in XL-45. Two of the first 83 sequences characterized from the library were found to be deleted in probands from family XL-45 as well as from family XL-62. Isolation of these sequences proves that XL-45 does contain a submicroscopic deletion and provides a starting point for identifying overlapping genomic sequences that span the XL-45 deletion. Each overlapping sequence will be studied to identify exons from the choroideremia locus.« less

Epilepsy and Mental Retardation: An Overview.

ERIC Educational Resources Information Center

Coulter, David L.

1993-01-01

The comprehensive management of epilepsy in people with mental retardation requires consideration of four aspects of care: diagnosis and classification, anticonvulsant drug treatment, safety and protection from injury, and psychosocial functioning. This paper outlines what is known and unknown in these four areas and introduces articles in this…

Proceedings of the Conference on Nutrition and Mental Retardation (Ann Arbor, Michigan, February 10-11, 1971).

ERIC Educational Resources Information Center

Springer, Ninfa Saturnino, Ed.

The conference, planned primarily for nutritionists and dieticians, dealt with the role of nutrition in the prevention and management of mental retardation. Proceedings include an overview of mental retardation, an examination of nutrition manpower needs in the fields of mental health and mental retardation on both the national and state levels,…

Oral Health of Institutionalized Individuals with Mental Retardation.

ERIC Educational Resources Information Center

Jurek, George H.; Reid, William H.

1993-01-01

Residents (n=702) of Texas Department of Mental Health and Mental Retardation institutions were given dental examinations, and findings were compared with data from 1,077 residents 9 years earlier. Data were analyzed for oral pathology, unmet dental needs, contribution of dental care to habilitation, and effectiveness of the dental service system.…

THE MENTALLY RETARDED CHILD, A PSYCHOLOGICAL APPROACH. MCGRAW-HILL SERIES IN PSYCHOLOGY.

ERIC Educational Resources Information Center

ROBINSON, HALBERT B.; ROBINSON, NANCY M.

PRESENTING A PSYCHOLOGICAL APPROACH TO MENTAL RETARDATION, THIS TEXT BEGINS WITH A DISCUSSION OF THEORIES OF INTELLIGENCE, PROBLEMS OF DEFINITION, AND THE CURRENT STATUS OF THE FIELD OF MENTAL RETARDATION. A SECTION ON ETIOLOGY AND SYNDROMES PRESENTS INFORMATION ON GENETIC FACTORS AND GENETIC SYNDROMES AND THE PHYSICAL AND PSYCHOLOGICAL…

International Directory of Mental Retardation Resources.

ERIC Educational Resources Information Center

Dybwad, Rosemary F., Ed.

The directory lists and describes governmental and voluntary agencies, research, and other resources in the field of mental retardation in foreign countries. The first section, on international organizations, gives names, addresses, names of directors, and one or more paragraphs of description for the United Nations and its specialized agencies,…

Diagnostic test for prenatal identification of Down's syndrome and mental retardation and gene therapy therefor

DOEpatents

Smith, Desmond J.; Rubin, Edward M.

2000-01-01

A a diagnostic test useful for prenatal identification of Down syndrome and mental retardation. A method for gene therapy for correction and treatment of Down syndrome. DYRK gene involved in the ability to learn. A method for diagnosing Down's syndrome and mental retardation and an assay therefor. A pharmaceutical composition for treatment of Down's syndrome mental retardation.

Roles of Fragile X Mental Retardation Protein in Dopaminergic Stimulation-induced Synapse-associated Protein Synthesis and Subsequent α-Amino-3-hydroxyl-5-methyl-4-isoxazole-4-propionate (AMPA) Receptor Internalization*

PubMed Central

Wang, Hansen; Kim, Susan S.; Zhuo, Min

2010-01-01

Fragile X syndrome, the most common form of inherited mental retardation, is caused by the absence of the RNA-binding protein fragile X mental retardation protein (FMRP). FMRP regulates local protein synthesis in dendritic spines. Dopamine (DA) is involved in the modulation of synaptic plasticity. Activation of DA receptors can regulate higher brain functions in a protein synthesis-dependent manner. Our recent study has shown that FMRP acts as a key messenger for DA modulation in forebrain neurons. Here, we demonstrate that FMRP is critical for DA D1 receptor-mediated synthesis of synapse-associated protein 90/PSD-95-associated protein 3 (SAPAP3) in the prefrontal cortex (PFC). DA D1 receptor stimulation induced dynamic changes of FMRP phosphorylation. The changes in FMRP phosphorylation temporally correspond with the expression of SAPAP3 after D1 receptor stimulation. Protein phosphatase 2A, ribosomal protein S6 kinase, and mammalian target of rapamycin are the key signaling molecules for FMRP linking DA D1 receptors to SAPAP3. Knockdown of SAPAP3 did not affect surface expression of α-amino-3-hydroxyl-5-methyl-4-isoxazole-4-propionate (AMPA) GluR1 receptors induced by D1 receptor activation but impaired their subsequent internalization in cultured PFC neurons; the subsequent internalization of GluR1 was also impaired in Fmr1 knock-out PFC neurons, suggesting that FMRP may be involved in subsequent internalization of GluR1 through regulating the abundance of SAPAP3 after DA D1 receptor stimulation. Our study thus provides further insights into FMRP involvement in DA modulation and may help to reveal the molecular mechanisms underlying impaired learning and memory in fragile X syndrome. PMID:20457613

INTEGRATION OF STIMULUS CUES BY NORMAL AND MENTALLY RETARDED CHILDREN. FINAL REPORT.

ERIC Educational Resources Information Center

ELAM, CLAUDE B.

TWO EXPERIMENTS WERE CONDUCTED IN ORDER TO OBTAIN A MATHEMATICAL DESCRIPTION OF THE PERCEPTUAL PROCESS BY WHICH NORMAL AND MENTALLY RETARDED SUBJECTS SYNTHESIZE STIMULUS CUES IN PERCEPTUAL IDENTIFICATION. THE INITIAL STUDY EMPLOYED 50 COLLEGE STUDENTS, 34 GRADE SCHOOL STUDENTS, AND 24 MENTALLY RETARDED CHILDREN (AGES 9-16) AS SUBJECTS. THE…

Preparing the Mentally Retarded in the Areas of Food Preparation and Service.

ERIC Educational Resources Information Center

Mattson, Bruce D.; And Others

The report of a project for planning a training program for the mentally retarded in the areas of food preparation and service summarizes activities of the planning project and makes recommendations for possible implementation. The training program is designed for educable mentally retarded youth who would benefit from specific vocational…

Haloperidol Treatment of Trichotillomania in a Boy with Autism and Mental Retardation.

ERIC Educational Resources Information Center

Ghaziuddin, M.; And Others

1991-01-01

The report describes the successful treatment of trichotillomania (compulsive hair pulling) in a mentally retarded 11-year-old boy with autism and severe mental retardation. Administration of haloperidol resulted in complete cessation of hair pulling which reappeared when the dosage was decreased and ceased again when dosage was reestablished. (DB)

Neurological impairments and sleep-wake behaviour among the mentally retarded.

PubMed

Lindblom, N; Heiskala, H; Kaski, M; Leinonen, L; Nevanlinna, A; Iivanainen, M; Laakso, M L

2001-12-01

The objective of the present study was to evaluate the relationship between the sleep-wake behaviour and neurological impairments among mentally retarded people. The sleep-wake behaviour of 293 mentally retarded subjects living in a rehabilitation center was studied by a standardized observation protocol carried out by trained staff members. The protocol consisted of brief check-ups of the subjects' sleep-wake status at 20-min intervals for five randomly chosen 24-h periods during 4 months. From the raw data five sleep-wake behaviour variables were formed. The data concerning the subject characteristics (age, body mass index (BMI), gender, degree of mental retardation, presence of locomotor disability, that of epilepsy, blindness or deafness and the usage of psychotropic medications) were collected from the medical records. Two main findings emerged: (1) severe locomotor disability, blindness and active epilepsy were found to be independent predictors of increased daytime sleep and increased number of wake-sleep transitions and (2) the subjects with a combination of two or all three of these impairments had a significantly more fragmented and abnormally distributed sleep than those with none or milder forms of these impairments. Age, BMI, degree of mental retardation and the studied medications played a minor role in the sleep disturbances of the study population. Finally, deafness was not found to be associated with any of the measured sleep-wake variables.

Regulation of neuronal excitability by interaction of fragile X mental retardation protein with slack potassium channels.

PubMed

Zhang, Yalan; Brown, Maile R; Hyland, Callen; Chen, Yi; Kronengold, Jack; Fleming, Matthew R; Kohn, Andrea B; Moroz, Leonid L; Kaczmarek, Leonard K

2012-10-31

Loss of the RNA-binding protein fragile X mental retardation protein (FMRP) represents the most common form of inherited intellectual disability. Studies with heterologous expression systems indicate that FMRP interacts directly with Slack Na(+)-activated K(+) channels (K(Na)), producing an enhancement of channel activity. We have now used Aplysia bag cell (BC) neurons, which regulate reproductive behaviors, to examine the effects of Slack and FMRP on excitability. FMRP and Slack immunoreactivity were colocalized at the periphery of isolated BC neurons, and the two proteins could be reciprocally coimmunoprecipitated. Intracellular injection of FMRP lacking its mRNA binding domain rapidly induced a biphasic outward current, with an early transient tetrodotoxin-sensitive component followed by a slowly activating sustained component. The properties of this current matched that of the native Slack potassium current, which was identified using an siRNA approach. Addition of FMRP to inside-out patches containing native Aplysia Slack channels increased channel opening and, in current-clamp recordings, produced narrowing of action potentials. Suppression of Slack expression did not alter the ability of BC neurons to undergo a characteristic prolonged discharge in response to synaptic stimulation, but prevented recovery from a prolonged inhibitory period that normally follows the discharge. Recovery from the inhibited period was also inhibited by the protein synthesis inhibitor anisomycin. Our studies indicate that, in BC neurons, Slack channels are required for prolonged changes in neuronal excitability that require new protein synthesis, and raise the possibility that channel-FMRP interactions may link changes in neuronal firing to changes in protein translation.

Sleep Studies of Adults with Severe or Profound Mental Retardation and Epilepsy.

ERIC Educational Resources Information Center

Espie, Colin A.; Paul, Audrey; McFie, Joyce; Amos, Pat; Hamilton, David; McColl, John H.; And Others

1998-01-01

A study of the sleep patterns of 28 people with severe or profound mental retardation and epilepsy found atypical sleep stages with significant depletion of REM sleep and a predominance of indiscriminate non-REM sleep. Sleep diaries completed by caregivers reveal lengthy sleep periods, especially among those with profound mental retardation.…

Becoming a Neighbor: An Examination of the Placement of People with Mental Retardation in Connecticut Communities.

ERIC Educational Resources Information Center

Bradley, Valerie J.; And Others

This study examined the impact of deinstitutionalization of the mentally retarded on their communities, the extent of availability of support services, and the extent to which the Connecticut Department of Mental Retardation assures adequate quality of support services and day programs for people with mental retardation living in community…

Veridical and False Pictorial Memory in Individuals with and without Mental Retardation

ERIC Educational Resources Information Center

Carlin, Michael T.; Toglia, Michael P.; Wakeford, Yvonne; Jakway, Allison; Sullivan, Kate; Hasel, Lisa

2008-01-01

Veridical and false pictorial recognition were assessed in individuals with mental retardation; groups were matched for MA and CA. Pictures were viewed in either a generative or static format at acquisition. The individuals with mental retardation and those in the MA-matched group had higher rates of false memories for critical items and lower hit…

Muscle fatigue during intermittent exercise in individuals with mental retardation.

PubMed

Zafeiridis, Andreas; Giagazoglou, Paraskevi; Dipla, Konstantina; Salonikidis, Konstantinos; Karra, Chrisanthi; Kellis, Eleftherios

2010-01-01

This study examined fatigue profile during intermittent exercise in 10 men with mild to moderate mental retardation (MR) and 10 men without mental retardation (C). They performed 4 x 30s maximal knee extensions and flexions with 1-min rest on an isokinetic dynamometer. Peak torque of flexors (PTFL) and extensors (PTEX), total work (TW), and lactate were measured. Fatigue was calculated as the magnitude of decline (%) in PTFL, PTEX, and TW and as rate of decline (linear slope) in TW from 1st to 4th set. MR had lower PTFL, PTEX, TW, and lactate throughout the protocol than C, while pre-motor time was greater in MR vs. C (p<0.05). MR demonstrated a delayed pattern of reduction in muscular performance. Lower values were observed in MR vs. C in the magnitude of decline for PTEX and TW and the rate of decline for TW. In conclusion, MR exhibit a different fatigue profile during intermittent exercise than C. The lower magnitude and decline rate in neuromuscular performance in MR during intermittent exercise is associated with their lower peak strength, short-term anaerobic capacity, and lactate accumulation. Rehabilitation and sport professionals should consider the differences in fatigue profile when designing intermittent exercise programs for MR. Copyright 2009 Elsevier Ltd. All rights reserved.

Individualized Educational Programming for the Mentally Retarded.

ERIC Educational Resources Information Center

Singh, Nirbhay N.; Ahrens, Michael G.

1980-01-01

The minimal components of a model which utilizes a computer for summarizing individual performance records for teaching educational skills to the mentally retarded are described. The most important components are assessment, individual and group programing, continuous data collection, and program evaluation. (Author)

Mental Retardation and the Problem of "Normality": Self-Determination and Identity Choice

ERIC Educational Resources Information Center

Smith, J. David

2007-01-01

The meaning of the category and concept of mental retardation is explored through the words of a fictional character, and the accounts of real people who have been injured and stigmatized by the label. Examples of the extremes to which people have gone to avoid or escape the term mental retardation are provided. The classification of retardation…

Teachers' and Mothers' Assessment of Social Skills of Students with Mental Retardation

ERIC Educational Resources Information Center

Cifci Tekinarslan, Ilknur; Sazak Pinar, Elif; Sucuoglu, Bulbin

2012-01-01

The purpose of this study is to compare the assessment results of social skills of students with mental retardation by their teachers and mothers through relational model by using descriptive statistics. The research group in this study consisted of mothers and teachers of 562 children with mental retardation aged between 6 and 12 who enrolled in…

The Mentally Retarded Inmate: Prison Adjustment and Implications for Treatment.

ERIC Educational Resources Information Center

Smith, Craig; And Others

1988-01-01

Examined whether or not there were differences in the prison adaptation of mentally retarded and nonretarded inmates. Compared 439 retarded inmates, 439 matched nonretarded inmates, and 439 unmatched nonretarded inmates. Found statistically significant differences between groups in the areas of assaults on correctional officers and other…

Fears of institutionalized mentally retarded adults.

PubMed

Sternlicht, M

1979-01-01

The patterns of fears of institutionalized mentally retarded adults were studied in a sample of i2 moderately retarded men and women between the ages of 21-49. The direct questioning method was employed. Two interviews were held, two weeks apart; the first interview elicited the Ss' fears, while the second concerned the fears of their friends. A total of 146 responses were obtained, and these were categorized according to the types of fears: supernatural-natural events, animals, physical injury, psychological stress, egocentric responses, and no fears. The Ss displayed a higher percentage of fears in the preoperational stage than in the concrete operational stage. In a comparison of male to female fears, only one category, that of fears of animals, reached significance. The study suggested that the same developmental trend of fears that appears in normal children appears in the retarded as well, and these fears follow Piaget's level of cognitive development, proceeding from egocentric perceptions of causality to realistic cause and effect thinking.

Medicaid-financed residential care for persons with mental retardation.

PubMed

Lakin, K C; Hall, M J

1990-12-01

Two sources of Medicaid support for persons with mental retardation and related conditions (MR/RC) are examined, the intermediate care facility for the mentally retarded (ICF/MR) program and the home and community-based services (HCBS) waiver. Results indicate that Medicaid support through the ICF/MR program has shown little recent growth in terms of number of persons served, although expenditures continue to increase. Medicaid's HCBS waiver is being used increasingly by States to support residential placement because of its greater flexibility and more individualized approach relative to ICF/MR care. Use of Medicaid to finance care for persons with MR/RC varies considerably across States.

Medicaid-financed residential care for persons with mental retardation

PubMed Central

Lakin, K. Charlie; Hall, Margaret Jean

1990-01-01

Two sources of Medicaid support for persons with mental retardation and related conditions (MRIRC) are examined, the intermediate care facility for the mentally retarded (ICF/MR) program and the home and community-based services (HCBS) waiver. Results indicate that Medicaid support through the ICF/MR program has shown little recent growth in terms of number of persons served, although expenditures continue to increase. Medicaid's HCBS waiver is being used increasingly by States to support residential placement because of its greater flexibility and more individualized approach relative to ICF/MR care. Use of Medicaid to finance care for persons with MR/RC varies considerably across States. PMID:10113489

Psychometric Properties of ADHD Rating Scales among Children with Mental Retardation I: Reliability

ERIC Educational Resources Information Center

Miller, Michael L.; Fee, Virginia E.; Netterville, Amanda K.

2004-01-01

The reliability of Attention-Deficit/Hyperactivity Disorder (ADHD) rating scales in children with mental retardation was assessed. Parents, teachers, and teaching assistants completed ADHD rating scales on 48 children aged 5-12 diagnosed with mental retardation. Measures included the Child Behavior Checklist (CBCL), Conners Rating Scales, the…

Clozapine in Three Individuals with Mild Mental Retardation and Treatment-Refractory Psychiatric Disorders.

ERIC Educational Resources Information Center

Pary, Robert J.

1994-01-01

Although clozapine is a drug specifically approved for people with schizophrenia, it has not been systematically evaluated with dually diagnosed individuals having mental retardation. This article reviews the drug's use in the general population, discusses potential difficulties in prescribing it for individuals with mental retardation, and…

Drugs in Mental Retardation: Treatment or Tragedy?

ERIC Educational Resources Information Center

Aman, Michael G.

1985-01-01

Treatment of mentally retarded persons with psychotropic and anticonvulsant drugs is discussed in terms of drug classification, rationale for use, attitudes toward use, and clinical research findings. The literature on neuroleptic, anticonvulsant, anxiolytic, and cerebral stimulant drugs is summarized. Controversial reports that some medications…

Autism and mental retardation among offspring born after in vitro fertilization.

PubMed

Sandin, Sven; Nygren, Karl-Gösta; Iliadou, Anastasia; Hultman, Christina M; Reichenberg, Abraham

2013-07-03

Between 1978 and 2010, approximately 5 million infants were born after in vitro fertilization (IVF) treatments. Yet limited information on neurodevelopment after IVF exists, especially after the first year of life. To examine the association between use of any IVF and different IVF procedures and the risk of autistic disorder and mental retardation in the offspring. A population-based, prospective cohort study using Swedish national health registers. Offspring born between 1982 and 2007 were followed up for a clinical diagnosis of autistic disorder or mental retardation until December 31, 2009. The exposure of interest was IVF, categorized according to whether intracytoplasmic sperm injection (ICSI) for male infertility was used and whether embryos were fresh or frozen. For ICSI, whether sperm were ejaculated or surgically extracted was also considered. Relative risks (RRs) for autistic disorder and mental retardation and rates per 100,000 person-years, comparing spontaneously conceived offspring with those born after an IVF procedure and comparing 5 IVF procedures used in Sweden vs IVF without ICSI with fresh embryo transfer, the most common treatment. We also analyzed the subgroup restricted to singletons. Of the more than 2.5 million infants born, 30,959 (1.2%) were conceived by IVF and were followed up for a mean 10 (SD, 6) years. Overall, 103 of 6959 children (1.5%) with autistic disorder and 180 of 15,830 (1.1%) with mental retardation were conceived by IVF. The RR for autistic disorder after any procedure compared with spontaneous conception was 1.14 (95% CI, 0.94-1.39; 19.0 vs 15.6 per 100,000 person-years). The RR for mental retardation was 1.18 (95% CI, 1.01-1.36; 46.3 vs 39.8 per 100,000 person-years). For both outcomes, there was no statistically significant association when restricting analysis to singletons. Compared with IVF without ICSI with fresh embryo transfer, there were statistically significantly increased risks of autistic disorder following

Utilization of Computer Technology To Facilitate Money Management by Individuals with Mental Retardation.

