Skip to main content Accessibility help
×
Home
Hostname: page-component-568f69f84b-d8fc5 Total loading time: 0.362 Render date: 2021-09-17T02:21:50.144Z Has data issue: true Feature Flags: { "shouldUseShareProductTool": true, "shouldUseHypothesis": true, "isUnsiloEnabled": true, "metricsAbstractViews": false, "figures": true, "newCiteModal": false, "newCitedByModal": true, "newEcommerce": true, "newUsageEvents": true }

The neuropsychology of mental retardation

Published online by Cambridge University Press:  26 February 2009

Margaret B. Pulsifer
Affiliation:
Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD 21287

Abstract

This critical review examines mental retardation (MR) from a neuropsychological perspective. Competing definitions of MR are discussed and the prevalence is estimated. Descriptions are given of idiopathic MR and the five major identifiable prenatal causes of MR: fetal alcohol syndrome, Down's syndrome, fragile X syndrome, Prader-Willi syndrome, and Angelman syndrome. Similarities and differences among syndromes are examined. Cognitive deficits common to all disorders were in attention, short-term memory, and sequential information processing, whereas language and visuospatial abilities were varied. Neuroanatomical abnormalities common to all disorders were in the hippocampus and cerebellum; individual disorders typically showed a unique pattern of other neurological abnormalities. Both knowledge of individual MR-related disorders and comparative research between disorders are important for researchers and clinicians. Further research is called for in both areas. (JINS, 1996, 2, 159–176.)

Type
Critical Review
Copyright
Copyright © The International Neuropsychological Society 1996

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Aase, J.M. (1994). Clinical recognition of FAS: Difficulties of detection and diagnosis. Alcohol Health & Research World, 18, 59.Google Scholar
Abel, E.L. (1990). Fetal alcohol syndrome. Oradell, NJ: Medical Economics Co., Inc.Google Scholar
American Psychiatric Association. (1980). Diagnostic and statistical manual of mental disorders (3rd ed.). Washington, DC: American Psychiatric Association.Google Scholar
American Psychiatric Association. (1994). Diagnostic and statistical manual of mental disorders (4th ed.). Washington, DC: American Psychiatric Association.Google Scholar
Angelman, H. (1965). “Puppet” children: A report on three cases. Developmental Medicine and Child Neurology, 7, 681688.CrossRefGoogle Scholar
Antonarakis, S.E. & the Down Syndrome Collaborative Group. (1991). Parental origin of the extra chromosome in Trisomy 21 as indicated by analysis of DNA polymorphisms. The New England Journal of Medicine, 324, 872976.CrossRefGoogle ScholarPubMed
Ashley, C.J., Wilkinson, K.D., Reines, D., & Warren, S.T. (1993). FMR1 protein: Conserved RNP family domains and selective RNA binding. Science, 262, 563566.CrossRefGoogle ScholarPubMed
Baird, P.A. & Sadovnick, A.D. (1985). Mental retardation in over half a million consecutive livebirths—An epidemiological study. American Journal of Mental Deficiency, 89, 323330.Google ScholarPubMed
Bartel, N., Bryen, D., & Keehn, S. (1973). Language comprehension in the mentally retarded child. Exceptional Child, 39, 375382.CrossRefGoogle Scholar
Bellugi, U., Bihrle, A., Neville, H., Jernigan, T.L., & Doherty, S. (1992). Language cognition and brain organization in a neurodevelopmental disorder. In Gunnar, M. & Nelson, C. (Eds.), Developmental behavioral neuroscience (pp. 201232). Hillsdale, NJ: Erlbaum Press.Google Scholar
Boyd, S.G., Harden, A., & Patton, M.A. (1988). The EEG in early diagnosis of the Angelman (happy puppet) syndrome. European Journal of Pediatrics, 147(5), 508513.CrossRefGoogle ScholarPubMed
Bregman, J.D., Leckman, J.F., & Ort, S.I. (1988). Fragile X syndrome: Genetic predisposition to psychopathology. Journal of Autism and Developmental Disorders, 18, 343354.CrossRefGoogle ScholarPubMed
Broman, S., Nichols, P.L., Shaughnessy, P., & Kennedy, W. (1987). Retardation in young children: A developmental study of cognitive deficit. Hillsdale, NJ: Erlbaum Press.Google Scholar
Burack, J.A., Hodapp, R.M., & Zigler, E. (1988). Issues in the classification of mental retardation: Differentiating among organic etiologies. Journal of Child Psychology and Psychiatry, 29, 765779.CrossRefGoogle ScholarPubMed
Burack, J.A. & Zigler, E. (1990). Intentional and incidental memory in organically mentally retarded, familial retarded, and nonretarded individuals. American Journal on Mental Retardation, 94, 532540.Google ScholarPubMed
Carr, J. (1970). Mental and motor development in young mongol children. Journal of Mental Deficiency Research, 14, 205220.Google ScholarPubMed
Cassidy, S.B. (1984). Prader-Willi syndrome. Current Problems in Pediatrics, 14, 155.CrossRefGoogle ScholarPubMed
Chavez, G.F., Cordero, J.F., & Becerra, J.E. (1988). Leading major congenital malformations among minority groups in the United States, 1981–1986. Morbidity and Mortality Weekly Report: CDC Surveillance Summaries, 37, 1724.Google ScholarPubMed
Clarren, S.K. (1986). Neuropathology in fetal alcohol syndrome. In West, J.R. (Ed.), Alcohol and brain development (pp. 158166). New York: Oxford University Press.Google Scholar
Clarren, S.K. & Smith, D.W. (1978). The fetal alcohol syndrome. New England Journal of Medicine, 298, 10631067.CrossRefGoogle ScholarPubMed
Clayton-Smith, J. (1993). Clinical research on Angelman syndrome in the United Kingdom: Observations on 82 affected individuals. American Journal of Medical Genetics, 46, 1215.CrossRefGoogle ScholarPubMed
Clayton-Smith, J. & Pembrey, M.E. (1992). Angelman syndrome. Journal of Medical Genetics, 29, 412415.CrossRefGoogle ScholarPubMed
Coles, C. (1994). Critical periods for prenatal alcohol exposure. Alcohol Health & Research World, 18, 2229.Google Scholar
Coles, C.D., Brown, R.T., Smith, I.E., Platzman, K.A., Erickson, S., & Falek, A. (1991). Effects of prenatal alcohol exposure at school age: I. Physical and cognitive development. Neurotoxicology and Teratology, 13(4), 357367.CrossRefGoogle ScholarPubMed
Conry, J. (1990). Neuropsychological deficits in fetal alcohol syndrome and fetal alcohol effects. Alcoholism: Clinical and Experimental Research, 14, 650655.CrossRefGoogle ScholarPubMed