ERIC Educational Resources Information Center

Davies, Daniel K.; Stock, Steven E.; Wehmeyer, Michael L.

2003-01-01

This report describes results of an initial investigation of the utility of a specially designed money management software program for improving management of personal checking accounts for individuals with mental retardation. Use with 19 adults with mental retardation indicated the software resulted in significant reduction in check writing and…

Medical Diagnostic Consultation concerning Mental Retardation: An Analogue Study of School Psychologists' Attitudes

ERIC Educational Resources Information Center

Wodrich, David L.; Tarbox, Jennifer; Balles, John; Gorin, Joanna

2010-01-01

Recent research of relevance to school psychologists suggests that the cause, or etiology, of mental retardation can be established by medical diagnosticians in approximately one-half of cases. In the current study, 109 practicing school psychologists considered a hypothetical case of an elementary student with mental retardation and indicated…

Development of Services for Elderly Persons with Mental Retardation in a Rural State.

ERIC Educational Resources Information Center

Cotten, Paul D.; Spirrison, Charles L.

1988-01-01

A model demonstration project to meet the needs of elderly mentally retarded individuals in a rural state was designed as a complementary, collaborative endeavor among service providers from the aging, mental retardation, and generic service system networks in Mississippi. Continuous training of staff members across networks was emphasized. (JW)

Transcription map of Xq27: candidates for several X-linked diseases.

PubMed

Zucchi, I; Jones, J; Affer, M; Montagna, C; Redolfi, E; Susani, L; Vezzoni, P; Parvari, R; Schlessinger, D; Whyte, M P; Mumm, S

1999-04-15

Human Xq27 contains candidate regions for several disorders, yet is predicted to be a gene-poor cytogenetic band. We have developed a transcription map for the entire cytogenetic band to facilitate the identification of the relatively small number of expected candidate genes. Two approaches were taken to identify genes: (1) a group of 64 unique STSs that were generated during the physical mapping of the region were used in RT-PCR with RNA from human adult and fetal brain and (2) ESTs that have been broadly mapped to this region of the chromosome were finely mapped using a high-resolution yeast artificial chromosome contig. This combined approach identified four distinct regions of transcriptional activity within the Xq27 band. Among them is a region at the centromeric boundary that contains candidate regions for several rare developmental disorders (X-linked recessive hypoparathyroidism, thoracoabdominal syndrome, albinism-deafness syndrome, and Borjeson-Forssman-Lehman syndrome). Two transcriptionally active regions were identified in the center of Xq27 and include candidate regions for X-linked mental retardation syndrome 6, X-linked progressive cone dystrophy, X-linked retinitis pigmentosa 24, and a prostate cancer susceptibility locus. The fourth region of transcriptional activity encompasses the FMR1 (FRAXA) and FMR2 (FRAXE) genes. The analysis thus suggests clustered transcription in Xq27 and provides candidates for several heritable disorders for which the causative genes have not yet been found. Copyright 1999 Academic Press.

X-linked cataract and Nance-Horan syndrome are allelic disorders.

PubMed

Coccia, Margherita; Brooks, Simon P; Webb, Tom R; Christodoulou, Katja; Wozniak, Izabella O; Murday, Victoria; Balicki, Martha; Yee, Harris A; Wangensteen, Teresia; Riise, Ruth; Saggar, Anand K; Park, Soo-Mi; Kanuga, Naheed; Francis, Peter J; Maher, Eamonn R; Moore, Anthony T; Russell-Eggitt, Isabelle M; Hardcastle, Alison J

2009-07-15

Nance-Horan syndrome (NHS) is an X-linked developmental disorder characterized by congenital cataract, dental anomalies, facial dysmorphism and, in some cases, mental retardation. Protein truncation mutations in a novel gene (NHS) have been identified in patients with this syndrome. We previously mapped X-linked congenital cataract (CXN) in one family to an interval on chromosome Xp22.13 which encompasses the NHS locus; however, no mutations were identified in the NHS gene. In this study, we show that NHS and X-linked cataract are allelic diseases. Two CXN families, which were negative for mutations in the NHS gene, were further analysed using array comparative genomic hybridization. CXN was found to be caused by novel copy number variations: a complex duplication-triplication re-arrangement and an intragenic deletion, predicted to result in altered transcriptional regulation of the NHS gene. Furthermore, we also describe the clinical and molecular analysis of seven families diagnosed with NHS, identifying four novel protein truncation mutations and a novel large deletion encompassing the majority of the NHS gene, all leading to no functional protein. We therefore show that different mechanisms, aberrant transcription of the NHS gene or no functional NHS protein, lead to different diseases. Our data highlight the importance of copy number variation and non-recurrent re-arrangements leading to different severity of disease and describe the potential mechanisms involved.

X-linked cataract and Nance-Horan syndrome are allelic disorders

PubMed Central

Coccia, Margherita; Brooks, Simon P.; Webb, Tom R.; Christodoulou, Katja; Wozniak, Izabella O.; Murday, Victoria; Balicki, Martha; Yee, Harris A.; Wangensteen, Teresia; Riise, Ruth; Saggar, Anand K.; Park, Soo-Mi; Kanuga, Naheed; Francis, Peter J.; Maher, Eamonn R.; Moore, Anthony T.; Russell-Eggitt, Isabelle M.; Hardcastle, Alison J.

2009-01-01

Nance-Horan syndrome (NHS) is an X-linked developmental disorder characterized by congenital cataract, dental anomalies, facial dysmorphism and, in some cases, mental retardation. Protein truncation mutations in a novel gene (NHS) have been identified in patients with this syndrome. We previously mapped X-linked congenital cataract (CXN) in one family to an interval on chromosome Xp22.13 which encompasses the NHS locus; however, no mutations were identified in the NHS gene. In this study, we show that NHS and X-linked cataract are allelic diseases. Two CXN families, which were negative for mutations in the NHS gene, were further analysed using array comparative genomic hybridization. CXN was found to be caused by novel copy number variations: a complex duplication–triplication re-arrangement and an intragenic deletion, predicted to result in altered transcriptional regulation of the NHS gene. Furthermore, we also describe the clinical and molecular analysis of seven families diagnosed with NHS, identifying four novel protein truncation mutations and a novel large deletion encompassing the majority of the NHS gene, all leading to no functional protein. We therefore show that different mechanisms, aberrant transcription of the NHS gene or no functional NHS protein, lead to different diseases. Our data highlight the importance of copy number variation and non-recurrent re-arrangements leading to different severity of disease and describe the potential mechanisms involved. PMID:19414485

Persons with Mental Retardation and Related Conditions in Mental Retardation Facilities: Selected Findings from the 1987 National Medical Expenditure Survey. Project Report 29.

ERIC Educational Resources Information Center

Lakin, K. Charlie; And Others

This report presents statistics on residential facilities for the mentally retarded and on the residents themselves, derived from the Institutional Population Component of the 1987 National Medical Expenditure Survey (NMES). Part 1 presents an overview of the NMES and discusses previous efforts to survey persons residing in mental retardation…

Computer Needs of Severely Mentally Retarded Persons.

ERIC Educational Resources Information Center

Flanagan, Kelly

1982-01-01

The article reviews technology applicable for use by severely mentally retarded learners. Descriptions are given of assistive devices (including communication aids), controls and interfaces (such as single switch access to standard software), and software (including games to teach cause and effect and simple matching and counting). (CL)

Institutionalization, Involuntary Sterilization, and Mental Retardation: Profiles from the History of the Practice.

ERIC Educational Resources Information Center

Smith, David J.; Polloway, Edward A.

1993-01-01

This study analyzes data on 212 individuals with mental retardation who were sterilized while residents of the Central Virginia Training Center and discharged from the facility between 1969 and 1989. The analysis includes gender, age at and date of sterilization, level of mental retardation, and location of subsequent discharge. (Author/JDD)

Evaluation of a Weight Loss Treatment Program for Individuals with Mild Mental Retardation

ERIC Educational Resources Information Center

Sailer, Angela B.; Miltenberger, Raymond G.; Johnson, Brigitte; Zetocha, Kim; Egemo, Kristin; Hegstad, Holly

2006-01-01

Obesity is a major problem for people in the United States. Individuals with mental retardation also are prone to obesity. The most successful programs for treating obesity typically are treatment packages consisting of multiple behavioral components. Research in the area of weight loss and mental retardation has not evaluated programs for…

Individuals with Mental Retardation and the Criminal Justice System: The View from States' Attorneys General.

ERIC Educational Resources Information Center

McAfee, James K.; Gural, Michele

1988-01-01

Results of a survey of state attorneys general (N=46) found that, with few exceptions, identification of persons with mental retardation in criminal justice is neither systematic nor probable. Protections lie in statutes pertaining to mental illness rather than to mental retardation. (Author/DB)

Short-Term Memory for Movement Duration in Mentally Retarded and Nonretarded Adults.

ERIC Educational Resources Information Center

Elliott, Digby; Grundy, Stephen B.

1984-01-01

Both retarded and nonretarded adults (N=26) failed to benefit from opportunities to mentally rehearse their criterion movement over the retention interval, suggesting that strategic differences between the groups cannot explain the poorer performances of the retarded Ss. The retarded Ss appeared to have problems remembering the sensory…

[Long-term dental interventions in mentally retarded children under general anesthesia with sevoflurane].

PubMed

Sitkin, S I; Gasparian, A L; Ivanova, T Iu; Nesterova, E Iu; Drozdova, N I

2015-01-01

Dental procedures in mentally retarded children is challenging for both dentist and for anesthesiologist. The aim of the study was to evaluate the efficacy and safety of dental care procedures under general anesthesia with sevoflurane by means of laryngeal mask in mentally retarded children. The randomized controlled study included 65 mentally retarded children with ASA 2-3 who underwent dental treatment. All patients had multiple caries. The children were divided into two groups. The first group included 35 children with normal body weigh while the second one - 30 obese children. All patients received a rapid induction with sevoflurane with the subsequent installation of the laryngeal mask. In the second group the signs of hypoventilation recorded an average of 10 ± 4 minutes after induction of anesthesia, which was manifested in increasing Pсо₂greater than 50 mm Hg. In the first group, the signs of hypoventilation marked an average of 18 ± 3.5 minutes from the start of induction of anesthesia. All patients were transferred to the artificial lung ventilation through the LMA. By dental treatment under general anesthesia with sevoflurane and laryngeal mask all mentally retarded children had respiratory depression with increased levels of carbon dioxide greater than 50 mmHg, but obese children developed these signs of hypoventilation twice as fast. Conducting long dental treatment in mentally retarded children require artificial lung ventilation.

Nonspecific Mental Retardation in British Columbia as Ascertained through a Registry.

ERIC Educational Resources Information Center

Herbst, Diana S.; Baird, Patricia A.

1983-01-01

Findings from the British Columbia Health Surveillance Registry revealed information about the age specific prevalence of mental retardation with no known etiology; association of mild and profound retardation with microcephalus, hydrocephalus, cerebral palsy, or epilepsy; and survival rates. (Author/CL)

Megavitamin treatment of mental retardation in children: a review of effects on behavior and cognition.

PubMed

Kozlowski, B W

1992-01-01

ABSTRACT Many forms of vitamin supplementation have been proposed for the treatment of behavioral and cognitive disorders in children with mental retardation. Except for nutrient deficiencies and selected inborn errors of metabolism, the efficacy of these treatments has not been established. Therapeutic supplementation with vitamin B(6) in Down syndrome and folie acid in fragile X syndrome was attempted following the identification of apparent biochemical aberrations involving the nutrients. Double-blind controlled trials have revealed no evidence that B(6) treatment was effective in Down syndrome. Very limited evidence, only among prepubertal subjects, suggested that behaviors improved with folie acid treatment in fragile X syndrome. There is less clarity of the scientific rationales for various combinations of vitamins (or vitamins and minerals) that have been promoted according to the concept of orthomolecular medicine. Well-designed controlled studies, with data presented in conformity with generally accepted scientific standards, have not supported the efficacy of megadose supplementation with 1) multivitamins in cognitive disabilities or attention deficit disorders, or 2) multivitamins and minerals in Down syndrome or other forms of mental retardation. Insufficient data are available to support claims made for vitamin B(6) and magnesium supplementation in autism. Research in the larger field of nutrient-behavior research has reinforced the need for studies to be more rigorously designed and to draw on the expertise of multiple disciplines. Evidence of toxic effects of nutrients continues to accrue, reinforcing the fact that megadoses should not be used indiscriminately or without physician monitoring. For children with mental retardation generally, assuring that established nutritional needs are met warrants primary consideration.

Enhancing independent time-management skills of individuals with mental retardation using a Palmtop personal computer.

PubMed

Davies, Daniel K; Stock, Steven E; Wehmeyer, Michael L

2002-10-01

Achieving greater independence for individuals with mental retardation depends upon the acquisition of several key skills, including time-management and scheduling skills. The ability to perform tasks according to a schedule is essential to domains like independent living and employment. The use of a portable schedule prompting system to increase independence and self-regulation in time-management for individuals with mental retardation was examined. Twelve people with mental retardation participated in a comparison of their use of the technology system to perform tasks on a schedule with use of a written schedule. Results demonstrated the utility of a Palmtop computer with schedule prompting software to increase independence in the performance of vocational and daily living tasks by individuals with mental retardation.

Mindfulness Training Assists Individuals with Moderate Mental Retardation to Maintain Their Community Placements

ERIC Educational Resources Information Center

Singh, Nirbhay N.; Lancioni, Giulio E.; Winton, Alan S. W.; Adkins, Angela D.; Singh, Judy; Singh, Ashvind N.

2007-01-01

The mindfulness procedure "Meditation on the Soles of the Feet" can help individuals with mild mental retardation control aggressive behavior. In this study, our aim was to teach this mindfulness technique, using a multiple baseline design, to 3 individuals with moderate mental retardation who were at risk of losing their community placements…

The Use of Published Language Tests with the Mentally Retarded: A National Survey.

ERIC Educational Resources Information Center

Rushakoff, Gary E.; Johnson, Pamela C.

This study reports the results of a nationwide questionnaire survey of 204 speech and language clinicians from 65 facilities for mentally retarded individuals. Information was requested from clinicians about which language test they used with the mentally retarded population, why they used these tests, and how often they used them. Residential and…

Effects of Age, Gender, and Causality on Perceptions of Persons with Mental Retardation

ERIC Educational Resources Information Center

Panek, Paul E.; Jungers, Melissa K.

2008-01-01

The present study examined the effects of age, gender, and causality on the perceptions of persons with mental retardation. Participants rated individuals with mental retardation using a semantic differential scale with three factors: activity, evaluation, and potency. Target individuals in each scenario varied on the variables of age (8, 20, 45),…

Stress and self-injurious behavior; hormonal and serotonergic parameters in mentally retarded subjects.

PubMed

Verhoeven, W M; Tuinier, S; van den Berg, Y W; Coppus, A M; Fekkes, D; Pepplinkhuizen, L; Thijssen, J H

1999-01-01

Self-injurious behavior (SIB) and stereotyped behavior (SB) are major challenges for professionals in the field of mental retardation. From animal experiments it has become obvious that these behavioral disturbances are not purposeless but may emerge secondary to restrictive environment and may serve de-arousing objectives. In mentally retarded subjects, several hypotheses have been formulated concerning the pathogenesis of SIB, particularly about the involvement of serotonin and beta-endorphin, which are supported by beneficial treatment effects of the opiate antagonist naltrexone and serotonin modulating compounds, respectively. The present study was designed to investigate basal levels of stress-hormonal and serotonergic parameters as well as plasma levels of amino-acids and the beta-carboline norharman in a group of 64 mentally retarded subjects with SB and/or SIB. Allocation to three different groups comprising 17 retarded controls, 26 subjects with mainly SIB and 21 subjects with mainly SB, was originally performed using the scores on the factors Irritability, Stereotypic Behaviour and Hyperactivity of the Aberrant Behavioral Checklist. Because of the overlapping nature of the behavioral parameters, subjects were subsequently divided into three maximally contrasting groups, viz. predominantly SIB, predominantly SB and retarded controls, each comprising 11 subjects. With respect to beta-endorphin, no differences were found either between both the original and maximally contrasting groups or in comparison to nonretarded controls. As compared to retarded controls, a tendency to lower values for total cortisol and cortisol binding globulin appeared to be present in the SIB group, whereas in the SB group a tendency toward higher levels of the major serotonin metabolite 5-HIAA was found. In the contrasting SB group, a trend toward decreased total cortisol level was observed as compared to the retarded control group. In addition, significantly lower values for

Of Atkins and men: deviations from clinical definitions of mental retardation in death penalty cases.

PubMed

Blume, John H; Johnson, Sheri Lynn; Seeds, Christopher

2009-01-01

Under Atkins v. Virginia, the Eighth Amendment exempts from execution individuals who meet the clinical definitions of mental retardation set forth by the American Association on Intellectual and Developmental Disabilities and the American Psychiatric Association. Both define mental retardation as significantly subaverage intellectual functioning accompanied by significant limitations in adaptive functioning, originating before the age of 18. Since Atkins, most jurisdictions have adopted definitions of mental retardation that conform to those definitions. But some states, looking often to stereotypes of persons with mental retardation, apply exclusion criteria that deviate from and are more restrictive than the accepted scientific and clinical definitions. These state deviations have the effect of excluding from Atkins's reach some individuals who plainly fall within the class it protects. This article focuses on the cases of Roger Cherry, Jeffrey Williams, Michael Stallings, and others, who represent an ever-growing number of individuals inappropriately excluded from Atkins. Left unaddressed, the state deviations discussed herein permit what Atkins does not: the death-sentencing and execution of some capital defendants who have mental retardation.

[The application of diminished criminal responsibility rating scale to mental retardation offenders].

PubMed

Guan, Wei; Cai, Wei-Xiong; Huang, Fu-Yin; Wu, Jia-Sheng

2009-10-01

To explore the application of Diminished Criminal Responsibility Rating Scale (DCRRS) to mental retardation offenders. The DCRRS was used to 121 cases of mental retardation offenders who were divided into three groups according to the degree of their diminished criminal responsibility. There were significant differences in rating score among the three groups (mild group 22.12+/-4.69, moderate group 25.50+/-5.48, major group 27.59+/-5.69), and 17 items had good correlation with the total score of the scale with the correlation coefficient from 0.289 to 0.665. Six factors were extracted by the factor analysis, and 69.392% variation could be explained. The DCRRS has rational items, its total score could show the difference among the three degree diminished criminal responsibility of mental retardation offenders.

The Role of Cognition and Adaptive Behavior in Employment of People with Mental Retardation

ERIC Educational Resources Information Center

Su, Chwen-Yng; Lin, Yueh-Hsien; Wu, Yuh-Yih; Chen, Ching-Chiang

2008-01-01

Few studies have specifically investigated the cognitive correlates of employment for persons with mental retardation. To evaluate the relationship of cognitive and adaptive functioning to work status, 56 competitively employed and 55 unemployed individuals with mental retardation underwent a comprehensive neuropsychological and adaptive…

Factors affecting social integration of noninstitutionalized mentally retarded adults.

PubMed

Reiter, S; Levi, A M

1980-07-01

The social integration of noninstitutionalized moderately and mildly mentally retarded young adults was investigated. A group of moderately and mildly retarded adults (study group) was compared with a group of borderline retarded (control group) adults on employability, behavior at work, social integration and social skills, personality, and self-concept. Findings indicated that the study group was less well integrated at work and in society than was the control group and showed lack of social skills. The retarded adults who had nonretarded friends showed better social-educational skills than did the other subjects. Findings suggest that even retarded individuals who grow up in the community need help in order to become socially independent. The existence of a special social club for retarded adults was found to fulfill the functions of a sheltered framework. Participants in the club showed more positive self-concepts; however, the club did not seem to prepare them for social integration in the general community.

Mental Retardation and the Law: A Report on Status of Current Court Cases.

ERIC Educational Resources Information Center

Friedman, Paul; Beck, Ronna Lee

Included in the report are summaries of four new cases and updated information on 38 cases regarding legal issues in mental retardation. Featured is a review of cases dealing with liability of judges and lawyers in violating the rights of mentally retarded persons. Other issues addressed (with sample court case in parentheses) include commitment…

Prevalence of Eating Disorders in Adults With Mental Retardation Living in the Community

ERIC Educational Resources Information Center

Hove, Oddbjorn

2004-01-01

The prevalence of eating disorders among 311 adults with mental retardation living in the West Coast of Norway was investigated. Reports stemming from a questionnaire completed by health workers were the data source. Diagnostic criteria adapted for persons with mental retardation were used. The main finding was that 27% of cases showed indices of…

Improving Outcomes for Workers with Mental Retardation

ERIC Educational Resources Information Center

Fornes, Sandra; Rocco, Tonette S.; Rosenberg, Howard

2008-01-01

This research presents an analysis of factors predicting job retention, job satisfaction, and job performance of workers with mental retardation. The findings highlight self-determination as a critical skill in predicting the three important employee outcomes. The study examined a hypothesized job retention model and the outcome of the three…

Genetic Issues in Mental Retardation, 1996-1997.

ERIC Educational Resources Information Center

Genetic Issues in Mental Retardation, 1996

1996-01-01

This document consists of the first six issues of a newsletter, which discusses current knowledge about and concerns related to genetics and mental retardation. The second issue addresses the problem of genetic discrimination. The third issue considers genetic testing, screening, and counseling. The fourth issue addresses genetic privacy issues.…

Euthanasia and Mental Retardation: Suggesting the Unthinkable.

ERIC Educational Resources Information Center

Hollander, Russell

1989-01-01

The article examines current opinions toward euthanasia of persons with mental retardation in light of the history of public and professional attitudes. It also discusses the rejection of euthanasia on moral and religious grounds, and notes the use of lifelong incarceration, based on eugenics principles, to accomplish similar ends. (DB)

Computer Assisted Instruction for the Mentally Retarded.

ERIC Educational Resources Information Center

Providence Coll., RI.

Computer Assisted Instruction (CAI) for the mentally retarded is described; the advantages of CAI (which generally follows the pattern of programed instruction) are listed; and the roles of the teacher and the student are summarized. The coursewriter is explained, and its use as an experimental tool discussed. Guidelines are given covering…

Hypothesis: A Role for Fragile X Mental Retardation Protein in Mediating and Relieving MicroRNA-Guided Translational Repression?

PubMed Central

Plante, Isabelle; Provost, Patrick

2006-01-01

MicroRNA (miRNA)-guided messenger RNA (mRNA) translational repression is believed to be mediated by effector miRNA-containing ribonucleoprotein (miRNP) complexes harboring fragile X mental retardation protein (FMRP). Recent studies documented the nucleic acid chaperone properties of FMRP and characterized its role and importance in RNA silencing in mammalian cells. We propose a model in which FMRP could facilitate miRNA assembly on target mRNAs in a process involving recognition of G quartet structures. Functioning within a duplex miRNP, FMRP may also mediate mRNA targeting through a strand exchange mechanism, in which the miRNA* of the duplex is swapped for the mRNA. Furthermore, FMRP may contribute to the relief of miRNA-guided mRNA repression through a reverse strand exchange reaction, possibly initiated by a specific cellular signal, that would liberate the mRNA for translation. Suboptimal utilization of miRNAs may thus account for some of the molecular defects in patients with the fragile X syndrome. PMID:17057359

Temporal requirements of the fragile X mental retardation protein in the regulation of synaptic structure.

PubMed

Gatto, Cheryl L; Broadie, Kendal

2008-08-01

Fragile X syndrome (FraX), caused by the loss-of-function of one gene (FMR1), is the most common inherited form of both mental retardation and autism spectrum disorders. The FMR1 product (FMRP) is an mRNA-binding translation regulator that mediates activity-dependent control of synaptic structure and function. To develop any FraX intervention strategy, it is essential to define when and where FMRP loss causes the manifestation of synaptic defects, and whether the reintroduction of FMRP can restore normal synapse properties. In the Drosophila FraX model, dFMRP loss causes neuromuscular junction (NMJ) synapse over-elaboration (overgrowth, overbranching, excess synaptic boutons), accumulation of development-arrested satellite boutons, and altered neurotransmission. We used the Gene-Switch method to conditionally drive dFMRP expression to define the spatiotemporal requirements in synaptic mechanisms. Constitutive induction of targeted neuronal dFMRP at wild-type levels rescues all synaptic architectural defects in Drosophila Fmr1 (dfmr1)-null mutants, demonstrating a presynaptic requirement for synapse structuring. By contrast, presynaptic dFMRP expression does not ameliorate functional neurotransmission defects, indicating a postsynaptic dFMRP requirement. Strikingly, targeted early induction of dFMRP effects nearly complete rescue of synaptic structure defects, showing a primarily early-development role. In addition, acute dFMRP expression at maturity partially alleviates dfmr1-null defects, although rescue is not as complete as either early or constitutive dFMRP expression, showing a modest capacity for late-stage structural plasticity. We conclude that dFMRP predominantly acts early in synaptogenesis to modulate architecture, but that late dFMRP introduction at maturity can weakly compensate for early absence of dFMRP function.

Slogans and Euphemisms: The Functions of Semantics in Mental Health and Mental Retardation Care.

ERIC Educational Resources Information Center

Bachrach, Leona L.

The paper examines the way in which semantics relates to policies of deinstitutionalization of persons with mental illness and mental retardation. Different understandings of common terms in the field are illustrated and it is suggested that the government plays many games, both number games and word games, in dealing with homeless people, migrant…

Facial Emotion Recognition by Persons with Mental Retardation: A Review of the Experimental Literature.

ERIC Educational Resources Information Center

Rojahn, Johannes; And Others

1995-01-01

This literature review discusses 21 studies on facial emotion recognition by persons with mental retardation in terms of methodological characteristics, stimulus material, salient variables and their relation to recognition tasks, and emotion recognition deficits in mental retardation. A table provides comparative data on all 21 studies. (DB)

DSM-III Diagnoses Compared with Factor Structure of the Psychopathology Instrument for Mentally Retarded Adults (PIMRA), in an Institutionalized, Mostly Severely Retarded Population.

ERIC Educational Resources Information Center

Linaker, Olav

1991-01-01

The Psychopathology Instrument for Mentally Retarded Adults was used to diagnose 163 mentally retarded institutionalized adults according to the Diagnostic and Statistical Manual-III axis 1 categories. Nine factors were extracted which contained 49.3 percent of the data variance and categorized correctly 69.3 percent of the cases. Factors included…

Autosomal-recessive and X-linked forms of hereditary motor and sensory neuropathy in childhood.

PubMed

Ouvrier, Robert; Geevasingha, Nimeshan; Ryan, Monique M

2007-08-01

The hereditary motor and sensory neuropathies (HMSNs, Charcot-Marie-Tooth neuropathies) are the most common degenerative disorders of the peripheral nervous system. In recent years a dramatic expansion has occurred in our understanding of the molecular basis and cell biology of the recessively inherited demyelinating and axonal neuropathies, with delineation of a number of new neuropathies. Mutations in some genes cause a wide variety of clinical, neurophysiologic, and pathologic phenotypes, rendering diagnosis difficult. The X-linked forms of HMSN represent at least 10%-15% of all HMSNs and have an expanded disease spectrum including demyelinating, intermediate, and axonal neuropathies, transient central nervous system (CNS) dysfunction, mental retardation, and hearing loss. This review presents an overview of the recessive and X-linked forms of HMSN observed in childhood, with particular reference to disease phenotype and neurophysiologic and pathologic abnormalities suggestive of specific diagnoses. These findings can be used by the clinician to formulate a differential diagnosis and guide targeted genetic testing.

Physical fitness and adults with mental retardation. An overview of current research and future directions.

PubMed

Pitetti, K H; Rimmer, J H; Fernhall, B

1993-07-01

The deinstitutionalization movement of the past 25 years has focused on the placement of people with mental retardation into community-based settings. There is a need for exercise- and health-related professionals to demonstrate a thorough understanding of the term mental retardation and all of the intellectual and behavioural ramifications that coexist with this condition before addressing the 'how to' of fitness evaluation. Therefore, the article outlines the range of intellectual and behavioural characteristics of this population, based on the level of retardation. Many researchers investigating body composition have reported that a disproportionate number of adults with mental retardation carry a percentage of body fat that would be considered unhealthy (e.g. it increases the risk of early onset of such diseases as hypertension and adult onset diabetes mellitus). Living arrangements (i.e. institution vs smaller residences) play a role in the prevalence of obesity. Many attempts of researchers to control weight in adults with mental retardation through caloric restriction, exercise, and a combination of diet and exercise, have had a varied outcome. Cardiovascular capacity is considered by most exercise physiologists as the major physiological indicator for overall fitness. The majority of researchers who have evaluated the cardiovascular fitness levels of adults with mental retardation have reported fitness levels representative of a very sedentary population. Therefore, one would expect a keen sense of urgency among researchers to develop training regimens targeted specifically for people with mental retardation. Many have been developed, but to date only 2 cardiovascular training regimens have been reported that specifically describe the necessary components of an exercise programme (i.e. frequency, duration, intensity) that would allow for reproducibility--a stationary bicycle routine using the Schwinn 'Air-Dyne' ergometer and a run/walk programme. Of these

Short- and long-term memory are modulated by multiple isoforms of the fragile X mental retardation protein.

PubMed

Banerjee, Paromita; Schoenfeld, Brian P; Bell, Aaron J; Choi, Catherine H; Bradley, Michael P; Hinchey, Paul; Kollaros, Maria; Park, Jae H; McBride, Sean M J; Dockendorff, Thomas C

2010-05-12

The diversity of protein isoforms arising from alternative splicing is thought to modulate fine-tuning of synaptic plasticity. Fragile X mental retardation protein (FMRP), a neuronal RNA binding protein, exists in isoforms as a result of alternative splicing, but the contribution of these isoforms to neural plasticity are not well understood. We show that two isoforms of Drosophila melanogaster FMRP (dFMR1) have differential roles in mediating neural development and behavior functions conferred by the dfmr1 gene. These isoforms differ in the presence of a protein interaction module that is related to prion domains and is functionally conserved between FMRPs. Expression of both isoforms is necessary for optimal performance in tests of short- and long-term memory of courtship training. The presence or absence of the protein interaction domain may govern the types of ribonucleoprotein (RNP) complexes dFMR1 assembles into, with different RNPs regulating gene expression in a manner necessary for establishing distinct phases of memory formation.

A Study on Characteristics and Competencies Needed by Teachers of the Mentally Retarded.

ERIC Educational Resources Information Center

Ho, Hua-Kuo

This study assessed the characteristics of 134 teachers of the mentally retarded in Taiwan, and surveyed 321 teachers of the retarded concerning desirable teacher characteristics and competencies. Four main conclusions were drawn: (1) outstanding teachers of the retarded were highly rated for such characteristics as "flexibility of…

Activational Peaking in Educable and Trainable Mentally Retarded Persons

ERIC Educational Resources Information Center

Gargiulo, Richard M.; Uno, Tad

1977-01-01

A study involving 10 educable and 10 trainable mentally retarded adolescents indicated that levels of intellectual functioning influenced patterns of autonomic activation as measured by magnitude of the galvanic skin response. (CL)

Preparing Teachers of Pupils with Mental Retardation: Changes in Course Offerings from 1970-1990.

ERIC Educational Resources Information Center

Brusca, Rita M.; Montemurro, Theodore J.

1994-01-01

Comparison of typical courses of study from 1970 to 1990 for students preparing to be teachers of pupils with mental retardation found little change in percentage of programs offering a special course on mental retardation; increased percentage of programs offering cross-categorical courses; and decreased percentage of programs requiring joint…

Awareness among Family Members Having Children with Mental Retardation on Relevant Legislations in India

ERIC Educational Resources Information Center

Kuppusamy, Balabaskar; Narayan, Jayanthi; Nair, Deepa

2012-01-01

In India, Acts and legislations are in place for persons with mental retardation. Three major Acts namely Rehabilitation Council of India Act (1992), Persons with Disabilities (Equal opportunities, protection of rights and full participation) Act (1995) and National Trust for welfare of persons with Autism, Cerebral Palsy, Mental retardation and…

Prevalence of Intestinal Parasite Infections among Individuals with Mental Retardation in New York State.

ERIC Educational Resources Information Center

Schupf, Nicole; And Others

1995-01-01

Prevalence of intestinal parasite infection among program participants of the New York State Office of Mental Retardation and Developmental Disabilities for 1986-87 was estimated at 7.3%, suggesting that management of parasitic infection is improving. Males and individuals with severe/profound mental retardation were twice as likely to have…

Measuring Interpersonal Problems in People with Mental Retardation

ERIC Educational Resources Information Center

Kellett, Stephen; Beail, Nigel; Newman, David W.

2005-01-01

Despite interpersonal problems being commonplace in the clinical presentations of people with mental retardation, previous efforts to index interpersonal difficulties have tended to unsatisfactorily rely on external ratings. The Inventory of Interpersonal Problems-32 is a psychometrically robust self-report measure of interpersonal problems in…

The Croydon Assessment of Learning Study: Prevalence and Educational Identification of Mild Mental Retardation

ERIC Educational Resources Information Center

Simonoff, Emily; Pickles, Andrew; Chadwick, Oliver; Gringras, Paul; Wood, Nicky; Higgins, Siobhan; Maney, Julie-Ann; Karia, Nisha; Iqbal, Huma; Moore, Anne

2006-01-01

Background: Mild mental retardation is an enduring and impairing condition. Its prevalence has varied widely across different studies from 0.5 to over 8%, with higher rates in completely ascertained samples. The current study estimates the prevalence of low IQ in the mental retardation range (intellectual disability) in a population sample and…

Allocation of Attention and Effect of Practice on Persons with and without Mental Retardation

ERIC Educational Resources Information Center

Oka, Kohei; Miura, Toshiaki

2008-01-01

Persons with mild and moderate mental retardation and CA-matched persons without mental retardation performed a dual-task, "pencil-and-paper task" (Baddeley, Della Sala, Gray, Papagno, & Spinnler (1997). Testing central executive functioning with a pencil-and-paper test. In Rabbit (Ed.), Methodology of Frontal and Executive Function (pp. 61-80).…

Genotype-phenotype variations in five Spanish families with Norrie disease or X-linked FEVR.

PubMed

Riveiro-Alvarez, Rosa; Trujillo-Tiebas, Maria José; Gimenez-Pardo, Ascension; Garcia-Hoyos, Maria; Cantalapiedra, Diego; Lorda-Sanchez, Isabel; Rodriguez de Alba, Marta; Ramos, Carmen; Ayuso, Carmen

2005-09-02

Norrie disease (OMIM 310600) is a rare X-linked disorder characterized by congenital blindness in males. Approximately 40 to 50% of the cases develop deafness and mental retardation. X-linked familial exudative vitreoretinopathy (XL-FEVR) is a hereditary ocular disorder characterized by a failure of peripheral retinal vascularization. Both X-linked disorders are due to mutations in the NDP gene, which encodes a 133 amino acid protein called Norrin, but autosomal recessive (AR) and autosomal dominant (AD) forms of FEVR have also been described. In this study, we report the molecular findings and the related phenotype in five Spanish families affected with Norrie disease or XL-FEVR due to mutations of the NDP gene. The study was conducted in 45 subjects from five Spanish families. These families were clinically diagnosed with Norrie disease or similar conditions. The three exons of the NDP gene were analyzed by automatic DNA sequencing. Haplotype analyses were also performed. Two new nonsense mutations, apart from other mutations previously described in the NDP gene, were found in those patients affected with ND or X-linked FEVR. An important genotype-phenotype variation was found in relation to the different mutations of the NDP gene. In fact, the same mutation may be responsible for different phenotypes. We speculate that there might be other molecular factors that interact in the retina with Norrin, which contribute to the resultant phenotypes.

Hybridisation-based resequencing of 17 X-linked intellectual disability genes in 135 patients reveals novel mutations in ATRX, SLC6A8 and PQBP1

PubMed Central

Jensen, Lars R; Chen, Wei; Moser, Bettina; Lipkowitz, Bettina; Schroeder, Christopher; Musante, Luciana; Tzschach, Andreas; Kalscheuer, Vera M; Meloni, Ilaria; Raynaud, Martine; van Esch, Hilde; Chelly, Jamel; de Brouwer, Arjan P M; Hackett, Anna; van der Haar, Sigrun; Henn, Wolfram; Gecz, Jozef; Riess, Olaf; Bonin, Michael; Reinhardt, Richard; Ropers, Hans-Hilger; Kuss, Andreas W

2011-01-01

X-linked intellectual disability (XLID), also known as X-linked mental retardation, is a highly genetically heterogeneous condition for which mutations in >90 different genes have been identified. In this study, we used a custom-made sequencing array based on the Affymetrix 50k platform for mutation screening in 17 known XLID genes in patients from 135 families and found eight single-nucleotide changes that were absent in controls. For four mutations affecting ATRX (p.1761M>T), PQBP1 (p.155R>X) and SLC6A8 (p.390P>L and p.477S>L), we provide evidence for a functional involvement of these changes in the aetiology of intellectual disability. PMID:21267006

X-linked recessive panhypopituitarism associated with a regional duplication in Xq25-q26.

PubMed Central

Lagerström-Fermér, M; Sundvall, M; Johnsen, E; Warne, G L; Forrest, S M; Zajac, J D; Rickards, A; Ravine, D; Landegren, U; Pettersson, U

1997-01-01

We present a linkage analysis and a clinical update on a previously reported family with X-linked recessive panhypopituitarism, now in its fourth generation. Affected members exhibit variable degrees of hypopituitarism and mental retardation. The markers DXS737 and DXS1187 in the q25-q26 region of the X chromosome showed evidence for linkage with a peak LOD score (Zmax) of 4.12 at zero recombination fraction (theta(max) = 0). An apparent extra copy of the marker DXS102, observed in the region of the disease gene in affected males and heterozygous carrier females, suggests that a segment including this marker is duplicated. The gene causing this disorder appears to code for a dosage-sensitive protein central to development of the pituitary. Images Figure 2 PMID:9106538

Correlates of Work Adjustment among the Mentally Retarded.

ERIC Educational Resources Information Center

Ho, Hua-Kuo

The study sought to determine the relationships between work adjustment and selected personal, educational, vocational, and familial variables among 116 mentally retarded trainees from two vocational training programs in Taiwan. Findings included, among others: older trainees were more likely to accept rules/authority than younger workers; males…

Administrative Issues in Institutions for the Mentally Retarded.

ERIC Educational Resources Information Center

Cleland, Charles C.; Swartz, Jon D.