Courchesne, E., Yeung-Courchesne, R., Press, G.A., Hesselink, J.R., & Jernigan, T.L. (1988). Hypoplasia of cerebellar vermal lobules VI and VII in autism. New England Journal of Medicine, 318, 13491354.CrossRefGoogle ScholarPubMed
Coyle, J.T, Oster-Granite, M.L., & Gearhart, J.D. (1986). The neurobiologic consequences of Down syndrome. Brain Research Bulletin, 16, 773787.CrossRefGoogle ScholarPubMed
Crnic, K.A., Sulzbacher, S., Snow, J., & Holm, V.A. (1980). Preventing mental retardation associated with gross obesity in the Prader-Willi syndrome. Pediatrics, 66, 787789.Google ScholarPubMed
Cronk, C., Crocker, A.C., Pueschel, S.M., Shea, A.M., Zachai, E., Pickens, G., & Reed, R.B. (1988). Growth charts for children with Down syndrome — 1 month to 18 years of age. Pediatrics, 81, 102110.Google ScholarPubMed
Cronwell, A. & Birch, H. (1969). Psychological and social development in home-reared children with Down’s syndrome (mongolism). American Journal of Mental Deficiency, 74, 341350.Google Scholar
Curfs, L.M.G., Wiegers, A.M., Sommers, J.R.M., Borghgraef, M., & Fryns, J.P. (1991). Strengths and weaknesses in the cognitive profile of youngsters with Prader-Willi syndrome. Clinical Genetics, 40, 430434.CrossRefGoogle ScholarPubMed
de la Cruz, F.F. (1985). Fragile X syndrome. American Journal of Mental Deficiency, 90, 119123.Google ScholarPubMed
Dicks-Mireaux, M.J. (1972). Mental development of infants with Down’s syndrome. American Journal of Mental Deficiency, 77, 2632.Google ScholarPubMed
Down, J.L. (1866). Observations on an ethnic classification of idiots. London Hospital, Clinical Lecture Report, 3, 259262.Google Scholar
Dunn, H.G. (1968). The Prader-Labhart-Willi syndrome: Review of the literature and report of nine cases. Acta Paediatrica Scandanavia, 186, 138.Google Scholar
Dykens, E.M., Hodapp, R.M., & Evans, D.W. (1994). Profiles and development of adaptive behavior in children with Down syndrome. American Journal on Mental Retardation, 98, 580587.Google ScholarPubMed
Dykens, E.M., Hodapp, R.M., & Leckman, J.F. (1987). Strengths and weaknesses in the intellectual functioning of males with fragile X syndrome. American Journal of Mental Deficiency, 92, 234236.Google ScholarPubMed
Dykens, E.M., Hodapp, R.M., & Leckman, J.F. (1989). Adaptive and maladaptive functioning of institutionalized and noninstitutionalized fragile X males. Journal of the American Academy of Child and Adolescent Psychiatry, 28, 427430.CrossRefGoogle ScholarPubMed
Dykens, E.M., Hodapp, R.M., Ort, S., Finucane, B., Shapiro, L., & Leckman, J. (1989). The trajectory of cognitive development in males with fragile X syndrome. Journal of the American Academy of Child and Adolescent Psychiatry, 28, 422428.CrossRefGoogle ScholarPubMed
Dykens, E.M., Hodapp, R.M., Walsh, K., & Nash, L. (1992a). Adaptive and maladaptive behavior in Prader-Willi syndrome. Journal of the American Academy of Child and Adolescent Psychiatry, 31(6), 11311136.CrossRefGoogle ScholarPubMed
Dykens, E.M., Hodapp, R.M., Walsh, K., & Nash, L. (1992b). Profiles, correlates and trajectories of intelligence in Prader-Willi syndrome. Journal of the American Academy of Child and Adolescent Psychiatry, 31(6), 11251130.CrossRefGoogle ScholarPubMed
Dykens, E.M., Leckman, J., Paul, R., & Watson, M. (1987). The cognitive, behavioral, and adaptive functioning of fragile X and non-fragile X retarded men. Journal of Autism and Developmental Disorders, 18, 4152.CrossRefGoogle Scholar
Epstein, C.J. (1989). Down syndrome. In Scriver, C.R., Beaudet, A.L., Sly, W.S., & Valle, P. (Eds.), The metabolic basis of inherited diseases (pp. 291396). New York: McGraw-Hill.Google Scholar
Fishler, K. & Koch, R. (1991). Mental development in Down syndrome mosaicism. American Journal on Mental Retardation, 96, 345351.Google ScholarPubMed
Fishler, K., Share, J., & Koch, R. (1964). Adaptation of Gesell developmental scales for evaluation of development in children with Down’s syndrome (mongolism). American Journal of Mental Deficiency, 68, 642646.Google Scholar
Fowler, A.E. (1990). Language abilities of children with Down syndrome: Evidence for a specific syntactic delay. In Cicchetti, D. & Beeghly, M. (Eds.), Children with Down syndrome: A developmental perspective (pp. 302328). New York: Cambridge University Press.CrossRefGoogle Scholar
Fowler, A.E., Gelman, R., & Gleitman, L.R. (1994). The course of language learning in children with Down syndrome: Longitudinal and language level comparisons with young normally developing children. In Tager-Flusberg, H. (Ed.), Constraints on language acquisition (pp. 91140). Hillsdale, NJ: Erlbaum Press.Google Scholar