Designed primarily for administrators of both public and private institutions for the mentally retarded, the volume offers guidelines for coping with three areas of modification of institutional image, daily operational problems concerning manpower and equipment, and future demands upon institutions. Brief exercises following some of the readings…

Mental Retardation: Topics of Today--Issues of Tomorrow.

ERIC Educational Resources Information Center

Meyen, Edward L., Ed.

1984-01-01

The monograph examines issues affecting the future of handicapped people generally, and mentally retarded people specifically. E. Meyen introduces the volume and describes how the issues were identified. H. Turnbull and P. Barber, in "Perspectives on Public Policy," discuss such issues as defederalization, redefinition of handicaps, and…

Fetishistic Transvestism in a Patient with Mental Retardation and Psychosis

PubMed Central

Velayudhan, Rajmohan; Khaleel, Asfia; Sankar, Nideesh; Kumar, Manoj; Kazhungil, Firoz; Raghuram, Thazhe Mangool

2014-01-01

Fetishistic transvestism is a disorder of sexual preference associated with fantasies and sexual urges to dress in opposite gender clothing as a means of arousal and as an adjunct to masturbation and coitus. The disorder has been reported in people with learning disabilities. The disorder has been reported in a young male with dull normal intelligence. Transvestism though has been described in schizophrenia and psychosis and fetishism has been described in the course of simple schizophrenia, there are no reports of fetishistic transvestism in a patient with mental retardation and psychosis. A case of fetishistic transvestism in a patient with mental retardation and psychosis with treatment and relevant review of literature is reported. PMID:24860226

Fetishistic transvestism in a patient with mental retardation and psychosis.

PubMed

Velayudhan, Rajmohan; Khaleel, Asfia; Sankar, Nideesh; Kumar, Manoj; Kazhungil, Firoz; Raghuram, Thazhe Mangool

2014-04-01

Fetishistic transvestism is a disorder of sexual preference associated with fantasies and sexual urges to dress in opposite gender clothing as a means of arousal and as an adjunct to masturbation and coitus. The disorder has been reported in people with learning disabilities. The disorder has been reported in a young male with dull normal intelligence. Transvestism though has been described in schizophrenia and psychosis and fetishism has been described in the course of simple schizophrenia, there are no reports of fetishistic transvestism in a patient with mental retardation and psychosis. A case of fetishistic transvestism in a patient with mental retardation and psychosis with treatment and relevant review of literature is reported.

A Plea for Consideration of Ecological Validity in the Experimental Psychology of Mental Retardation: A Guest Editorial

ERIC Educational Resources Information Center

Brooks, Penelope H.; Baumeister, Alfred A.

1977-01-01

The authors contend that the experimental psychology of mental retardation suffers from methatheoretical and methodological weaknesses, preeminently the failure to consider the ecology of mental retardation. (CL)

Walking Habits of Adults with Mental Retardation

ERIC Educational Resources Information Center

Stanish, Heidi I.; Draheim, Christopher C.

2005-01-01

The walking activity of men and women with mental retardation residing in community settings was described. Participants were 38 women (M age = 0.7, SD = 9.5) and 65 men (M age = 35.9, SD = 11.2). They wore pedometers for 7 days. A 2 ? 2 factorial ANOVA indicated no significant gender differences in total step counts or between participants with…

Aerobic Dance and the Mentally Retarded--A Winning Combination.

ERIC Educational Resources Information Center

Barton, Bonnie J.

1982-01-01

The results of a study on an experimental dance program for mentally retarded children show that these children can improve in physical fitness and that success through physical activities can enhance their generally poor self-concept. (JN)

Mental Retardation: Determining Eligibility for Social Security Benefits.

ERIC Educational Resources Information Center

Reschly, Daniel J., Ed.; Myers, Tracy G., Ed.; Hartel, Christine R., Ed.

The Social Security Administration (SSA) provides income support and medical benefits for adults with mental retardation unable to perform substantial gainful activity through the Disability Insurance (DI) program and the Supplemental Security Income (SSI) program. SSI benefits are also provided to families of children and adolescents who evidence…

Health Care Needs of Adults with Mental Retardation.

ERIC Educational Resources Information Center

Rubin, I. Leslie

1987-01-01

Experience with provision of optimal health services to mentally retarded individuals in institutional settings can be applied and adapted to design services for such adults living in the community. Coordination of health care by a team of nurses, primary care physicians, and special medical services is recommended. (Author/DB)

Mental Retardation FY 1983. Special Report to Congress.

ERIC Educational Resources Information Center

National Inst. of Child Health and Human Development (NIH), Bethesda, MD.

The paper reviews mental retardation research activities of the National Institute of Child Health and Human Development (NICHHD) and the National Institute of Neurological and Communicative Disorders and Stroke (NINCDS). Research activities are cited to illustrate the scope of NICHHD's efforts in a variety of biomedial areas. Activities are…

Sexual Abuse of the Mentally Retarded Patient: Medical and Legal Analysis for the Primary Care Physician

PubMed Central

Morano, Jamie P.

2001-01-01

The primary care physician has a vital role in documenting and preventing sexual abuse among the mentally retarded populations in our community. Since the current national trend is to integrate citizens with mental retardation into the community away from institutionalized care, it is essential that all physicians have a basic understanding of the unique medical and legal ramifications of their clinical diagnoses. As the legal arena is currently revising laws concerning rights of sexual consent among the mentally retarded, it is essential that determinations of mental competency follow national standards in order to delineate clearly any instance of sexual abuse. Clinical documentation of sexual abuse and sexually transmitted disease is an important part of a routine examination since many such individuals are indeed sexually active. Legal codes adjudicating sexual abuse cases of the mentally retarded often offer scant protection and vague terminology. Thus, medical documentation and physician competency rulings form a solid foundation for future work toward legal recourse for the abused. PMID:15014610

Consumer Roulette: The Shopping Patterns of Mentally Retarded Persons.

ERIC Educational Resources Information Center

Williams, Randall D.; Ewing, Sheryl

1981-01-01

To develop information regarding the commercial product purchasing behavior of mildly mentally retarded adults, 11 participants were included in a mock shopping exercise. The results indicated several serious deficiencies in the purchasing strategies of the participants. (Author/SB)

Clinical Comparison of Haloperidol with Chlorpromazine in Mentally Retarded Children

ERIC Educational Resources Information Center

LeVann, Leonard J.

1971-01-01

In an 8-week double-blind comparison, haloperidol reduced the severity of the target symptoms impulsiveness, hostility, and aggressiveness in significantly more mentally retarded children than did chlorpromazine. (Author)

Teaching Moderately Mentally Retarded Children Basic Reading Skills.

ERIC Educational Resources Information Center

Hoogeveen, Frans R.; And Others

1989-01-01

Four moderately mentally retarded students, aged 8-13, were instructed in a basic skills reading program which emphasized a phonemic alphabet, pictorial cueing, and stimulus manipulation techniques. The training improved the Dutch students' ability to read one- and two-syllable words, and was generalizable to untrained words of the same…

Horticultural Careers for Persons with Mental Retardation. Expanding Opportunities.

ERIC Educational Resources Information Center

Dehart-Bennett, Mary E.; Relf, Diane

1990-01-01

Horticulture careers provide therapeutic, rewarding employment for persons with mental retardation. Rehabilitation experts should become aware of the potential employment opportunities in horticulture so that individuals with disabilities can receive the training and job placement support they need. (Author)

Ex-post-facto analysis of competitive employment outcomes for individuals with mental retardation: national perspective.

PubMed

Moore, Corey L; Harley, Debra A; Gamble, David

2004-08-01

Disparities in proportions of competitive job placements and provision of vocational rehabilitation services by level of mental retardation were identified for 28,565 individuals. Chi-square results reveal that consumers with mild mental retardation are significantly more likely to achieve competitive jobs compared to those with more severe levels. Logistic regression indicated that the odds of achieving a competitive job for consumers receiving job placement services, business/vocational training, and counseling were nearly two times that of individuals not receiving such services. Findings suggest that a significantly lower proportion of these services were provided to consumers with severe/profound mental retardation. Implications of findings for service, research, and policy are discussed.

Interference and Inhibition in Tasks of Selective Attention by Persons with and without Mental Retardation

ERIC Educational Resources Information Center

Merrill, Edward C.

2006-01-01

Persons with mental retardation often exhibit greater interference in visual selective attention tasks than do persons matched with them on CA. My goal here was to evaluate whether differences in distractor interference between persons with and without mental retardation may be related to differences in negative priming. Fifteen participants with…

Prevalence of Obesity in Adults with Mental Retardation: Implications for Health Promotion and Disease Prevention.

ERIC Educational Resources Information Center

Rimmer, James H.; And Others

1993-01-01

Measurement of obesity among 364 adults with mental retardation indicated that rates of obesity were significantly higher among females than males; subjects with severe mental retardation had the lowest overall rates of obesity; and residents of a state-operated institution had lower obesity rates than group home, family, and Intermediate Care…

Fire Safety Training with Adults Who Are Profoundly Mentally Retarded.

ERIC Educational Resources Information Center

Rae, Rosamond; Roll, David

1985-01-01

An intensive fire safety training program for profoundly mentally retarded institutionalized persons resulted in a significant decrease in mean evacuation time and gradual substitution of verbal for physical prompts. (CL)

Physiological Arousal in Autism and Fragile X Syndrome: Group Comparisons and Links With Pragmatic Language

PubMed Central

Klusek, Jessica; Martin, Gary E.; Losh, Molly

2014-01-01

This study tested the hypothesis that pragmatic (i.e., social) language impairment is linked to arousal dysregulation in autism spectrum disorder (ASD) and fragile X syndrome (FXS). Forty boys with ASD, 39 with FXS, and 28 with typical development (TD), aged 4–15 years, participated. Boys with FXS were hyperaroused compared to boys with TD but did not differ from boys with ASD. Dampened vagal tone predicted pragmatic impairment in ASD, and associations emerged between cardiac activity and receptive/expressive vocabulary across groups. Findings support autonomic dysfunction as a mechanism underlying pragmatic impairment in ASD and suggest that biophysiological profiles are shared in ASD and FXS, which has implications for understanding the role of fragile X mental retardation-1 (FMR1, the FXS gene) in the pathophysiology of ASD. PMID:24432860

New domains of neural cell-adhesion molecule L1 implicated in X-linked hydrocephalus and MASA syndrome

DOE Office of Scientific and Technical Information (OSTI.GOV)

Jouet, M.; Kenwick, S.; Moncla, A.

1995-06-01

The neural cell-adhesion molecule L1 is involved in intercellular recognition and neuronal migration in the CNS. Recently, we have shown that mutations in the gene encoding L1 are responsible for three related disorders; X-linked hydrocephalus, MASA (mental retardation, aphasia, shuffling gait, and adducted thumbs) syndrome, and spastic paraplegia type I (SPG1). These three disorders represent a clinical spectrum that varies not only between families but sometimes also within families. To date, 14 independent L1 mutations have been reported and shown to be disease causing. Here we report nine novel L1 mutations in X-linked hydrocephalus and MASA-syndrome families, including the firstmore » examples of mutations affecting the fibronectin type III domains of the molecule. They are discussed in relation both to phenotypes and to the insights that they provide into L1 function. 39 refs., 5 figs., 3 tabs.« less

Metabotropic glutamate receptor 5 upregulation in children with autism is associated with underexpression of both Fragile X mental retardation protein and GABAA receptor beta 3 in adults with autism

PubMed Central

Fatemi, S. Hossein; Folsom, Timothy D.; Kneeland, Rachel E.; Liesch, Stephanie B.

2011-01-01

Recent work has demonstrated the impact of dysfunction of the GABAergic signaling system in brain and the resultant behavioral pathologies in subjects with autism. In animal models, altered expression of Fragile X mental retardation protein (FMRP) has been linked to downregulation of GABA receptors. Interestingly, the autistic phenotype is also observed in individuals with Fragile X syndrome. This study was undertaken to test previous theories relating abnormalities in levels of FMRP to GABAA receptor underexpression. We observed a significant reduction in levels of FMRP in the vermis of adults with autism. Additionally, we found that levels of metabotropic glutamate receptor 5 (mGluR5) protein were significantly increased in vermis of children with autism vs. age and postmortem interval (PMI) matched controls. There was also a significant decrease in level of GABAA receptor beta 3 (GABRβ3) protein in vermis of adult subjects with autism. Finally, we found significant increases in glial fibrillary acidic protein (GFAP) in vermis of both children and adults with autism when compared with controls. Taken together, our results provide further evidence that altered FMRP expression and increased mGluR5 protein production potentially leads to altered expression of GABAA receptors. PMID:21901840

BCOR analysis in patients with OFCD and Lenz microphthalmia syndromes, mental retardation with ocular anomalies, and cardiac laterality defects

PubMed Central

Hilton, Emma; Johnston, Jennifer; Whalen, Sandra; Okamoto, Nobuhiko; Hatsukawa, Yoshikazu; Nishio, Juntaro; Kohara, Hiroshi; Hirano, Yoshiko; Mizuno, Seiji; Torii, Chiharu; Kosaki, Kenjiro; Manouvrier, Sylvie; Boute, Odile; Perveen, Rahat; Law, Caroline; Moore, Anthony; Fitzpatrick, David; Lemke, Johannes; Fellmann, Florence; Debray, François-Guillaume; Dastot-Le-Moal, Florence; Gerard, Marion; Martin, Josiane; Bitoun, Pierre; Goossens, Michel; Verloes, Alain; Schinzel, Albert; Bartholdi, Deborah; Bardakjian, Tanya; Hay, Beverly; Jenny, Kim; Johnston, Kathreen; Lyons, Michael; Belmont, John W; Biesecker, Leslie G; Giurgea, Irina; Black, Graeme

2009-01-01

Oculofaciocardiodental (OFCD) and Lenz microphthalmia syndromes form part of a spectrum of X-linked microphthalmia disorders characterized by ocular, dental, cardiac and skeletal anomalies and mental retardation. The two syndromes are allelic, caused by mutations in the BCL-6 corepressor gene (BCOR). To extend the series of phenotypes associated with pathogenic mutations in BCOR, we sequenced the BCOR gene in patients with (1) OFCD syndrome, (2) putative X-linked (‘Lenz') microphthalmia syndrome, (3) isolated ocular defects and (4) laterality phenotypes. We present a new cohort of females with OFCD syndrome and null mutations in BCOR, supporting the hypothesis that BCOR is the sole molecular cause of this syndrome. We identify for the first time mosaic BCOR mutations in two females with OFCD syndrome and one apparently asymptomatic female. We present a female diagnosed with isolated ocular defects and identify minor features of OFCD syndrome, suggesting that OFCD syndrome may be mild and underdiagnosed. We have sequenced a cohort of males diagnosed with putative X-linked microphthalmia and found a mutation, p.P85L, in a single case, suggesting that BCOR mutations are not a major cause of X-linked microphthalmia in males. The absence of BCOR mutations in a panel of patients with non-specific laterality defects suggests that mutations in BCOR are not a major cause of isolated heart and laterality defects. Phenotypic analysis of OFCD and Lenz microphthalmia syndromes shows that in addition to the standard diagnostic criteria of congenital cataract, microphthalmia and radiculomegaly, patients should be examined for skeletal defects, particularly radioulnar synostosis, and cardiac/laterality defects. PMID:19367324

South Asian Indian Cultural Orientations toward Mental Retardation

ERIC Educational Resources Information Center

Gabel, Susan

2004-01-01

The findings of a portion of a 2-year ethnographic study involving North Indian Hindu immigrants living in the mid-Western United States is discussed. These findings illuminate the ways in which participants think and talk about mental retardation, how this linguistic information was obtained, and the cultural context within which participants…

Mainstreaming: Educable Mentally Retarded Children in Regular Classes.

ERIC Educational Resources Information Center

Birch, Jack W.

Described in the monograph are mainstreaming programs for educable mentally retarded (EMR) children in six variously sized school districts within five states. It is noted that mainstreaming is based on the principle of educating most children in the regular classroom and providing special education on the basis of learning needs rather than…

Neglected and Abused Children of Mentally Retarded Parents.

ERIC Educational Resources Information Center

Seagull, Elizabeth A. W.; Scheurer, Susan L.

1986-01-01

Follow-up of 64 neglected and abused children, seen one to seven years earlier, with mentally retarded parent revealed that six children had been relinquished voluntarily for adoption; that courts had terminated parental rights for 34 children; and that nine had been placed in foster care. Cognitive limitations prevented parents from utilizing…

Representation of international authorship across prominent journals in the field of mental retardation.

PubMed

Matson, Michael L; Matso, Johnny L; Lott, Julia D; Logan, James R

2002-01-01

This article is a reflection of international publication trends across prominent journals in the field of mental retardation. Journals reviewed were the Journal of Applied Behavior Analysis, Journal of Intellectual Disability Research, Mental Retardation, and Research in Developmental Disabilities. Percentage of authors from the United States (US) and other countries were evaluated. US authors represented 84-91% in US journals, while US representation in foreign journals was much lower at 21%. The implications of these findings are discussed.

The New Jersey Comprehensive Plan to Combat Mental Retardation.

ERIC Educational Resources Information Center

New Jersey State Interdepartmental Committee on Lifetime Disability, Trenton.

Recommendations to combat mental retardation in New Jersey are made in the following areas: coordination; research; prevention, detection, and treatment; clinical, social, recreation, and religious services; residential care; education; vocational rehabilitation; law; manpower and training; and public awareness. Additional information is given…

Support Systems of Mothers of Mentally Retarded Children.

ERIC Educational Resources Information Center

Chinkanda, E. N.

A study was conducted with a sample of 80 black mothers of mildly to profoundly mentally retarded children in the Pretoria, South Africa, townships of Atteridgeville and Mamelodi. The study sought to identify support systems utilized by the mothers, identify latent systems that could be supportive to the mothers, ascertain the extent to which…

A Helpful Guide in the Training of a Mentally Retarded Child.

ERIC Educational Resources Information Center

Blanton, Elsie

The guide offers numerous practical suggestions for training mentally retarded children in self care skills, and catalogs developmental characteristics of children from birth to a mental age of 11 years. Included are brief sections on the management of neurologically impaired children and mongoloid babies, the importance of speech stimulation, and…

References to people in the communications of female and male youths with mental retardation.

PubMed

Wilkinson, K M; Murphy, N A

1998-01-01

Gender-related differences have consistently been reported in the language of adults and children with no disabilities. One well-replicated finding is that females discuss people and relationships more often than do males, particularly in conversations with other females. These stylistic variations in language are considered to have implications for the adaptive functioning of language users, most particularly females. Although studied thus far only in nondisabled individuals, such issues of language style use may be of equal or greater concern for those with mental retardation. How does a cognitive impairment intensify or reduce gender-linked language styles and their effects? Language transcripts were obtained from eight male and eight female participants with retardation, interacting separately with one male and one female adult partner. Half of the participants used speech as their primary mode of communication: the others relied on vocalization, gesture, or augmented modes. Participants using speech showed gender-linked language patterns similar to nondisabled individuals, with females discussing people significantly more often than males. Females using nonspeech modes, in contrast, showed a severe reduction in person-referencing that was not accountable by their expressive speech limitations.

Aetiologic spectrum of mental retardation & developmental delay in India

PubMed Central

Aggarwal, Shagun; Bogula, Vijay Raju; Mandal, Kausik; Kumar, Rashmi; Phadke, Shubha R.

2012-01-01

Background & objectives: The aetiology of mental retardation is varied and difficult to establish. Reports from India on the spectrum of underlying causative conditions are lacking. This retrospective study was conducted to establish the various aetiologies of mental retardation (MR) and developmental delay (DD) in patients attending a medical genetics centre in north India and to assess the contribution of genetic disorders. Methods: This retrospective study was conducted at a tertiary care centre in north India. All patients attending the centre with MR or DD from January 2007 to December 2009 were included. The aetiology of MR/DD was ascertained after clinical assessment and targeted laboratory evaluation. The spectrum of causative conditions and contribution of genetic disorders was established. Results: A total of 338 patients were included in the study, of whom definite diagnosis was established in 253 (74.8%). The various aetiological categories were: chromosomal disorders in 112 (33.1%), non chromosomal syndromes in 32 (9.5%), neurometabolic disorders in 34 (10.1%), central nervous system structural defects in 25 (7.4%), cerebral palsy in 43 (12.7%) and environmental insults in 7 (2%). Eighty five patients (25.2%) had idiopathic mental retardation. A total of 196 (58%) patients had a genetic disorder as the cause of MR/DD. Interpretation & conclusions: The aetiology of MR/DD is varied and difficult to establish in a significant proportion of patients. Chromosomal and various monogenic disorders contribute to a large number of MR/DD cases and hence a genetic work up is essential for all such patients. PMID:23041737

Clinical Study of the Effects of Age on the Physical Health of Adults with Mental Retardation.

ERIC Educational Resources Information Center

Cooper, Sally-Ann

1998-01-01

Physical disorders and pharmacotherapy for 134 people with mental retardation (ages 65 years and older) living in the United Kingdom were compared to 73 younger adults with mental retardation. Results showed the older group had higher rates of urinary incontinence, immobility, hearing impairments, arthritis, hypertension, and cerebrovascular…

Progress in Mental Development of Retarded Cerebral Palsied Infants

ERIC Educational Resources Information Center

Banham, Katharine M.

1972-01-01

Reported were scores on the Cattell Infant Intelligence Scale, the Stanford Binet Intelligence Scale (Form L-M), the Quick Screening Scale of Mental Development, and the Vineland Social Maturity Scale achieved by 102 retarded, cerebral palsied infants who were in a hospital rehabilitation program. (GW)

Editorial: Trends in Mental Retardation in the 1990s.

ERIC Educational Resources Information Center

Rowitz, Louis

1989-01-01

Issues in mental retardation to be addressed in the next decade include homogenization of disabilities, aging of the population, increasing information and research needs, diagnosis, Acquired Immune Deficiency Syndrome, legal and ethical problems, caregiving, adolescent care, health care delivery, lifelong disabilities, vocational opportunities,…

Severe Mental Retardation in Children in a Northern Swedish County

ERIC Educational Resources Information Center

And Others; Gustavson, K. H.

1977-01-01

Presented are results of a study of the incidence, prevalence, gestational age, birth weight, associated central nervous system disorders, and etiological and pathogenetic aspects of 161 severely mentally retarded children in Northern Sweden. (CL)

IQ and the Death Penalty: Verifying Mental Retardation.

ERIC Educational Resources Information Center

Keyes, Denis William

Whether or not subjects can simulate mental retardation, a consideration that has implications in criminal cases, was studied using 21 adult Caucasian males between 20 and 30 years of age, largely comprised of students and staff employees of the University of New Mexico. Subjects were asked to give genuine and simulated responses to two major test…

Towards Defining Adequate Lithium Trials for Individuals with Mental Retardation and Mental Illness.

ERIC Educational Resources Information Center

Pary, Robert J.

1991-01-01

Use of lithium with mentally retarded individuals with psychiatric conditions and/or behavior disturbances is discussed. The paper describes components of an adequate clinical trial and reviews case studies and double-blind cases. The paper concludes that aggression is the best indicator for lithium use, and reviews treatment parameters and…

Visual evoked potentials of mildly mentally retarded and control children.

PubMed

Gasser, T; Pietz, J; Schellberg, D; Köhler, W

1988-10-01

Visual evoked potentials (VEPs) were recorded from 25 10- to 13-year-old mildly mentally retarded children and compared with those from 31 control children of the same age-range. Correlations of VEPs with age were weak, but a relationship between VEPs and IQ was demonstrated for the control group. The retarded group had significantly longer latencies and higher amplitude peaks than the control group, with the differences occurring primarily over non-specific cortex and for secondary components. Analysis also showed that the retarded group were neurophysiologically heterogeneous. Since the same children had been analyzed earlier by quantitative EEG methods, comparisons are made with respect to these two methods of investigating brain function.

Divorce in Families of Genetically Handicapped/Mentally Retarded Individuals.

ERIC Educational Resources Information Center

Roesel, Rosalyn; Lawlis, G. Frank

1983-01-01

Investigated divorce among parents (N=113) of mentally retarded/genetically handicapped children and found a divorce rate significantly lower than the general population. Young parents of handicapped first born males and older mothers of handicapped first borns were at a higher risk of divorce. (Author/JAC)

Issues in the Taxonomy of Psychopathology in Mental Retardation.

ERIC Educational Resources Information Center

Einfeld, Stewart L.; Aman, Michael

1995-01-01

Factors affecting the development of a taxonomy of psychopathology for children with mental retardation are considered, along with the following diagnostic areas to address: stereotypic behavior and self-injury, autism, organic brain syndromes, and pervasive developmental disorders. Using data from multivariate studies and findings related to…

PHENYLKETONURIA, AN INHERITED METABOLIC DISORDER ASSOCIATED WITH MENTAL RETARDATION.

ERIC Educational Resources Information Center

CENTERWALL, WILLARD R.; CENTERWALL, SIEGRIED A.

ADDRESSED TO PUBLIC HEALTH WORKERS AND PHYSICIANS IN GENERAL PRACTICE, THE PAMPHLET INTRODUCES METHODS OF DETECTING AND MANAGING PHENYLKETONURIA, AN INHERITED METABOLIC DISORDER ASSOCIATED WITH MENTAL RETARDATION. INFORMATION, UPDATED FROM THE 1961 EDITION, IS INCLUDED ON THE INCIDENCE AND GENETICS, BIOCHEMISTRY, AND CLINICAL COURSE OF THE…

Occupational Interests and Mentally Retarded People: Review and Recommendations.

ERIC Educational Resources Information Center

Stodden, Robert A.; And Others

1979-01-01

The article reviews several studies regarding the choice of occupational interests for the mentally retarded adolescent. Several concerns about current evaluation practices are discussed. Recommendations are offered for a client centered, developmental model, making the evaluation of occupational interests a viable part of the client's…

Large state-level fluctuations in mental retardation classifications related to introduction of renormed intelligence test.

PubMed

Scullin, Matthew H

2006-09-01

Oppositely valenced forces may be at work to influence rates of placement of children into mental retardation programs. On one hand, educational policies regarding intellectual disability and concerns about overrepresentation of minorities in special education may contribute to lower placement rates; on the other hand, more difficult intelligence test norms may be a countervailing force, increasing placement rates. An analysis of longitudinal data on state and national level placement rates reveals that a lengthy and steep 12-year decline in students receiving mental retardation services reversed shortly after the introduction of the WISC-III in 1991. This phenomenon has relevance for death-penalty cases, because this historical pattern may affect the ability to establish whether an adult meets the developmental period onset criterion for mental retardation.

The Mentally Retarded Worker: An Economic Discovery. Report to the President. 14th Annual Report.

ERIC Educational Resources Information Center

President's Committee on Mental Retardation, Washington, DC.

The booklet examines potential contributions that can be made by mentally retarded workers and exposes common misconceptions about their employability. Initial sections introduce the nature of retardation and describe real-life situations showing economic benefits of employment to employers, taxpayers, and retarded persons themselves. Among the…

A Job Retention Model for Individuals with Mental Retardation

ERIC Educational Resources Information Center

Fornes, Sandra

2006-01-01

This structured literature review examines the literature and addresses issues of job retention for adult workers with moderate to mild mental retardation (MR), investigating the relationships between work-related social behaviors, self-determination, person-job congruency of individuals with MR, and their job performance and job satisfaction with…

Cognitive Development of Severely and Profoundly Mentally Retarded Individuals.

ERIC Educational Resources Information Center

Silverstein, A. B.; And Others

1982-01-01

H. Corman and S. Escalona's scales for object permanence and spatial relationships were readministered to 71 severely and profoundly mentally retarded individuals (mean age 19 years) five years after the last previous administration of the scales. Gains in mean scores were small but statistically significant for both scales. (Author)

The New Morbidity and the Prevention of Mental Retardation.

ERIC Educational Resources Information Center

Baumeister, Alfred A.

1988-01-01

Efforts to prevent mental retardation have been encumbered by lack of scientific and technical knowledge, vague understanding of incidence and prevalence, and scarcity of resources to implement effective public policies. Scientific and social progress toward prevention has pursued a wavelike, erratic course, driven primarily by prevailing social,…

Japanese Studies on Attitudes towards Persons with Mental Retardation.

ERIC Educational Resources Information Center

Tachibana, Toshiaki; Watanabe, Kanji

2002-01-01

Review of Japanese studies on attitudes toward persons with mental retardation first notes origins in increased support for special classes in schools. Reported findings focus on items most affecting attitude differences, gender differences, effect of contact, educational differences, and age differences. A unique sociological study in a fishery…

Hepatitis B Infection in a Day School of Mentally Retarded Students: Transmission from Students to Staff.

ERIC Educational Resources Information Center

Remis, Robert S.; And Others

1987-01-01

Teaching Staff in day schools for mentally retarded students with direct classroom contact may carry an occupational risk of HBV infection. A seroepidemiologic study associated the prevalence of HBV markers with classroom contact with an HBsAg-positive student, duration of employment at school, and previous work with mentally retarded individuals.…

Equivalence relations in individuals with language limitations and mental retardation.

PubMed Central

O'Donnell, Jennifer; Saunders, Kathryn J

2003-01-01

The study of equivalence relations exhibited by individuals with mental retardation and language limitations holds the promise of providing information of both theoretical and practical significance. We reviewed the equivalence literature with this population, defined in terms of subjects having moderate, severe, or profound mental retardation. The literature includes 55 such individuals, most of whom showed positive outcomes on equivalence tests. The results to date suggest that naming skills are not necessary for positive equivalence test outcomes. Thus far, however, relatively few subjects with minimal language have been studied. Moreover, we suggest that the scientific contributions of studies in this area would be enhanced with better documentation of language skills and other subject characteristics. With recent advances in laboratory procedures for establishing the baseline performances necessary for equivalence tests, this research area is poised for rapid growth. PMID:13677612

Establishing Mental Retardation in Capital Cases: A Potential Matter of Life and Death.

ERIC Educational Resources Information Center

Baroff, George S.

1991-01-01

This paper discusses psychological test obstacles to gaining the acceptance of a diagnosis of mental retardation in criminal defendants, use of the Revised Beta intelligence test with defendants who may be retarded, possible modification of the adaptive behavior criterion for criminal defendants, and appropriateness of the death penalty for…

The Human Genome Project and Mental Retardation: An Educational Program. Final Progress Report

DOE Office of Scientific and Technical Information (OSTI.GOV)

Davis, Sharon

The Arc, a national organization on mental retardation, conducted an educational program for members, many of whom have a family member with a genetic condition causing mental retardation. The project informed members about the Human Genome scientific efforts, conducted training regarding ethical, legal and social implications and involved members in issue discussions. Short reports and fact sheets on genetic and ELSI topics were disseminated to 2,200 of the Arc's leaders across the country and to other interested individuals. Materials produced by the project can e found on the Arc's web site, TheArc.org.

Learning and behavioral deficits associated with the absence of the fragile X mental retardation protein: what a fly and mouse model can teach us

PubMed Central

Santos, Ana Rita; Kanellopoulos, Alexandros K.

2014-01-01

The Fragile X syndrome (FXS) is the most frequent form of inherited mental disability and is considered a monogenic cause of autism spectrum disorder. FXS is caused by a triplet expansion that inhibits the expression of the FMR1 gene. The gene product, the Fragile X Mental Retardation Protein (FMRP), regulates mRNA metabolism in brain and nonneuronal cells. During brain development, FMRP controls the expression of key molecules involved in receptor signaling, cytoskeleton remodeling, protein synthesis and, ultimately, spine morphology. Symptoms associated with FXS include neurodevelopmental delay, cognitive impairment, anxiety, hyperactivity, and autistic-like behavior. Twenty years ago the first Fmr1 KO mouse to study FXS was generated, and several years later other key models including the mutant Drosophila melanogaster, dFmr1, have further helped the understanding of the cellular and molecular causes behind this complex syndrome. Here, we review to which extent these biological models are affected by the absence of FMRP, pointing out the similarities with the observed human dysfunction. Additionally, we discuss several potential treatments under study in animal models that are able to partially revert some of the FXS abnormalities. PMID:25227249

The Effects of Spiritual Self-Care Training on Caregiving Strain in Mothers of Mentally Retarded Children.

PubMed

Dindar, Mitra; Rahnama, Mozhgan; Afshari, Mehdi; Moghadam, Mahdieh Poodineh

2016-12-01

Care for a mentally retarded child induces a lot of problems for the mother and leads her to care giving strain and ignorning her self-care. Spiritual health will co-ordinate all aspects of human life and is necessary for coping with diseases in mother of mentally retarded children. To evaluate the effects of spiritual self-care training on care giving strain in mothers of mentally retarded children. The present study, is a before and after type quasi-experimental research based on which 60 mothers of mentally retarded children who were hospitalized in Elahi Rehabilitation Center in Quchan City, were selected using convenience sampling and were randomly assigned to intervention and control groups. Data was collected by demographic characteristic questionnaire and care giving strain questionnaire that were filled by groups before, immediately and two weeks after spiritual self-care training. Data was analysed using SPSS version 20. According to the results, there was no significant difference between the mean score of care giving strain in intervention and control groups before and immediately after the intervention. However, among the members of the intervention group the score of mother care giving strain decreased an average of 87.21% within two weeks after the intervention, which was statistically significant over time (p=0.001). The score of mothers in the control group increased an average of 5% over time which was not statistically significant (p=0.4). The observed differences between these groups were also statistically significant even after controlling the effects of such intervening factors as marital status, children age and the years of caring for children (p=0.001). Spiritual self-care training can decrease care giving strain in mothers of mentally retarded children. Therefore, strengthening their spiritual beliefs and backgrounds, mothers can greatly reduce the strain caused by care giving problems of mentally retarded children.

Shaping with Visual Feedback and Token Reinforcement: Effects on Voice Volume Changes in Mentally Retarded Adults.

ERIC Educational Resources Information Center

Bieber, Carrie; Gurski, John C.

In an attempt to confirm earlier results with a group of mentally retarded females, 12 mentally retarded institutionalized adults (8 male, 4 female) were trained to either reduce (Loud group) or increase (Soft group) their voice volumes with a combination of visual feedback and token reinforcement. The feedback unit provided a binary light on-off…

Effects of Physical Training and Calcium Intake on Bone Mineral Density of Students with Mental Retardation

ERIC Educational Resources Information Center

Hemayattalab, Rasool

2010-01-01

The purpose of this study was to investigate the effects of physical training and calcium intake on bone mineral density (BMD) of students with mental retardation. Forty mentally retarded boys (age 7-10 years old) were randomly assigned to four groups (no differences in age, BMD, calcium intake and physical activity): training groups with or…

Learning and memory deficits consequent to reduction of the fragile X mental retardation protein result from metabotropic glutamate receptor-mediated inhibition of cAMP signaling in Drosophila.

PubMed

Kanellopoulos, Alexandros K; Semelidou, Ourania; Kotini, Andriana G; Anezaki, Maria; Skoulakis, Efthimios M C

2012-09-19

Loss of the RNA-binding fragile X protein [fragile X mental retardation protein (FMRP)] results in a spectrum of cognitive deficits, the fragile X syndrome (FXS), while aging individuals with decreased protein levels present with a subset of these symptoms and tremor. The broad range of behavioral deficits likely reflects the ubiquitous distribution and multiple functions of the protein. FMRP loss is expected to affect multiple neuronal proteins and intracellular signaling pathways, whose identity and interactions are essential in understanding and ameliorating FXS symptoms. We used heterozygous mutants and targeted RNA interference-mediated abrogation in Drosophila to uncover molecular pathways affected by FMRP reduction. We present evidence that FMRP loss results in excess metabotropic glutamate receptor (mGluR) activity, attributable at least in part to elevation of the protein in affected neurons. Using high-resolution behavioral, genetic, and biochemical analyses, we present evidence that excess mGluR upon FMRP attenuation is linked to the cAMP decrement reported in patients and models, and underlies olfactory associative learning and memory deficits. Furthermore, our data indicate positive transcriptional regulation of the fly fmr1 gene by cAMP, via protein kinase A, likely through the transcription factor CREB. Because the human Fmr1 gene also contains CREB binding sites, the interaction of mGluR excess and cAMP signaling defects we present suggests novel combinatorial pharmaceutical approaches to symptom amelioration upon FMRP attenuation.

Project Job: Vocational Training Program for Mentally Retarded Adults.

ERIC Educational Resources Information Center

Career Development for Exceptional Individuals, 1983

1983-01-01

A vocational training program for trainable mentally retarded youth and adults provided instruction on functional skills, travel training, and independent living skills in addition to supervised performance in part time jobs in the community. The program expanded into small business subsidiaries for boat cleaning and detailing and marine serving.…

Task Monotony and Performance Efficacy of Mentally Retarded Young Adults.

ERIC Educational Resources Information Center

Locke, Bill J.; And Others

1982-01-01

Thirty-six mildly mentally retarded young adults were exposed to one of three training arrangements for vigilance performance, a monitoring task that some professionals consider uniquely appropriate for such persons because they are assumed to be less susceptible to boredom. (Author)

Effect of computer game intervention on the attention capacity of mentally retarded children.

PubMed

Rezaiyan, Akram; Mohammadi, Eesa; Fallah, Parviz A

2007-10-01

The low rate of understanding and the limited domain of attention are two important and distinctive characteristics of mental retardation. One of the important strategies to provide effective education for these subjects is to lessen the backgrounds and impacts of these affective factors. The aim of this research was to find the effect of computer games program on the amount of mentally retarded persons' attention. Sixty educable male mentally retarded subjects were selected from two 24-h care centres in Tehran. The Toulouse-Pieron Scale was used to determinate the subjects' attention at pre-post test. Members of the experimental group were subjected to 35 sets of computer games. After use of the sets of computer games, the attention scores of the subjects were assessed immediately after the intervention and 5 weeks later. The results showed that exactly after the intervention the average attention scores of the experimental group were significantly higher than those of the control group. But, 5 weeks after the intervention, there was actually no significant difference.

Arts and Crafts for Use with Mentally Retarded Students.

ERIC Educational Resources Information Center

Child, Toni, Ed.

GRADES OR AGES: Mentally retarded children of any age. SUBJECT MATTER: Arts and crafts. ORGANIZATION AND PHYSICAL APPEARANCE: The guide is divided into sections according to the material to be used. Section headings are: Paper, Fabrics, Wood, Metal, Nature, Miscellaneous, and Painting. Each section is laid out in three parallel columns, one each…

Children Served in Mental Retardation Clinics: Fiscal Year 1973.

ERIC Educational Resources Information Center

Health Services Administration (DHEW/PHS), Rockville, MD. Bureau of Community Health Services.

Presented is statistical information on children served in 166 mental retardation clinics in 44 states, the District of Columbia, and Puerto Rico funded through the maternal and child health program of the U.S. Department of Health, Education, and Welfare during fiscal year 1973. Brief sections cover characteristics of children served, services…

Injury Prevalence among Children and Adolescents with Mental Retardation

ERIC Educational Resources Information Center

Slayter, Elspeth M.; Garnick, Deborah W.; Kubisiak, Joanna M.; Bishop, Christine E.; Gilden, Daniel M.; Hakim, Rosemarie B.