Freund, L.S. (1994). Diagnosis and developmental issues for young children with fragile X syndrome. Infants and Young Children, 5(3), 3445.CrossRefGoogle Scholar
Freund, L.S. & Reiss, A.L. (1991). Cognitive profiles associated with fragile X syndrome in males and females. American Journal of Medical Genetics, 38, 542547.CrossRefGoogle ScholarPubMed
Freund, L.S., Reiss, A.L., & Abrams, M.T. (1993). Psychiatric disorders associated with Fragile X in the young female. Pediatrics, 91, 321329.Google ScholarPubMed
Gabel, S., Tarter, R.E., Gavaler, J., Golden, W.L., Hegedus, A.M., & Maier, B. (1986). Neuropsychological capacity of Prader-Willi children: General and specific aspects of impairment. Applied Research in Mental Retardation, 7, 459466.CrossRefGoogle Scholar
Gaffney, G.R., Tsai, L.Y., Kuperman, S., & Minchin, S. (1987). Cerebellar structure in autism. American Journal of Diseases in Childhood, 141, 13301332.Google ScholarPubMed
Gesell, A. & Amatruda, C.S. (1941). Developmental diagnosis. New York: Paul Hoeber, Inc.Google Scholar
Gibson, D. (1978). Down’s syndrome: The psychology of mongolism. New York: Cambridge University Press.Google Scholar
Greene, T., Ernhart, C.B., Sokol, R.J., Martier, S., Marler, M.R., Boyd, T.A., & Ager, J.A. (1991). Prenatal alcohol exposure and preschool physical growth: A longitudinal analysis. Alcoholism: Clinical and Experimental Research, 15, 905913.CrossRefGoogle ScholarPubMed
Greenswag, L.R. (1987). Adults with Prader-Willi syndrome: A survey of 232 cases. Developmental Medicine and Child Neurology, 29, 145152.CrossRefGoogle ScholarPubMed
Grossman, H.J. (Ed.). (1983). Classification of mental retardation (3rd revision). Washington, DC: American Association on Mental Deficiency.Google Scholar
Hagbcrg, B., Hagberg, G., Lcwerth, A., & Holmgren, G. (1981). Mild mental retardation in Swedish school children. II. Etiologic and pathogenic aspects. Acta Paediatrica Scandinavia, 70, 445452.CrossRefGoogle Scholar
Hagerman, R., Jackson, A., Levitas, A., Rimland, B., & Braden, M. (1986). An analysis of autism in 50 males with fragile X syndrome. American Journal of Medical Genetics, 23, 359374.CrossRefGoogle ScholarPubMed
Hagerman, R.J., Schreiner, R.A., Kemper, M.B., Wittenberger, M.D., Zahn, B., Habicht, K. (1989). Longitudinal IQ changes in fragile X males. American Journal of Medical Genetics, 33, 513518.CrossRefGoogle ScholarPubMed
Hagerman, R.J. (1991). Physical and behavioral phenotype. In Hagerman, R.J. & Silverman, A.C. (Eds.), Fragile X syndrome: Diagnosis, treatment and research (pp. 367). Baltimore: Johns Hopkins University Press.Google Scholar
Hall, B.D. & Smith, D.W. (1972). Prader-Willi syndrome. Journal of Pediatrics, 81, 286293.CrossRefGoogle ScholarPubMed
Harris, J.C. (1988). Psychological adaptation and psychiatric disorders in adolescents and young adults with Down syndrome. In Pueschel, S.M. (Ed.), The young person with Down syndrome: Transition from adolescence to adulthood (pp. 3552). Baltimore: Paul Brookes.Google Scholar
Haxby, J.V. & Shapiro, B. (1992). Longitudinal study of neuropsychological function in older adults with Down syndrome. In Nadel, L. & Epstein, C. (Eds.), Alzheimer’s disease and Down syndrome (pp. 3550). New York: Wiley-Liss.Google ScholarPubMed
Hayashi, M., Itoh, M., Kabasawa, Y., Hayashi, H., Satoh, J., & Morimatsu, Y. (1992). Drain and Development, 14, 5862.Google Scholar
Hayes, A. & Batshaw, M.L. (1993). Down syndrome. In Batshaw, M. (Ed.), Pediatric Clinics of North America: The child with developmental disabilities, 40,(3), 523535.CrossRefGoogle ScholarPubMed
Ho, H.Z., Glahn, T.J., & Ho, J.C. (1988). The fragile X syndrome. Developmental Medicine and Child Neurology, 30, 257261.CrossRefGoogle ScholarPubMed
Hodapp, R.M., Leckman, J.F., Dykens, E.M., Sparrow, S.S., Zelinsky, D.G., & Ort, S.I. (1992). K-ABC profiles in children with fragile X syndrome, Down syndrome, and nonspecific mental retardation. American Journal on Mental Retardation, 97, 3946.Google ScholarPubMed
Holm, V. (1981). The diagnosis of Prader-Willi syndrome. In Holm, V.Sulzbacher, S., & Pipes, P. (Eds.), Prader-Willi syndrome (pp. 2740). Baltimore: University Park Press.Google ScholarPubMed
Hook, E. (1982). The epidemiology of Down syndrome. In Pueschel, S. & Rynders, J. (Eds.), Down syndrome: Advances in biomedicine and the behavioral sciences (p. 11). Cambridge, MA: Ware Press.Google Scholar
Hynd, G.W., Semrud-Clikeman, M., Lorys, A.R., Novey, E.S., Eliopulos, D., & Lyytinen, H. (1991). Corpus callosum morphology in attention-deficit hyperactivity disorder: Morphometric analysis of MRI. Journal of Learning Disorders, 24, 141146.CrossRefGoogle ScholarPubMed