2006-01-01

Childhood injuries lead to increased morbidity and result in significant costs to public insurance programs. People with mental retardation, most of whom are covered by Medicaid, are at high risk for injury, which has implications for community inclusion, a central policy goal. Medicaid data from inpatient, outpatient, and long-term care settings…

Characterization of Mental Retardation and Autism in Children's Books.

ERIC Educational Resources Information Center

Dyches, Tina Taylor; Prater, Mary Anne; Cramer, Sharon F.

2001-01-01

A study involving 12 books, six picture books and six chapter books, examined how individuals with mental retardation and autism are depicted in children's literature. Results indicate characters were portrayed as making more choices in their lives and were educated in more inclusive environments than in an earlier study. (Contains references.)…

Coping Styles among Mothers of Adults with Mental Retardation.

ERIC Educational Resources Information Center

Krauss, Marty Wyngaarden

This paper describes the use of various coping strategies of mothers of adult children with mental retardation, and examines whether there is a relation between specific styles of coping and maternal characteristics and personal well-being. A group of 349 mothers completed the "Coping Orientations to Problems Experienced" scale. On average,…

Fragile X mental retardation protein has a unique, evolutionarily conserved neuronal function not shared with FXR1P or FXR2P

PubMed Central

Coffee, R. Lane; Tessier, Charles R.; Woodruff, Elvin A.; Broadie, Kendal

2010-01-01

SUMMARY Fragile X syndrome (FXS), resulting solely from the loss of function of the human fragile X mental retardation 1 (hFMR1) gene, is the most common heritable cause of mental retardation and autism disorders, with syndromic defects also in non-neuronal tissues. In addition, the human genome encodes two closely related hFMR1 paralogs: hFXR1 and hFXR2. The Drosophila genome, by contrast, encodes a single dFMR1 gene with close sequence homology to all three human genes. Drosophila that lack the dFMR1 gene (dfmr1 null mutants) recapitulate FXS-associated molecular, cellular and behavioral phenotypes, suggesting that FMR1 function has been conserved, albeit with specific functions possibly sub-served by the expanded human gene family. To test evolutionary conservation, we used tissue-targeted transgenic expression of all three human genes in the Drosophila disease model to investigate function at (1) molecular, (2) neuronal and (3) non-neuronal levels. In neurons, dfmr1 null mutants exhibit elevated protein levels that alter the central brain and neuromuscular junction (NMJ) synaptic architecture, including an increase in synapse area, branching and bouton numbers. Importantly, hFMR1 can, comparably to dFMR1, fully rescue both the molecular and cellular defects in neurons, whereas hFXR1 and hFXR2 provide absolutely no rescue. For non-neuronal requirements, we assayed male fecundity and testes function. dfmr1 null mutants are effectively sterile owing to disruption of the 9+2 microtubule organization in the sperm tail. Importantly, all three human genes fully and equally rescue mutant fecundity and spermatogenesis defects. These results indicate that FMR1 gene function is evolutionarily conserved in neural mechanisms and cannot be compensated by either FXR1 or FXR2, but that all three proteins can substitute for each other in non-neuronal requirements. We conclude that FMR1 has a neural-specific function that is distinct from its paralogs, and that the unique FMR1

Comparison of Risperidone and Methylphenidate for Reducing ADHD Symptoms in Children and Adolescents with Moderate Mental Retardation.

ERIC Educational Resources Information Center

Filho, Alceu Gomes Correia; Bodanese, Rafael; Silva, Tatiana Laufer; Alvares, Julia Paglioza; Aman, Michael; Rohde, Luis Augusto

2005-01-01

Objective: To evaluate the short-term efficacy and tolerability of risperidone and methylphenidate for reducing symptoms related to attention-deficit/hyperactivity disorder (ADHD) in children and adolescents with moderate mental retardation. Method: In a 4-week, single-blind, parallel-group trial, 45 subjects with moderate mental retardation and…

Examining the Beliefs of Involved Adults Concerning the Moral and Religious Development of Individuals with Mental Retardation.

ERIC Educational Resources Information Center

Brown, Randel D.; Shepherd, Terry L.

This report discusses the outcomes of a study that examined the beliefs concerning the moral and religious development of individuals with mental retardation. Forty-four individuals who met the criteria of professionals who provide direct care to, or had administrative responsibility for, individuals with mental retardation participated in the…

Mental Retardation and the Law: A Report on Status of Current Court Cases.

ERIC Educational Resources Information Center

President's Committee on Mental Retardation, Washington, DC.

Presented by the President's Committee on Mental Retardation are an analysis of architectural barriers and a summary of new state cases, updated information on previously reported cases, and a listing of cases all concerned with the legal rights of the retarded. Architectural barriers are considered in terms of statutory actions and constitutional…

Short-Term Memory for Pictures and Words by Mentally Retarded and Nonretarded Persons.

ERIC Educational Resources Information Center

Ellis, Norman R.; Wooldridge, Peter W.

1985-01-01

Twelve mentally retarded and 12 nonretarded adults were compared in a Brown-Peterson short-term memory task for the retention of words and pictures over intervals up to 30 seconds. The retarded subjects forgot more rapidly over the initial 10 seconds. They also retained pictures better than they did words. (Author/DB)

Short-Term Memory of Children with Mental Retardation: Structural Defects or Control Deficits.

ERIC Educational Resources Information Center

Katims, David S.

The short-term memory of 24 retarded and 24 nonretarded individuals, aged 10 to 14, under conditions of restricted cognitive strategy use was investigated. An attempt was made to determine whether short-term memory difficulties of persons with mental retardation are caused by deficits in voluntary cognitive strategies, such as the organization and…

Location interval at Xp22.3 for X-linked chondrodysplasia punctata

DOE Office of Scientific and Technical Information (OSTI.GOV)

Sheffield, L.; Hutchison, W.; Holloway, A.

1994-09-01

The literature shows that there is a gene for chondropdysplasia punctata (CDP) located at Xp22.3 from the study of chromosomal rearrangements involving Xp. It is also suspected that there are genes for short stature and mental retardation nearby. Petit has described a family of brachytelephalangic CDP that was due to a submicroscopic interstitial deletion of Xp22.3. Symmetrical (mild) CDP seems to be identical to brachytelephalangic CDP clinically, has variable features of short stature and mental retardation, and has a preponderance of affected males. We describe results using DNA probes from Xp22.3 in 10 patients with radiologically proven symmetrical CDP. Othermore » known genes in this region have a high proportion of deletions as we screened our patients for deletions in DXYS20, MIC2, PABX, DXYS159X, DXS283, DXS285, J502(PCR), DXS31, DXS43 (listed distally to proximally). No deletions were found. We have also studied a fetus with proven CDP who has a X,Y translocation (46,V,t(X;Y)(p22.3;q12)mat). This patient was deleted for all distal probes up to J502(PCR). It is not yet known where the breakpoint lies but it may be just proximal to the CDP gene. The results of the 10 symmetrical CDPs and the X;Y translocation fetus are presented with further definition of the X chromosomal breakpoint in the translocation.« less

Nutritional status of mentally retarded children in north-west Spain. I. Anthropometric indicators.

PubMed

Sánchez-Lastres, J; Eirís-Puñal, J; Otero-Cepeda, J L; Pavón-Belinchón, P; Castro-Gago, M

2003-06-01

To evaluate the nutritional status of mentally retarded children in the region of Galicia in north-west Spain, on the basis of anthropometric variables. The following variables were determined in a sample of 128 mentally retarded children (81 M, 47 F): birthweight, bodyweight, height/length, head circumference, mid-arm circumference, mid-arm muscle circumference, triceps skinfold thickness, subscapular skinfold thickness, arm area, arm muscle area, arm fat area, arm lean-to-fat ratio, Shukla's nutrition index and Quetelet's body mass index (BMI). A preliminary statistical analysis indicated that most information content could be maintained considering only eight of these variables. A factor analysis of the resulting 8 x 128 (variables by subjects) data matrix was performed, identifying two factors (FA1 and FA2) that together explained 82% of total variance. Taking these factors as indicators of nutritional status, the data were analysed for possible effects of age, gender, socioeconomic and family environment, intelligence quotient (IQ), presence/absence of cerebral palsy, quality of diet, appetite and antiepileptic use. The analysis suggested that most subjects were in the normal nutrition range, but about 33% showed either borderline or definite malnutrition. Mean score on FA2 showed a significant negative correlation with age. Children with cerebral palsy had lower mean scores on both factors, and scores on both factors varied with IQ. Children with definite malnutrition had a significantly lower IQ than those in the normal nutrition range. Mean score on both factors varied with appetite and quality of diet. The mean FAI score of children from inland-rural areas was significantly lower than that of children from coastal or urban areas. Mean FA1 score increased with increasing age of the parents. The prevalence of obesity was 13% when obesity was defined on the basis of Shukla's nutrition index, and 19% when defined on the basis of FA1 score. Malnutrition as

Short-term memory for pictures and words by mentally retarded and nonretarded persons.

PubMed

Ellis, N R; Wooldridge, P W

1985-05-01

Mentally retarded and nonretarded persons were compared in a Brown-Peterson short-term memory task for the retention of words and pictures over intervals up to 30 seconds. The retarded subjects forgot more rapidly over the initial 10 seconds. They also retained pictures better than they did words; the nonretarded subjects retained these stimuli equally well. The results were theoretically interpreted as reflecting a structural memory deficit in retarded individuals, who were viewed as having greater facility with an imaginal memory code than with a verbal code. Transforming information from one code to another may also have been more difficult for retarded persons.

Issues in Fertility Control for Mentally Retarded Female Adolescents: II. Parental Attitudes toward Sterilization.

ERIC Educational Resources Information Center

Passer, Alice; And Others

1984-01-01

Interviews with 69 parents of mentally retarded adolescents revealed that 46 percent had considered sterilization for their daughters. Interest in sterilization especially correlated with increased severity of retardation and with difficulty teaching menstrual hygiene. Few parents supported either an extremely restrictive or permissive position.…

Enhancing Free-Recall Rates of Individuals with Mental Retardation.

ERIC Educational Resources Information Center

Carlin, Michael T.; Soraci, Sal A.; Dennis, Nancy A.; Chechile, Nicholas A.; Loiselle, Raquel C.

2001-01-01

This study with 16 adolescents with mental retardation compared free-recall rates under two encoding conditions: (1) fade-in, initially presenting pictures out of focus then slowly fading them into focus; and (2) fade-out, slowly blurring originally clear pictures. Results indicated that free-recall rates were greater for the fade-in items for…

Attitude Change Toward Trainable Mentally Retarded: "Mainstreaming in Reverse".

ERIC Educational Resources Information Center

Cronk, Mildred S.

An assessment was made of change in attitude toward trainable mentally retarded (TMR) students of 42 adults (members of the PTA Board and teachers of the experimental groups) and 127 children (in first, third, and sixth grades) as a result of special instruction and an integrated program. Regular class students in small groups were systematically…

Teaching Social Problem Solving to Individuals with Mental Retardation

ERIC Educational Resources Information Center

Crites, Steven A.; Dunn, Caroline

2004-01-01

The purpose of this study was to determine effectiveness of a problem-solving curriculum for transition-age students with mental retardation. The interactive training program Solving Your Problems (Browning, n.d.) was used to teach a five-step process for solving problems. Results indicate participants in the training group were able to use the…

Electroencephalography and phenytoin toxicity in mentally retarded epileptic patients.

PubMed Central

Iivanainen, M; Viukari, M; Seppäläinen, A M; Helle, E P

1978-01-01

There were significantly more diffuse and focal electroencephalographic abnormalities in 127 mentally retarded epileptic patients treated with phenytoin than in 68 epileptics without phenytoin. Phenytoin intoxication made the difference still more pronounced. Monitoring drug levels and electroencephalograms appears to be the method of choice for ensuring safe and effective medication in intractable epilepsy. PMID:632825

Case Management for Individuals with Mental Retardation. ARC Facts.

ERIC Educational Resources Information Center

Association for Retarded Citizens, Arlington, TX.

A question-and-answer format is used in this fact sheet to provide information on case management for individuals with mental retardation. The fact sheet describes the major components of a case management system, the role of the case manager, the individual's or family's role in case management, providers of case management services and systems…

OCCUPATIONS IN THE CARE AND REHABILITATION OF THE MENTALLY RETARDED.

ERIC Educational Resources Information Center

BOSS, MILTON R.; GREGG, RANDOLPH M.

TWENTY-SEVEN FULL TIME OCCUPATIONS INVOLVING DIAGNOSIS, CARE, AND REHABILITATION OF THE MENTALLY RETARDED ARE DISCUSSED. FOR EACH, AN OCCUPATIONAL DEFINITION, THE NEEDED QUALIFICATIONS, AND SOME INDICATION OF THE NECESSARY WORKER TRAIT REQUIREMENTS SUCH AS APTITUDES, INTERESTS, TEMPERAMENT, AND PHYSICAL DEMANDS AND WORKING CONDITIONS ARE…

Teaching and Training Relevant Community Skills to Mentally Retarded Persons.

ERIC Educational Resources Information Center

Matson, Johnny L.

1988-01-01

Reviews some of the major developments in teaching and training relevant community skills to mentally retarded persons. The following adaptive skills are discussed: (1) toilet use and bed wetting; (2) eating, dressing, and personal hygiene; (3) community survival; and (4) vocational and social skills. (BJV)

Behavioral Therapy of Phobias: A Case with Gynecomastia and Mental Retardation

ERIC Educational Resources Information Center

Revenq, Bernard

1974-01-01

A 13-year-old boy, institutionalized for severe mental retardation, who was found to have obsessive tendencies and an IQ of 71 to 78, was systematically desensitized for phobias associated with ambigous sexual identity. (MC)

The Effects of Motor Remediation with the Mentally Retarded: A Review of Research. Working Paper No. 74.

ERIC Educational Resources Information Center

DiRocco, Patrick J.

The author reviews research on the effects of motor remediation with the mentally retarded on the areas of motor ability, physical fitness, social and emotional competency, reading achievement, and intelligence. It is concluded that motor development theories have led to an increase of motor training programs for the mentally retarded, and that…

Effects of Different Variations of Mental and Physical Practice on Sport Skill Learning in Adolescents with Mental Retardation

ERIC Educational Resources Information Center

Hemayattalab, Rasool; Movahedi, Ahmadreza

2010-01-01

The purpose of this study was to investigate the effect of five variations of imagery and physical practice on learning of Basketball free throws in adolescents with mental retardation (AWMR). Forty AWMR were randomly assigned to five groups and performed a variation of practice: physical practice, mental practice, physical practice followed by…

Housekeeping Management Assistant Manual for Training of the Mentally Retarded.

ERIC Educational Resources Information Center

Texas Tech Univ., Lubbock. Research and Training Center in Mental Retardation.

Presented is an instructional guide for teaching mentally retarded persons to become housekeeping assistants. Listed are tasks, objectives, and background information for the following six units: general safety procedures; daily duties (general cleaning, use of supply carts, and bathroom cleaning); laundry procedures (including use of washer and…

Reducing Barriers to Sex Education for Adults with Mental Retardation.

ERIC Educational Resources Information Center

Huntley, Cristy F.; Benner, Susan M.

1993-01-01

Opinions of professionals from 16 agencies were obtained to identify techniques that agencies serving adults or adolescents with mental retardation could utilize to provide systematic sex education and counseling. Recommendations are offered in five areas: self-advocacy and self-determination, individual design, staff training and support,…

Behavior Disorders in Persons with Mental Retardation Receiving Antipsychotic Medication.

ERIC Educational Resources Information Center

Ono, Yoshiro

1998-01-01

The behavior disorders of 54 Japanese individuals with mental retardation receiving antipsychotic medication were compared to 52 subjects receiving anticonvulsants and 202 subjects without medication. Results found the problem behaviors of subjects receiving antipsychotic drugs were more severe and severity of disability was associated with higher…

A Bibliography of Bibliographies on Mental Retardation, 1963-June 1975.

ERIC Educational Resources Information Center

Black, Lawrence, Comp.

Presented is a bibliography of over 100 bibliographies (published in 1963-1975) intended for social workers, teachers, and other professionals working in the field of mental retardation. Entries are arranged alphabetically by author or contributing organization under the following categories: general references, biochemistry, care of the mentally…

Persons with Mental Retardation and Technology Use Patterns and Needs.

ERIC Educational Resources Information Center

Parette, Howard P., Jr.; VanBiervliet, Alan

A questionnaire examining consumer needs, spending, travel, credit options and utilization practices in adaptive/assistive and educational technology was answered by 2,201 Arkansans with disabilities of all ages. This paper emphasizes results relating to Arkansans with mental retardation. Results indicate Medicare/Medicaid as the single most…

Neuroleptic-Induced Dementia Documented in Four Adults with Mental Retardation.

ERIC Educational Resources Information Center

Gedye, A.

1998-01-01

Describes four case studies of neuroleptic-induced dementia in four adults with mental retardation. The neuroleptic-induced dementia occurred while the individuals were on a low dose of either loxapine, thioridazine, haloperidol, or pimozide. The individuals showed full recovery after the offending medication was withdrawn. (Author/CR)

The Effects of Spiritual Self-Care Training on Caregiving Strain in Mothers of Mentally Retarded Children

PubMed Central

Dindar, Mitra; Afshari, Mehdi; Moghadam, Mahdieh Poodineh

2016-01-01

Introduction Care for a mentally retarded child induces a lot of problems for the mother and leads her to care giving strain and ignorning her self-care. Spiritual health will co-ordinate all aspects of human life and is necessary for coping with diseases in mother of mentally retarded children. Aim To evaluate the effects of spiritual self-care training on care giving strain in mothers of mentally retarded children. Materials and Methods The present study, is a before and after type quasi-experimental research based on which 60 mothers of mentally retarded children who were hospitalized in Elahi Rehabilitation Center in Quchan City, were selected using convenience sampling and were randomly assigned to intervention and control groups. Data was collected by demographic characteristic questionnaire and care giving strain questionnaire that were filled by groups before, immediately and two weeks after spiritual self-care training. Data was analysed using SPSS version 20. Results According to the results, there was no significant difference between the mean score of care giving strain in intervention and control groups before and immediately after the intervention. However, among the members of the intervention group the score of mother care giving strain decreased an average of 87.21% within two weeks after the intervention, which was statistically significant over time (p=0.001). The score of mothers in the control group increased an average of 5% over time which was not statistically significant (p=0.4). The observed differences between these groups were also statistically significant even after controlling the effects of such intervening factors as marital status, children age and the years of caring for children (p=0.001). Conclusion Spiritual self-care training can decrease care giving strain in mothers of mentally retarded children. Therefore, strengthening their spiritual beliefs and backgrounds, mothers can greatly reduce the strain caused by care giving

Conceptual systems and teacher attitudes toward regular classroom placement of mildly mentally retarded students.

PubMed

Feldman, D; Altman, R

1985-01-01

The effects of a teacher personality construct (abstract vs. concrete conceptual system) and two pupil variables (race, school behavior) on 454 regular classroom teachers' attitudes toward mainstreaming were determined. Following administration of the Conceptual Systems Test, teachers were randomly assigned a profile of a mildly mentally retarded student that held pupil IQ and school achievement constant while varying pupil's race and school behavior. Subjects responded on an integration inventory comprised of three subscales: social-psychological classroom environment, self-actualization, and classroom cohesiveness. Results revealed a significant main effect on the behavior variable and a significant Personality X Race interaction on all inventory dimensions, suggesting that these teachers perceived maladaptive behavior of mainstreamed retarded students as a significant threat to a conducive instructional atmosphere and the capability of nonretarded students to achieve to their potential. These results have implications for inservice training for teachers based on the pupil race and teacher conceptual system findings.

Growth, Hypothalamic Function, and Brain Ventricle Size in Mentally Retarded Subjects

ERIC Educational Resources Information Center

Leisti, S.; Iianainen, M.

1978-01-01

To determine whether moderate enlargement of the third brain ventricle or the temporal horns of the lateral ventricles was associated with hypothalamic dysfunction, 15 mentally retarded Ss (ages 12-25 years) with such characteristics were studies. (DLS)

In vivo neuronal function of the fragile X mental retardation protein is regulated by phosphorylation

PubMed Central

Coffee, R. Lane; Williamson, Ashley J.; Adkins, Christopher M.; Gray, Marisa C.; Page, Terry L.; Broadie, Kendal

2012-01-01

Fragile X syndrome (FXS), caused by loss of the Fragile X Mental Retardation 1 (FMR1) gene product (FMRP), is the most common heritable cause of intellectual disability and autism spectrum disorders. It has been long hypothesized that the phosphorylation of serine 500 (S500) in human FMRP controls its function as an RNA-binding translational repressor. To test this hypothesis in vivo, we employed neuronally targeted expression of three human FMR1 transgenes, including wild-type (hFMR1), dephosphomimetic (S500A-hFMR1) and phosphomimetic (S500D-hFMR1), in the Drosophila FXS disease model to investigate phosphorylation requirements. At the molecular level, dfmr1 null mutants exhibit elevated brain protein levels due to loss of translational repressor activity. This defect is rescued for an individual target protein and across the population of brain proteins by the phosphomimetic, whereas the dephosphomimetic phenocopies the null condition. At the cellular level, dfmr1 null synapse architecture exhibits increased area, branching and bouton number. The phosphomimetic fully rescues these synaptogenesis defects, whereas the dephosphomimetic provides no rescue. The presence of Futsch-positive (microtubule-associated protein 1B) supernumerary microtubule loops is elevated in dfmr1 null synapses. The human phosphomimetic restores normal Futsch loops, whereas the dephosphomimetic provides no activity. At the behavioral level, dfmr1 null mutants exhibit strongly impaired olfactory associative learning. The human phosphomimetic targeted only to the brain-learning center restores normal learning ability, whereas the dephosphomimetic provides absolutely no rescue. We conclude that human FMRP S500 phosphorylation is necessary for its in vivo function as a neuronal translational repressor and regulator of synaptic architecture, and for the manifestation of FMRP-dependent learning behavior. PMID:22080836

Mental Retardation and Developmental Disabilities: 1981 Research Programs of the National Institute of Child Health and Human Development.

ERIC Educational Resources Information Center

National Inst. of Child Health and Human Development (NIH), Bethesda, MD.

The monograph reviews federal research activities and progress in biomedical and behavioral/social science research in mental retardation. Activities represent the National Institute of Child Health and Human Development and the Mental Retardation and Developmental Disabilities branch. The following categories are addressed in terms of biomedical…

Book of Proceedings of Workshops Held on Physical Education for the Mentally Retarded.

ERIC Educational Resources Information Center

Mississippi Univ. for Women, Columbus.

Proceedings are presented from three workshops concerning physical education for the mentally retarded held at Mississippi State University, University of Mississippi, and University of Southern Mississippi in 1967. Topics covered in the workshops include program development of physical education with the mentally handicapped, reports of research…

Dietary Habits and Nutritional Status in Mentally Retarded Children and Adolescents: A Study from North Western India

ERIC Educational Resources Information Center

Mathur, Manju; Bhargava, Rachna; Benipal, Ramandeep; Luthra, Neena; Basu, Sabita; Kaur, Jasbinder; Chavan, B. S.

2007-01-01

Objective: To compare the dietary habits and nutritional status of mentally retarded (MR) and normal (NG) subjects and to examine the relationship between the dietary habits and nutritional status and the level of mental retardation in the MR group. Method: A case control design was utilized: 117 MR (random sampling) and 100 NG (quota sampling)…

Increasing Wearing of Prescription Glasses in Individuals with Mental Retardation

ERIC Educational Resources Information Center

DeLeon, Iser G.; Hagopian, Louis P.; Rodriguez-Catter, Vanessa; Bowman, Lynn G.; Long, Ethan S.; Boelter, Eric W.

2008-01-01

This study evaluated an intervention for promoting wearing of prescription glasses in 4 individuals with mental retardation who had refused to wear their glasses previously. Distraction through noncontingent reinforcement (NCR) increased independent glasses wearing for 1 of the 4 participants. An intervention consisting of NCR, response cost, and…

Screening for Osteoporosis in Community-Dwelling Adults with Mental Retardation.

ERIC Educational Resources Information Center

Tyler, Carl V., Jr.; Snyder, Clint W.; Zyzanski, Stephen

2000-01-01

Osteoporosis screening of 107 adults, ages 40 to 60, with mental retardation who attended community-based training centers found 21 percent had osteoporosis and 34 percent had osteopenia. The most significant predictor of lower bone mineral densities were Down syndrome, mobility status, and race. (Contains references.) (Author/DB)

A controlled single-case treatment of severe long-term selective mutism in a child with mental retardation.

PubMed

Facon, Bruno; Sahiri, Safia; Rivière, Vinca

2008-12-01

The aim of the present study was to demonstrate the efficacy of combining two operant learning procedures--shaping and fading--for treating selective mutism. The participant was a 12-year-old boy with mental retardation presenting a severe long-term selective mutism. The treatment was aimed at increasing the loudness of his vocalizations in an increasingly social milieu. The treatment was conducted over the course of about 20 weeks, with four 15-minute sessions per week. A gradual increase in speech loudness was observed. Data indicated a close correspondence between the changes in speech loudness and the criteria for reinforcement successively applied by the therapist, thereby confirming the causal link between the child's progress and the changes in reinforcement contingencies. In addition, good generalization was noted during the stimulus fading phase. Six-month follow up showed that loudness of verbalizations was still satisfactory in the classroom despite a change of school and peer group. The impressive improvement of the child's verbal behavior shows that the implementation of a treatment package including both shaping and stimulus fading is a worthwhile therapeutic option, even in the case of severe long-term selective mutism associated with mental retardation.

Syndromes and disorders associated with mental retardation.

PubMed

Greydanus, Donald E; Pratt, Helen D

2005-10-01

This article reviews selective comments on the concept of Mental Retardation (MR) in adolescents. Issues covered include the definition, prevalence, and differential diagnosis of MR. Some of the syndromes and disorders associated with MR in the adolescents are also considered with emphasis on the behavioral concerns that may be present in this age group. Finally, concepts of management by the clinician are reviewed. It is recommended that health care professionals caring for adolescents with MR should help these youths maximize their potential as human beings, helping them achieve meaningful functioning in adulthood.

A locus for axonal motor-sensory neuropathy with deafness and mental retardation maps to Xq26-q27

DOE Office of Scientific and Technical Information (OSTI.GOV)

Priest, J.M.; Nouri, N.; Keats, B.J.B.

1994-09-01

Twenty-two DNA markers spanning the X chromosome have been analyzed for linkage to the locus causing an unusual form of X-linked recessive hereditary motor and sensory neuropathy in a Pennsylvania family of Italian ancestry. This 3 generation family which was originally reported by Cowchock includes 7 affected males, 3 obligate carrier females, and 4 unaffected males. Males are severely affected at birth or within the first few years of life with areflexia, slowly progressive axonal atrophy, and absence of large myelinated fibers, and they all develop pes cavus and hammer toes. Five of the 7 affected males show associated deafnessmore » and 3 of these 5 individuals also presented with mental retardation or social developmental delay. Motor nerve conduction velocities in affected males are normal to mildly delayed and sensory conduction velocities are markedly abnormal. Heterozygous females are asymptomatic. Close linkage to the Xg blood group locus (Xp22) was previously excluded in this family while weak linkage of the disease gene to DXYS1 (Xq13-q21) was suggested. The current study excludes the short arm and the proximal long arm of the X chromosome. Haplotype analysis of markers on the long arm demonstrates that HPRT is a proximal flanking marker and that the disease gene is closely linked to the marker DXS984. Further microsatellite markers are being studied in order to refine the region of the distal long arm of the X chromosome containing the gene causing the motor-sensory neuropathy in this family. This is the first such gene assigned to the distal region of Xq.« less

Some Aspects of Mental Retardation. A Training Guide for the Vocational Rehabilitation Counselor. Final Report.

ERIC Educational Resources Information Center

Phelps, William R.

Presented for vocational counselors is an overview of mental retardation, including the definition, and etiology. Described are the prenatal factors of heredity (including familial retardation and cranial anomalies), infections (including syphilis and encephalitis), radiation, blood group incompatibility, and unknown or variable etiological…

Efficacy of Short Term Training in Education of Children with Mental Retardation to Teachers of Visually Impaired Students.

ERIC Educational Resources Information Center

Narayan, J.; Subba Rao, T. A.; Reddy, O. R.

1998-01-01

A one-week inservice program on mental retardation was provided to 24 teachers of students with visual impairments in India. Emphasis was on community based rehabilitation and the teaching of children with mental retardation who also have visual impairment. Testing revealed participants had gained knowledge and their evaluations indicated they…

Vitamin/Mineral Supplements and Intelligence of Institutionalized Mentally Retarded Adults.

ERIC Educational Resources Information Center

Ellis, Norman R.; Tomporowski, Phillip D.

1983-01-01

Vitamin/mineral supplements were administered to 19 institutionalized mentally retarded adults in a double-blind study over a seven-month period. No changes were observed in IQ nor in adaptive behavior. This was a replication with adults of a previous study of children that had obtained positive results. (Author/CL)

A Behavioral Weight Reduction Model for Moderately Mentally Retarded Adolescents.

ERIC Educational Resources Information Center

Rotatori, Anthony F.; And Others

1980-01-01

A behavioral weight reduction treatment and maintenance program for moderately mentally retarded adolescents which involves six phases from background information collection to followup relies on stimulus control procedures to modify eating behaviors. Data from pilot studies show an average weekly weight loss of .5 to 1 pound per S. (CL)

The Juberg-Marsidi syndrome maps to the proximal long arm of the X chromosome (Xq12-q21)

DOE Office of Scientific and Technical Information (OSTI.GOV)

Saugier-Veber, P.; Abadie, V.; Turleau, C.

Juberg-Marsidi syndrome (McKusick 309590) is a rare X-linked recessive condition characterized by severe mental retardation, growth failure, sensorineural deafness, and microgenitalism. Here the authors report on the genetic mapping of the Juberg-Marsidi gene to the proximal long arm of the X chromosome (Xq12-q21) by linkage to probe pRX214H1 at the DXS441 locus (Z = 3.24 at [theta] = .00). Multipoint linkage analysis placed the Juberg-Marsidi gene within the interval defined by the DXS159 and the DXYS1X loci in the Xq12-q21 region. These data provide evidence for the genetic distinction between Juberg-Marsidi syndrome and several other X-linked mental retardation syndromes thatmore » have hypogonadism and hypogenitalism and that have been localized previously. Finally, the mapping of the Juberg-Marsidi gene is of potential interest for reliable genetic counseling of at-risk women. 25 refs., 2 figs., 3 tabs.« less

Self-Management Training of Mentally Retarded Adults with Chronic Conduct Difficulties. RT11.

ERIC Educational Resources Information Center

Cole, Christine L.; And Others

Six mildly and moderately mentally retarded adults with chronic and severe emotional/behavioral problems participated in a study of the assumption that aggression and conduct disorders reflected self-managed coping skills deficits. The study was conducted in a vocational training workshop setting located in the Waisman Center on Mental Retardation…

Piaget's Developmental Theories Used in an Early Childhood Program for Mentally Retarded Students.

ERIC Educational Resources Information Center

Francis Christi, Sister

The author describes a Piaget based curriculum involving three main themes (classification, number measurement/space, and seriation) for young mentally retarded children. Students solve problems by doing something physically and mentally to the data, shuffling the facts about in their minds, so that they will come to a logical solution. Language…

The Drosophila homolog of Down's syndrome critical region 1 gene regulates learning: Implications for mental retardation

PubMed Central

Chang, Karen T.; Shi, Yi-Jun; Min, Kyung-Tai

2003-01-01

Mental retardation is the most common phenotypic abnormality seen in Down's syndrome (DS) patients, yet the underlying mechanism remains mysterious. DS critical region 1 (DSCR1), located on chromosome 21, is overexpressed in the brain of DS fetus and encodes an inhibitor of calcineurin, but its physiological significance is unknown. To study its functional importance and role in mental retardation in DS, we generated Drosophila mutants of nebula, an ortholog of human DSCR1. Here, we report that both nebula loss-of-function and overexpression mutants exhibit severe learning defects that are attributed by biochemical perturbations rather than maldevelopment of the brain. These results, combined with our data showing that the same biochemical signaling pathway is altered in human DS fetal brain tissue overexpressing DSCR1, suggest that alteration of DSCR1 expression could contribute to mental retardation in DS. PMID:14668437

Simple mental addition in children with and without mild mental retardation.

PubMed

Janssen, R; De Boeck, P; Viaene, M; Vallaeys, L

1999-11-01

The speeded performance on simple mental addition problems of 6- and 7-year-old children with and without mild mental retardation is modeled from a person perspective and an item perspective. On the person side, it was found that a single cognitive dimension spanned the performance differences between the two ability groups. However, a discontinuity, or "jump," was observed in the performance of the normal ability group on the easier items. On the item side, the addition problems were almost perfectly ordered in difficulty according to their problem size. Differences in difficulty were explained by factors related to the difficulty of executing nonretrieval strategies. All findings were interpreted within the framework of Siegler's (e.g., R. S. Siegler & C. Shipley, 1995) model of children's strategy choices in arithmetic. Models from item response theory were used to test the hypotheses. Copyright 1999 Academic Press.

Comparison of Sustained and Selective Attention in Children Who Have Mental Retardation with and without Attention Deficit Hyperactivity Disorder.

ERIC Educational Resources Information Center

Pearson, Deborah A.; And Others

1996-01-01

This study compared children with attention deficit hyperactivity disorder (ADHD) and mental retardation with children with only mental retardation on tasks involving sustained and selective attention. No compelling evidence emerged for sustained attention deficits in the ADHD children, though evidence suggesting selective attention deficits was…

Decreasing Self-Stimulating Behaviors with the Profoundly Mentally Retarded, While Acknowledging Obstacles and Frustrations.

ERIC Educational Resources Information Center

Smith, Susan J.; Solimani, Genevieve

Two studies examined different treatment procedures to suppress self-stimulating behaviors with the profoundly mentally retarded. In experiment 1, a fine mist of cold water from a spray bottle was applied to the neck of a teenaged student in a class for the profoundly retarded. The intervention was very successful in reducing inappropriate humming…

Foundation Care: A Treatment Model for Nonambulatory Profoundly Mentally Retarded Persons.

ERIC Educational Resources Information Center

LaMendola, Walter F.; And Others

1987-01-01

Two institutional treatment models--Intermediate Care Facilities and Foundation Care--were compared with 30 profoundly mentally retarded adults. The Foundation Care model (which emphasized habilitation through health, nurturance, stimulation, and play) demonstrated more resident-oriented management practices and used more tactile modalities and…

On-The-Job Training Program, Educable Mentally Retarded. Final Report.

ERIC Educational Resources Information Center

Pocatello School District 25, ID.

School training and vocational services were combined into a program designed to demonstrate that educable mentally retarded students could develop well rounded working habits that would mutually benefit them and the community. On-the-job training was used as a demonstration of the practical use of academic, social, and vocational skills learned…

Sexual Health and Behavior of Mentally Retarded Pupils in Japan

ERIC Educational Resources Information Center

Tsutsumi, Angela Aparecida

2009-01-01

Sex education has always been a controversial topic. Although sex education at schools has improved in general, sex education for mentally retarded pupils still encounters prejudice and conservatism as a result of several misconceptions about the subject. This research was performed in the form of a survey of opinions about sex education in…

Sudden Unexpected Death in Epilepsy in Adults with Mental Retardation.

ERIC Educational Resources Information Center

McKee, Jerry R.; Bodfish, James W.

2000-01-01

Medical records of residents of a facility for persons with mental retardation from January 1, 1978, through December 31, 1997, were analyzed to identify incidence of sudden unexpected death for 180 individuals with and 125 without comorbid epilepsy. Eighty deaths were identified, with 55 occurring in those with epilepsy. (Contains 15 references.)…

THE MENTALLY RETARDED CHILD AT HOME, A MANUAL FOR PARENTS.

ERIC Educational Resources Information Center

DITTMANN, LAURA L.

THIS MANUAL IS DEVOTED CHIEFLY TO THE MANAGEMENT OF YOUNG RETARDED CHILDREN AND CONCENTRATES ON DAY-TO-DAY ACTIVITIES--PHYSICAL HEALTH, MENTAL HEALTH, NEED FOR LOVE AND AFFECTION, SIBLING UNDERSTANDING, AND TRAINING IN SELF HELP AND OTHER SKILLS. EARLY CHARACTERISTICS AND NEEDS ARE DISCUSSED. THE FOLLOWING SKILLS ARE TREATED--FEEDING, DRINKING,…

Individuals with Mental Retardation from the Perspective of Turkish People

ERIC Educational Resources Information Center

Güner Yildiz, Nevin

2015-01-01

This descriptive study examined the attitude of Turkish people towards the mentally retarded. The working group was composed of 329 male and female staff in various places of employment in Eskisehir provincial center, 87 non-working women living in Eskisehir provincial center and 49 non-working women living in Mugla provincial center. Research…

International Summit on Prevention of Mental Retardation from Biomedical Causes.

ERIC Educational Resources Information Center

Koch, Jean, Ed.

Proceedings from the International Summit on Prevention of Mental Retardation from Biomedical Causes are provided. After a list of participants and summary of the highlights of the conference, the keynote address by H. Moser is presented. The following 13 papers are provided along with workshop recommendations and a list of main points elicited…

Oral rehabilitation and management of mentally retarded.

PubMed

Solanki, Jitender; Khetan, Jitendra; Gupta, Sarika; Tomar, Deepak; Singh, Meenakshi

2015-01-01

High level of periodontal problems of dental caries are frequently observed in mentally handicapped children. This group of patients presents various problems when they face dental treatments. Identification of such population and providing them affordable oral health care is the new concept. A systematic method for identification and screening of persons with mental retardation has been developed and is being followed. Cost and fear are the most commonly cited barriers to dental care. Physical or mental may lead to deterioration in self-care, and oral care state have a low priority. Risk factors are inter-related and are often barriers to oral health. With advancements in today's world sufficient information and support is available for each and every individual to lead a healthy life which include the access to the oral health care. Factors such as fear, anxiety and dental phobia plays a vital role in acceptance of dental care and also the delaying of dental care. Lack of knowledge of oral and dental disease, awareness or oral need, oral side-effects of medication and organization of dental services are highlighted in the literature. All health personnel should receive training to support the concept of primary oral health care. Training about dealing with such mentally handicapped people should be addressed urgently among the health professionals.

Investigation of the Effectiveness of the Story-Map Method on Reading Comprehension Skills among Students with Mental Retardation

ERIC Educational Resources Information Center

Isikdogan, Necla; Kargin, Tevhide

2010-01-01

The purpose of this study was to investigate the effectiveness of the story-map technique on reading comprehension skills among students with mild mental retardation. The research group consisted of 14 students with mild mental retardation. The students in the research group were chosen from students who attended to an elementary school and a…

Bone Mineral Density and Respiratory Muscle Strength in Male Individuals with Mental Retardation (with and without Down Syndrome)

ERIC Educational Resources Information Center

da Silva, Vinicius Zacarias Maldaner; Barros, Jonatas de Franca; de Azevedo, Monique; de Godoy, Jose Roberto Pimenta; Arena, Ross; Cipriano, Gerson, Jr.