Jacobson, J.L. & Jacobson, S.W. (1994). Prenatal alcohol exposure and neurobehavioral development. Alcohol Health & Research World, 18, 30303036.Google Scholar
Jacobson, J.L., Jacobson, S.W., Sokol, R.J., Martier, S.S., Ager, J.W., & Kaplan-Estrin, M.G. (1993). Teratogenic effects of alcohol on infant development. Alcoholism: Clinical and Experimental Research, 17, 174183.CrossRefGoogle ScholarPubMed
Jeret, J.S., Serur, D., Wisniewski, K., & Fisch, C. (1986). Frequency of agenesis of the corpus callosum in the developmentally disabled population as determined by computerized tomography. Pediatric Neurosciences, 12, 101103.CrossRefGoogle Scholar
Jernigan, T.L., Bellugi, U., Sowell, E., Doherty, S., & Hesselink, J.R. (1993). Cerebral morphologic distinctions between Williams and Down syndromes. Archives of Neurology, 50, 186191.CrossRefGoogle ScholarPubMed
Johanson, A., Gustafson, L., Brun, A., Risberg, J., Rosen, I., & Tideman, E. (1991). A longitudinal study of dementia of Alzheimer type in Down’s syndrome. Dementia, 2, 159168.Google Scholar
Jones, K.L. & Smith, D.W. (1973). Recognition of the fetal alcohol syndrome in early infancy. The Lancet, 2, 9991001.CrossRefGoogle Scholar
Jones, K.L., Smith, D.W., Ulleland, C.N., & Streissguth, A.P. (1973). Pattern of malformation in offspring of chronic alcoholic mothers. The Lancet, 1, 12671271.CrossRefGoogle ScholarPubMed
Kaplan, L.C., Wharton, R., Elias, E., Mendell, F., Donlon, T., & Latt, S. (1987). Clinical heterogeneity associated with deletions in the long arm of chromosome 15: Report of 3 new cases and their possible genetic significance. American Journal of Medical Genetics, 28, 4553.CrossRefGoogle Scholar
Kaufman, A.S. & Kaufman, N.L. (1983). Kaufman Assessment Battery for Children. Circle Pines, MN: American Guidance Service.Google Scholar
Kiely, M. (1987). The prevalence of mental retardation. Epidemiological Review, 9, 194.CrossRefGoogle ScholarPubMed
Knoll, J.H., Nicholls, R.D., Magenis, R.E., & Graham, J.M. (1989). Angelman and Prader-Willi syndromes share a common chromosome 15 deletion but differ in parental origin of the deletion. American Journal of Medical Genetics, 32, 285290.CrossRefGoogle Scholar
Lai, F. & Williams, R.S. (1989). A prospective study of Alzheimer’s disease in Down syndrome. Archives of Neurology, 46, 849853.CrossRefGoogle ScholarPubMed
Landesman-Dwyer, S., Ragozin, A.S., & Little, R.E. (1981). Behavioral correlates of prenatal alcohol exposure: A four-year follow-up study. Neurobehavioral Toxicology and Teratology, 3, 187193.Google ScholarPubMed
Laurance, B.M., Brito, A., & Wilkinson, J. (1981). Prader-Willi syndrome after 15 years. Archives of Disease in Childhood, 56, 181186.CrossRefGoogle ScholarPubMed
Leifer, J. & Lewis, M. (1984). Acquisition of conversational response skills by young Down syndrome and non-retarded children. American Journal of Mental Deficiency, 88, 610618.Google Scholar
Lenneberg, E.H. (1967). Biological foundations of language. New York: Wiley.Google Scholar
Leonard, C.M., Williams, C.A., Nicholls, R.D., Agee, O.F., Voeller, K.K., Honeyman, J.C., & Staab, E.V. (1993). Angelman and Prader-Willi syndrome: A magnetic resonance imaging study of differences in cerebral structure. American Journal of Medical Genetics, 46(1), 2633.CrossRefGoogle ScholarPubMed
Lewis, A. (1953). Health as a social concept. British Journal of Sociology, 4, 109124.CrossRefGoogle Scholar
Lipkin, P. (1991). Epidemiology of the developmental disabilities. In Capute, A.J. & Accardo, P.J. (Eds.), Developmental Disabilities in Infancy and Childhood (pp. 4363). Baltimore: Paul Brookes.Google Scholar
Lott, I.T. & Lai, F.L. (1982). Dementia in Down syndrome. Annals of Neurology, 12, 210215.Google Scholar
Lubs, H.A. (1969). A marker X chromosome. American Journal of Human Genetics, 21, 231244.Google ScholarPubMed
Luckasson, R., Coulter, D.L., Polloway, E.A., Reiss, S., Schalock, R.L., Snell, M.E., Spitalnick, D.M., & Stark, J.A. (1992). Mental retardation: Definition, classification, and systems of support. Washington, DC: American Association on Mental Retardation.Google Scholar
Luria, A.R. (1966). Higher cortical functions in man. New York: Basic Books.Google Scholar
MacMillan, D.L., Gresham, F.M., & Siperstein, G.N. (1993). Conceptual and psychometric concerns about the 1992 AAMR definition of mental retardation. American Journal on Mental Retardation, 98, 325335.Google ScholarPubMed