2010-01-01

The purpose of this study was to assess the respiratory muscle strength (RMS) in individuals with mental retardation (MR), with or without Down Syndrome (DS), and its association with bone mineral density (BMD). Forty-five male individuals (15 with DS, 15 with mental retardation (MR) and 15 apparently healthy controls), aged 20-35, participated in…

Chronological age and performance of persons with mental retardation on verbal subtests of the Wechsler Intelligence Scale for Children--Revised, French Version.

PubMed

Facon, B; Facon-Bollengier, T

1999-12-01

The Influence of chronological age on verbal intelligence of persons with metal retardation was studied using the French version of the Wechsler Intelligence Scale for Children--Revised. Participants were 1,101 children and adolescents divided into two groups according to the severity of metal retardation. The first group included 551 participants with moderate mental retardation, the second 550 participants with mild mental retardation. Analyses indicated a significant association between chronological age and raw scores on the four Verbal Comprehension subtests, even with performance on Perceptual Organization and Freedom from Distractibility subtests held constant. This finding suggests the relationship between chronological age and verbal intelligence is valid for persons with moderate mental retardation as well as for those with mild metal retardation.

Do adults with mental retardation show pictorial superiority effects in recall and recognition?

PubMed

Cherry, Katie E; Applegate, Heather; Reese, Celinda M

2002-01-01

We examined memory for pictures and words in adults with mental retardation and a control group of adults of normal intelligence. During acquisition, sets of simple line drawings and matching words were presented for study using an intentional learning procedure. The principle dependent measures were free recall and recognition. Measures of working memory span were also administered. Pictorial superiority effects occurred in free recall and recognition for both intelligence-level groups. Correlational analyses indicated that working memory span was primarily related to recall performance, irrespective of stimulus format. These data strongly suggest that persons with mental retardation can utilize nonverbal memory codes to support long-term retention as effectively as do adults of normal intelligence.

Psychological Effects on the Family of a Mentally Retarded Child.

ERIC Educational Resources Information Center

Russell, Dorothy S.

The paper discusses the birth of a mentally retarded infant in terms of initial parent reactions, adjustment, decision to institutionalize, psychological effect on the mother, psychological effect on the father, impact on the marriage, and impact on siblings. The birth is a traumatic experience for the parents and can result in initial feelings of…

Automation in Vocational Training of the Mentally Retarded. Final Report.

ERIC Educational Resources Information Center

Platt, Henry; And Others

Various uses of automation in teaching were studied with mentally retarded (IQ 70 to 90) and/or emotionally disturbed (IQ 80 to 90) youth aged 16 to 20. Programed instruction was presented by six audiovisual devices and techniques: the Devereux Model 50 Teaching Aid, the Learn-Ease Teaching Device, the Mast Teaching Machine, the Graflex…

Co-Worker Involvement in Employment Programs for Persons with Mental Retardation.

ERIC Educational Resources Information Center

McNair, Jeff

This paper reviews recent literature related to co-worker involvement in non-sheltered community-based employment programs for persons with mental retardation. Discussion of co-worker roles is categorized into six areas: training, associating, befriending, advocating, evaluating, and information giving. Also discussed are other variables…

Assessing Student Teachers' Attitudes toward Mentally Retarded and Physically Handicapped Persons.

ERIC Educational Resources Information Center

Ashman, Adrian F.

1984-01-01

Analysis of questionnaires completed by 104 postgraduate level education students revealed that Ss were more accepting of physical than mental handicaps. No sex differences were found. Identified five factors: perceived rights and privileges of physically handicapped and retarded persons, interpersonal comfort with handicapped people, attitudes…

A Survey of School Psychologists' Practices for Identifying Mentally Retarded Students.

ERIC Educational Resources Information Center

Wodrich, David L.; Barry, Christine T.

1991-01-01

Surveyed school psychologists regarding identification of mentally retarded students. The Wechsler scales were the most frequently used tests for deriving intelligence quotient scores, which together with adaptive behavior scale scores were rated as most influential in identification-placement decisions. The Vineland Adaptive Behavior Scales were…

Dementia and Depression in Elders with Mental Retardation: A Pilot Study.

ERIC Educational Resources Information Center

Harper, Dennis C.; Wadsworth, John S.

1990-01-01

This article investigates cognitive decline and depressive symptomatology among older adults with mental retardation. A pilot study of assessment instruments is reported. Findings reveal that decreasing cognitive ability is associated with higher rates of observed depression and reported behavioral problems. Cognitive decline was associated with…

[Development of an Occupational Training Center for the Mentally Retarded.] Final Project Report.

ERIC Educational Resources Information Center

Hastbacka, Edwin A.

The Occupational Training Center prepared mentally retarded young adults (over age 15, IQ range 30 to 70, average IQ 55) who were beyond regular schooling for competitive employment. The training program consisted of 60 to 90 mentally and multiply handicapped students per year (35 at a time) and used subcontracts from local businesses. The…

Proceedings of a National Multicultural Seminar on Mental Retardation among Minority Disadvantaged Populations (Norfolk, Virginia, October 10-12, 1977).

ERIC Educational Resources Information Center

President's Committee on Mental Retardation, Washington, DC.

The National Multicultural Seminar on Mental Retardation among Minority Disadvantaged Populations was initiated in response to concern about the root causes of mental retardation especially in mild forms which tend to be more prevalent and more devastating among persons living in depressed, disrupted, and impoverished environments. Nineteen papers…

Community Supports for People Labeled by Both the Mental Retardation and the Mental Health Systems.

ERIC Educational Resources Information Center

Knoll, James; Racino, Julie Ann

The paper addresses issues in providing community services for people with both mental retardation and emotional or behavioral disorders. First, it explores the meaning often found at the root of the often elusive definition of dual diagnosis concluding that this has become a catch-all category for people who present existing service systems with…

Brief report: linkage between G6PD and fragile-X syndrome.

PubMed

Filippi, G; Rinaldi, A; Archidiacono, N; Rocchi, M; Balazs, I; Siniscalco, M

1983-05-01

Eighteen Sardinian pedigrees segregating for the X-fragile site syndrome were studied with respect to the segregation of the fragile site (FS) at Xq28, mental retardation, and macro-orchidism. No exception was found in the association of this symptomatic triad (MOM-X) in 41 out of 42 patients examined. The exceptional individual had micro- rather than macro-orchidism and was found to have a 47, XXY sex chromosome complement. In six informative sibships, the MOM-X syndrome was found to segregate in close linkage association with G6PD-deficiency or protan colorblindness. The maximum likelihood estimate of recombination if 6% with 90% fiducial limits between 2.5 and 19.5% and an odds ratio in favor of measurable linkage of 428:1. However, no hint of measurable linkage was found in six pedigrees segregating for G6PD and the Renpenning syndrome or other unspecified types of X-linked mental retardation. These data give strong support to the generally held hypothesis that the FS at Zq28, characteristic of the MOM-X syndrome, is a direct expression of a genetic change in the same chromosomal region. They also clearly suggest that X-linked MR without FS may be the result of different allelic mutations at the same locus.

A Consultation-Conference on the Gerontological Aspects of Mental Retardation.

ERIC Educational Resources Information Center

Hamilton, Jane C., Ed.; Segal, Robert M., Ed.

Presented are the proceedings of a 1975 conference on the aged mentally retarded which focused on the following needs: health and medical needs, emotional and social needs, housing needs, and financial needs. Explained is the design and use of a workbook distributed to participants prior to the conference. The delivery of services on the state and…

State of Ohio Curriculum Guide for Moderately Mentally Retarded Learners.

ERIC Educational Resources Information Center

Mays, Maxine.

Provided is a curriculum guide based on the lifelong learning needs of moderately retarded persons. Behavioral objectives are identified at each of six levels of functioning which approximate the mental age levels of 1.5, 3.0, 5.0, 7.0, 8.0, and 8.0 + (adult) years. Sections cover the following persisting life problem areas: (1) developing the…

MID-LIFE ATTAINMENT OF THE MENTALLY RETARDED, A LONGITUDINAL STUDY.

ERIC Educational Resources Information Center

BALLER, WARREN R.; AND OTHERS

THIS STUDY LOCATED MENTALLY RETARDED, SLOW LEARNING, AND NORMAL SUBJECTS WHO HAD BEEN EVALUATED DURING THE 1930'S. THEIR PRESENT STATUS AND FUNCTIONING WERE REPORTED AND THE STUDY SEARCHED FOR REASONS FOR THE SUBJECTS' DIFFERENT LEVELS OF LIFE ADJUSTMENT. OVER 400 OF THE ORIGINAL SUBJECTS WERE LOCATED. THEY WERE IN THEIR MIDDLE FIFTIES AT THE TIME…

Psychopathology in Children and Adolescents with ASD without Mental Retardation

ERIC Educational Resources Information Center

Caamaño, Marta; Boada, Leticia; Merchán-Naranjo, Jessica; Moreno, Carmen; Llorente, Cloe; Moreno, Dolores; Arango, Celso; Parellada, Mara

2013-01-01

This study analyzes subclinical psychopathology in children and adolescents with autism spectrum disorders (ASD) without mental retardation with no comorbid disorder, assessed by an extensive general psychopathology interview. The K-SADS-PL was administered to a group of 25 patients with ASD (mean age = 12.80 ± 2.86 years) and 25 healthy controls…

Psychological Aspects of Sleep Disorders in Children with Mental Retardation.

ERIC Educational Resources Information Center

Smith, David T.

This paper reviews literature and clinical experiences on the neurobiological and psychological aspects of sleep in children with mental retardation. The lack of a universal, operational definition of sleep disorders is noted, and a study is cited in which 61% of a group of 20 children (ages 2-13) with developmental disabilities were found to have…

MR78, Mental Retardation: The Leading Edge. Service Programs That Work.

ERIC Educational Resources Information Center

Perske, Robert

The book highlights 30 effective service programs for mentally retarded persons. The first section presents information on eight family oriented programs serving high risk, Down's syndrome, urban poor, and profoundly handicapped children. Included are descriptions of parent education, support, and advocacy programs. Among seven public school…

USE OF NEW AUDIO-VISUAL TECHNIQUES TO TEACH MENTALLY-RETARDED CHILDREN.

ERIC Educational Resources Information Center

ROSS, DOROTHEA M.

DEPENDENCY LEARNING, THE ACQUISITION AND DEVELOPMENT OF PERSONAL INTERRELATIONSHIP VALUES, WAS STUDIED AS A TECHNIQUE FOR FOSTERING AUDIOVISUAL ACADEMIC LEARNING AMONG 54 YOUNG, EDUCABLE MENTAL RETARDATES. SOME OF THESE SUBJECTS WERE TAUGHT TO VALUE SIMULATED DEPENDENCY MODELS. THESE MODELS WERE CONSISTENTLY PAIRED WITH SUCH REWARDING STIMULI AS…

Mental Retardation Programs of the Department of Health, Education, and Welfare.

ERIC Educational Resources Information Center

Department of Health, Education, and Welfare, Washington, DC. Office of Mental Retardation Coordination.

The mental retardation programs of the Department of Health, Education, and Welfare are arranged and explained according to the categories of preventive services, basic and supportive services, training of personnel, research, construction, and income maintenance. Then follow eight agency reports that generally contain introductory information,…

Planning Curriculum Development: With Examples from Projects for the Mentally Retarded.

ERIC Educational Resources Information Center

Mayer, William V., Ed.

Presented are guidelines based on five federally funded projects for planning and developing curriculum for the mentally retarded. Discussed are the initial steps of identifying the target population, needs, and objectives. Examples of curriculum design, including the rationale and conceptual framework, are provided. Considered are elements of…

Total Habilitation as a Major Goal of Intervention in Mental Retardation.

ERIC Educational Resources Information Center

Drash, Philip W.; And Others

1987-01-01

Intervention advances in mental retardation have enabled some individuals to function as independent, nonretarded persons. Establishing total habilitation (a term needed to describe this outcome) as a major goal of intervention will promote research and the development of an intervention continuum that includes prevention, total habilitation, and…

Building a Network of Services: 1963-1971. Final Report: Greater Cleveland Mental Retardation Development Project.

ERIC Educational Resources Information Center

Buckman, Rilma Oxley

The final report documents the Greater Cleveland Mental Retardation Development Project, a 5-year demonstration project concerned with solving such problems as how a community can provide all the services and programs needed by the retarded and their families, ensure successful and appropriate employment, and staff and finance such programs.…

Contraception or eugenics? Sterilization and "mental retardation" in the 1970s and 1980s.

PubMed

Ladd-Taylor, Molly

2014-01-01

Nonconsensual sterilization is usually seen as the by-product of a classist and racist society; disability is ignored. This article examines the 1973 sterilization of two young black girls from Alabama and other precedent-setting court cases involving the sterilization of "mentally retarded" white women to make disability more central to the historical analysis of sterilization. It analyzes the concept of mental retardation and the appeal of a surgical solution to birth control, assesses judicial deliberations over the "right to choose" contraceptive sterilization when the capacity to consent is in doubt, and reflects on the shadow of eugenics that hung over the sterilization debate in the 1970s and 1980s.

EFFECTS OF A COMPREHENSIVE OPPORTUNITY PROGRAM ON THE DEVELOPMENT OF EDUCABLE MENTALLY RETARDED CHILDREN.

ERIC Educational Resources Information Center

SMITH, LLOYD L.; STROUD, JAMES B.

THIS STUDY INVESTIGATED THE EFFECTS UPON EDUCABLE MENTALLY RETARDED CHILDREN OF A COMPREHENSIVE OPPORTUNITY PROGRAM EMBRACING NOT ONLY GOOD INSTRUCTION, BUT ALSO PSYCHOTHERAPY, A GOOD MENTAL-HYGIENE ATMOSPHERE, AND WHOLESOME AND EFFECTIVE HUMAN RELATIONSHIPS IN SCHOOL, HOME, AND COMMUNITY. THE MAJOR OBJECTIVES WERE--(1) TO SELECT AND/OR DEVELOP…

Starter Kit: Instructional Activities for Educable and Trainable Mentally Retarded Pupils.

ERIC Educational Resources Information Center

Drain, Theodore R.; And Others

Provided are 225 instructional activities for educable and trainable mentally retarded children from primary through secondary levels in the areas of social skills, communication skills, and number skills. Each page of the kit is divided into the following headings: activities (listed from simplest to most difficult), correlated activities (to…

National Study of Public Spending for Mental Retardation and Developmental Disabilities.

ERIC Educational Resources Information Center

Braddock, David; And Others

1987-01-01

Results of a nationwide study of public mental retardation/developmental disabilities spending in the states during Fiscal Years 1977 through 1986 were analyzed and identified trends such as continuing growth in spending for community services, contraction of total spending for institutional operations, and predominance of support for intermediate…

Mastery Learning of Stunts and Tumbling Activities for the Mentally Retarded.

ERIC Educational Resources Information Center

Chambless, Jim R.; And Others

Intended for physical education teachers of the handicapped, teacher educators at teacher training institutions, and research workers in areas of the handicapped, the document reports the effects of a theoretical model of teaching psychomotor tasks on the motor behavior of mentally retarded children. Chapter 1 introduces a psychomotor teaching…

Cardiovascular Disease Prevalence and Risk Factors of Persons with Mental Retardation

ERIC Educational Resources Information Center

Draheim, Christopher C.

2006-01-01

This paper reviews the recent literature on cardiovascular disease (CVD) prevalence, CVD-related mortality, physiological CVD risk factors, and behavioral CVD risk factors in adults with mental retardation (MR). The literature on the potential influences of modifiable behavioral CVD risk factors and the physiological CVD risk factors are also…

Teaching Meal Planning to Youth with Mental Retardation in Natural Settings.

ERIC Educational Resources Information Center

Arnold-Reid, Gae S.; And Others

1997-01-01

Three adolescent roommates with mild mental retardation were provided training in planning nutritious meals based on the food groups. A multiple probe design across individuals was used to evaluate training efficacy. Results indicated that the training procedures were effective in increasing healthy food choices. All participants maintained 100%…

A Report of Two Cases of Morquio's Syndrome Associated with Mental Retardation.

ERIC Educational Resources Information Center

Kumar, Y. Vikram; And Others

1979-01-01

The paper details the case studies of two siblings, a boy aged 7 and a girl aged 5 1/2, who suffer from Morquio's Syndrome (a disease characterized by cartilagenous and bony abnormalities) as well as mental retardation. (PHR)

Oral Rehabilitation and Management of Mentally Retarded

PubMed Central

Khetan, Jitendra; Gupta, Sarika; Tomar, Deepak; Singh, Meenakshi

2015-01-01

High level of periodontal problems of dental caries are frequently observed in mentally handicapped children. This group of patients presents various problems when they face dental treatments. Identification of such population and providing them affordable oral health care is the new concept. A systematic method for identification and screening of persons with mental retardation has been developed and is being followed. Cost and fear are the most commonly cited barriers to dental care. Physical or mental may lead to deterioration in self-care, and oral care state have a low priority. Risk factors are inter-related and are often barriers to oral health. With advancements in today’s world sufficient information and support is available for each and every individual to lead a healthy life which include the access to the oral health care. Factors such as fear, anxiety and dental phobia plays a vital role in acceptance of dental care and also the delaying of dental care. Lack of knowledge of oral and dental disease, awareness or oral need, oral side-effects of medication and organization of dental services are highlighted in the literature. All health personnel should receive training to support the concept of primary oral health care. Training about dealing with such mentally handicapped people should be addressed urgently among the health professionals. PMID:25738098

An Evaluation of in Vivo Desensitization and Video Modeling to Increase Compliance with Dental Procedures in Persons with Mental Retardation

ERIC Educational Resources Information Center

Conyers, Carole; Miltenberger, Raymond G.; Peterson, Blake; Gubin, Amber; Jurgens, Mandy; Selders, Andrew; Dickinson, Jessica; Barenz, Rebecca

2004-01-01

Fear of dental procedures deters many individuals with mental retardation from accepting dental treatment. This study was conducted to assess the effectiveness of two procedures, in vivo desensitization and video modeling, for increasing compliance with dental procedures in participants with severe or profound mental retardation. Desensitization…

Drosophila Torsin Protein Regulates Motor Control and Stress Sensitivity and Forms a Complex with Fragile-X Mental Retardation Protein

PubMed Central

Ahn, Hyo-Min; Koh, Young Ho

2016-01-01

We investigated unknown in vivo functions of Torsin by using Drosophila as a model. Downregulation of Drosophila Torsin (DTor) by DTor-specific inhibitory double-stranded RNA (RNAi) induced abnormal locomotor behavior and increased susceptibility to H2O2. In addition, altered expression of DTor significantly increased the numbers of synaptic boutons. One important biochemical consequence of DTor-RNAi expression in fly brains was upregulation of alcohol dehydrogenase (ADH). Altered expression of ADH has also been reported in Drosophila Fragile-X mental retardation protein (DFMRP) mutant flies. Interestingly, expression of DFMRP was altered in DTor mutant flies, and DTor and DFMRP were present in the same protein complexes. In addition, DTor and DFMRP immunoreactivities were partially colocalized in several cellular organelles in larval muscles. Furthermore, there were no significant differences between synaptic morphologies of dfmrp null mutants and dfmrp mutants expressing DTor-RNAi. Taken together, our evidences suggested that DTor and DFMRP might be present in the same signaling pathway regulating synaptic plasticity. In addition, we also found that human Torsin1A and human FMRP were present in the same protein complexes, suggesting that this phenomenon is evolutionarily conserved. PMID:27313903