Magenis, R., Brown, M.G., Lacy, D.A., Budden, S., & LaFranchi, S. (1987). Is Angelman syndrome an alternate result of del (15) (q11-q13)? American Journal of Medical Genetics, 28, 829838.CrossRefGoogle Scholar
Malamud, N. (1964). Neuropathology. In Stevens, H.A. & Heber, R. (Eds.), Mental retardation. A review of research (pp. 429452). Chicago: University of Chicago Press.Google Scholar
Malamud, N. (1973). Neuropathology of Down syndrome. Advances in Behavioral Biology, 3, 6387.CrossRefGoogle Scholar
Malcolm, S., Clayton-Smith, J., Nichols, M., Robb, S., Webb, T., Armour, J.A.L., Jeffreys, A.J., & Pembrey, M.E. (1991). Uniparental paternal disomy in Angelman’s syndrome. The Lancet, 337, 694697.CrossRefGoogle ScholarPubMed
Marcell, M.M. & Armstrong, V. (1982). Auditory and visual sequential memory of Down syndrome and nonretarded children. American Journal of Mental Deficiency, 87, 8695.Google ScholarPubMed
Mattson, S.N., Jernigan, T.L., & Riley, E.P. (1994). MRI and prenatal alcohol exposure. Alcohol Health and Research World, 18, 4954.Google Scholar
Mattson, S.N., Riley, E.P., Jernigan, T.L., Ehlers, C.L., Delis, D.C., Jones, K.L., Stern, C., Johnson, K.A., Hesselink, J.R., & Bellugi, U. (1992). Fetal alcohol syndrome: A case report of neuropsychological, MRI, and EEG assessment of two children. Alcoholism: Clinical and Experimental Research, 16, 10011003.CrossRefGoogle ScholarPubMed
Mazzocco, M.M.M., Pennington, B.F., & Hagerman, R.J. (1993). The neurocognitive phenotype of female carriers of fragile X: Additional evidence of specificity. Journal of Developmental and Behavioral Pediatrics, 14, 328335.CrossRefGoogle ScholarPubMed
McDonald, A.D. (1973). Severely retarded children in Quebec: Prevalence, causes and care. American Journal of Mental Deficiency, 78, 205215.Google Scholar
McLaren, J. & Bryson, S.E. (1987). Review of recent epidemiological studies of mental retardation: Prevalence, associated disorders, and etiology. American Journal of Mental Retardation, 92, 243254.Google ScholarPubMed
Melyn, M. & White, D. (1973). Mental and developmental milestones of noninstitutionalized Down’s syndrome children. Pediatrics, 52, 542545.Google ScholarPubMed
Menolascino, F.J. (1965). Psychiatric aspects of mongolism. American Journal of Mental Deficiency, 69, 653660.Google ScholarPubMed
Michaelis, E.K. & Michaelis, M.L. (1994). Cellular and molecular bases of alcohol’s teratogenic effects. Alcohol Health and Research World, 18, 1721.Google Scholar
Mikkelsen, M. (1977). Down syndrome: Cytogenetic epidemiology. Hereditas, 86, 4559.CrossRefGoogle Scholar
Moore, B.C. (1973). Some characteristics of institutionalized mongols. Journal of Mental Deficiency Research, 17, 4651.Google Scholar
Nanson, J.L. & Hiscock, M. (1990). Attention deficits in children exposed to alcohol prenatally. Alcoholism: Clinical and Experimental Research, 14, 656661.CrossRefGoogle ScholarPubMed
National Institute on Alcohol Abuse and Alcoholism. (1990). Seventh special report to the U.S. Congress on Alcohol and Health. DHHS Pub. No. (ADM)90–1656. Washington, DC: Superintendent of Documents, U.S. Government Printing Office.Google Scholar
Nicholls, R. (1993). Genomic imprinting and uniparental disomy in Angelman and Prader-Willi syndromes: A review. American Journal of Medical Genetics, 46, 1625.CrossRefGoogle ScholarPubMed
Oliver, C. & Holland, A.J. (1986). Down’s syndrome and Alzheimer’s disease: A review. Psychological Medicine, 16, 307322.CrossRefGoogle ScholarPubMed
Penner, K.A., Johnston, J., Faircloth, B.H., Irish, P., & Williams, C. (1993). Communication, cognition, and social interaction in the Angelman syndrome. American Journal of Medical Genetics, 46, 3439.CrossRefGoogle ScholarPubMed
Pueschel, S.M. (1990). Clinical aspects of Down syndrome from infancy to adulthood. American Journal of Medical Genetics, 7, 5256.Google Scholar
Pueschel, S.M., Gallagher, P.L., Zartler, A.S., & Pezzullo, J.C. (1987). Cognitive and learning processes in children with Down syndrome. Research in Developmental Disabilities, 8, 2137.CrossRefGoogle ScholarPubMed
Purpura, D.P. (1974). Dendritic spine “dysgencsis” and mental retardation. Science, 186, 11261128.CrossRefGoogle Scholar
Reiss, S. (1994). Issues in defining mental retardation. American Journal of Mental Retardation, 99, 17.Google ScholarPubMed
Reiss, A.L., Abrams, M.T., Greenlaw, R., Freund, L., & Denckla, M. (1995). Neurodevelopmental effects of the FMR-1 full mutation in humans. Nature Medicine, 1, 159167.CrossRefGoogle ScholarPubMed
Reiss, A.L. & Freund, L. (1990). Fragile X syndrome, DSM-III-R, and Autism. Journal of the American Academy of Child and Adolescent Psychiatry, 29, 885891.CrossRefGoogle ScholarPubMed
Reiss, A.L., Freund, L., Abrams, M.T., Boehm, C., & Kazazian, H. (1993). Neurobehavioral effects of the fragile X permutation in adult women: A controlled study. American Journal of Human Genetics, 52, 884894.Google Scholar
Reiss, A.L., Hagerman, R.J., Vingradov, S., Abrams, M., & King, R.J. (1988). Psychiatric disability in female carriers of the fragile X chromosome. Archives of General Psychiatry, 45, 2530.CrossRefGoogle ScholarPubMed
Rosett, H.L. (1980). A clinical perspective of the fetal alcohol syndrome. Alcoholism, 4, 119122.CrossRefGoogle ScholarPubMed
Rousseau, F., Heitz, D., Tarleton, J., MacPherson, J., Malmgren, H., Dahl, N., Barnicoat, A., Mathew, C., Mornet, E., Tejada, I., Maddalena, A., Spiegel, R., Schinzel, A., Marcos, J.A.G., Schorderet, D.F., Schapp, T., Maccioni, L., Russo, S., Jacobs, P.A., Schwartz, C., & Mandel, J.L. (1994). A multicenter study on genotype-phenotype correlations in the fragile X syndrome using direct diagnosis with probe StB12.3: The first 2,253 cases. American Journal of Human Genetics, 55, 225237.Google Scholar
Russell, M., Czarnecki, D.M., Cowan, R., McPhearson, E., & Mudar, P.J. (1991). Measures of maternal alcohol use as predictors of development in early childhood. Alcoholism: Clinical and Experimental Research, 15, 9911000.CrossRefGoogle ScholarPubMed
Rutter, M., Graham, P., & Yule, W. (1970). A neuropsychiatric study in childhood. London: SIMP with Heineman Medical.Google Scholar
Schaefer, G.B. & Bodensteincr, J.B. (1992). Evaluation of the child with idiopathic mental retardation. Pediatric Clinics of North America, 39(4), 929943.CrossRefGoogle Scholar
Share, J., Koch, R., Webb, A., & Graliker, B. (1964). The longitudinal development of infants and young children with Down’s syndrome (mongolism). American Journal of Mental Deficiency, 68, 685692.Google Scholar
Sherman, S. (1991). Epidemiology. In Hagerman, R.J. & Silverman, A.C. (Eds.), The fragile X syndrome: Diagnosis, treatment and research (pp. 6997). Baltimore: Johns Hopkins University Press.Google Scholar
Simko, A., Hornstein, L., Soukup, S., & Bagamery, N. (1989). Fragile X syndrome: Recognition in young children. Pediatrics, 83, 547552.Google ScholarPubMed
Smith, D.W. & Bostian, K.E. (1964). Congenital anomalies associated with idiopathic mental retardation. Journal of Pediatrics, 65, 189.CrossRefGoogle ScholarPubMed
SonBlomquist, H.K., Gustavson, K.H., & Holmgren, G. (1981). Mild mental retardation in children in a northern Swedish county. Journal of Mental Deficiency Research, 25, 169186.Google Scholar
Sparrow, S., Balla, D., & Cicchetti, D. (1984). Vineland Scales of Adaptive Behavior, survey form manual. Circle Pines, MN: American Guidance Service.Google Scholar
Spohr, H.L., Willms, J., & Steinhausen, H.C. (1993). Prenatal alcohol exposure and long-term developmental consequences. The Lancet, 341, 907910.CrossRefGoogle ScholarPubMed
Stein, D.J., Keating, J., Zar, H.J., & Hollander, E. (1994). A survey of the phenomenology and pharmacotherapy of compulsive and impulsive-aggressive symptoms in Prader-Willi syndrome. Journal of Neuropsychiatry, 6, 2329.Google ScholarPubMed
Streissguth, A.P., Aase, J.M., Clarren, S.K., Randels, S.P., LaDue, R.A., & Smith, D.F. (1991). Fetal alcohol syndrome in adolescents and adults. Journal of the American Medical Association, 265, 19611965.CrossRefGoogle ScholarPubMed
Streissguth, A.P., Barr, H.M., Olson, H.C., Sampson, P.D., Bookstein, F.L., & Burgess, D.M. (1994). Drinking during pregnancy decreases word attack and arithmetic scores on standardized tests: Adolescent data from a population-based prospective study. Alcoholism: Clinical and Experimental Research, 18, 248254.CrossRefGoogle ScholarPubMed
Streissguth, A.P., Barr, H.M., & Sampson, P.D. (1990). Moderate prenatal alcohol exposure: Effects on child IQ and learning problems at age 7½ years. Alcoholism: Clinical and Experimental Research, 14, 662669.CrossRefGoogle ScholarPubMed
Streissguth, A.P., Barr, H.M., Sampson, P.D., Parrish-Johnson, J.C., Kirchner, G.L., & Martin, D.C. (1986). Attention, distraction, and reaction time at age 7 years and prenatal alcohol exposure. Neurobehavioral Toxicology and Teratology, 8, 717725.Google ScholarPubMed
Streissguth, A.P., Randels, S.P., & Smith, D.F. (1991). A test-retest study of intelligence in patients with fetal alcohol syndrome: Implications for care. Journal of the American Academy of Child and Adolescent Psychiatry, 30, 584587.CrossRefGoogle Scholar
Sylvester, P.E. (1983). The hippocampus in Down’s syndrome. Journal of Mental Deficiency Research, 27, 227236.Google ScholarPubMed
Sylvester, P.E. (1986). The anterior commissure in Down’s syndrome. Journal of Mental Deficiency Research, 30, 1926.Google ScholarPubMed
Theobold, T., Hay, D., & Judge, C. (1987). Individual variation and specific cognitive deficits in the fragile X syndrome. American Journal of Medical Genetics, 28, 111.CrossRefGoogle Scholar
Thuline, H. & Pueschel, S. (1982). Cytogenetics in Down syndrome. In Pueschel, S. & Rynders, J. (Eds.), Down syndrome: Advances in biomedicine and behavioral sciences (p. 133). Cambridge, MA: Ware Press.Google Scholar
Varnhagen, C.K., Das, J.P., & Varnhagen, S. (1987). Auditory and visual memory span: Cognitive processing by TMR individuals with Down syndrome or other etiologies. American Journal of Mental Deficiency, 91, 398405.Google ScholarPubMed
Verkerk, A.J.M.H., Pieretti, M., Sutcliffe, J.S., Fu, Y.H., Kuhl, D.P.A., Pizzuti, A., Reiner, O., Richards, S., Victoria, M.F., Zhang, F., Riggins, G.J., Chastain, J.L., Kunst, C.B., Galjaard, H., Caskey, C.T., Nelson, D.L., Oostra, B.A., & Warren, S.T. (1991). Identification of a gene (FMR-1) containing a CGG repeat coincident with a breakpoint cluster region exhibiting length variation in fragile X syndrome. Cell, 65, 905914.CrossRefGoogle ScholarPubMed
Vitiello, B., Spreat, S., & Behar, D. (1989). Obsessive-compulsive disorder in mentally retarded patients. Journal of Nervous and Mental Disorders, 177, 232236.CrossRefGoogle ScholarPubMed
Warren, J. & Hunt, E. (1981). Cognitive processing in children with Prader-Willi syndrome. In Holm, V.Sulzbacher, S., & Pipes, P. (Eds.), Prader-Willi Syndrome (pp. 161177). Baltimore: University Park Press.Google Scholar
Williams, C., Hendrickson, J., Whidden, E., & Buehler, B. (1991). Facts About Angelman Syndrome. Gainesville, FL: Angelman Syndrome Foundation.Google Scholar
Wishart, J. (1988). Early learning in infants and young children with Down syndrome. In Nadel, L. (Ed.), The psychobiology of Down syndrome. Cambridge, MA: The MIT Press.Google Scholar
Wisniewski, K.E. (1990). Down syndrome children often have brains with maturational delay, retardation of growth, and cortical dysgenesis. American Journal of Medical Genetics, 7, 274281.Google Scholar
Wisniewski, K.E., Laure-Kamionowska, M., Connell, F., & Wen, G.Y. (1986). Neuronal density and synaptogenesis in the postnatal stage of brain maturation in Down syndrome. In Epstein, C.J. (Ed.), The Neurobiology of Down Syndrome (pp. 2943). New York: Raven Press.Google Scholar
Young, E.C. & Kramer, B.M. (1991). Characteristics of age-related language decline in adults with Down syndrome. Mental Retardation, 29, 7579.Google ScholarPubMed
Zellweger, H. (1977). Down syndrome. In Vinken, P.J. & Bruyn, G.W. (Eds.), Handbook of clinical neurology (pp. 367470). New York: North Holland Publishing.Google Scholar
Zellweger, H. (1981). Diagnosis and therapy in the first phase of Prader-Willi syndrome. In Holme, V.A.Sulzbacher, S.J., & Pipes, P. (Eds.), Prader-Willi syndrome. Baltimore: University Park Press.Google Scholar
Zellweger, H. & Schneider, H.J. (1968). Syndrome of hypotonia-hypomentia- hypogonadism-obesity (HHHO) or Prader-Willi syndrome. American Journal of Disease in Children, 115, 588598.Google ScholarPubMed
Zigler, E., Balla, D., & Hodapp, R.M. (1984). On the definition and classification of mental retardation. American Journal of Mental Deficiency, 89, 215230.Google ScholarPubMed
Zigler, E. & Hodapp, R. (1986). Understanding mental retardation. New York: Cambridge University Press.Google Scholar
86
Cited by

Send article to Kindle

To send this article to your Kindle, first ensure no-reply@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about sending to your Kindle. Find out more about sending to your Kindle.

Note you can select to send to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be sent to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

The neuropsychology of mental retardation
Available formats
×

Send article to Dropbox

To send this article to your Dropbox account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Dropbox.

The neuropsychology of mental retardation
Available formats
×

Send article to Google Drive

To send this article to your Google Drive account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your <service> account. Find out more about sending content to Google Drive.

The neuropsychology of mental retardation
Available formats
×
×

Reply to: Submit a response

Please enter your response.

Your details