Chapter 4 Alzheimer's Disease in Adults with Down Syndrome

International Review of Research in Mental Retardation

Volumn 36, Issue , 2008, Pages 103-145

  Zigman, Warren B ^a   Devenny, Darlynne A ^a   Krinsky McHale, Sharon J ^a   Jenkins, Edmund C ^a   Urv, Tiina K ^b   Wegiel, Jerzy ^a   Schupf, Nicole ^a,c,d   Silverman, Wayne ^e,f  

^a NEW YORK STATE INSTITUTE FOR BASIC RESEARCH IN DEVELOPMENTAL DISABILITIES   (United States)

^b EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH AND HUMAN DEVELOPMENT   (United States)

^c Columbia University College of Physicians and Surgeons ^*  (United States)

^d NewYork Presbyterian Hospital   (United States)

^e KENNEDY KRIEGER INSTITUTE   (United States)

^f JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

EID: 58649091967     PISSN: 00747750     EISSN: None     Source Type: Book Series    
DOI: 10.1016/S0074-7750(08)00004-9     Document Type: Review

References (179)

1. Albert M., and Cohen C. The test for severe impairment: An instrument for the assessment of patients with severe cognitive dysfunction. Journal of the American Geriatrics Society 40 (1992) 449-453
2. Alzheimer's Association. Alzheimer's disease facts and figures (2007), Author, Chicago, IL 1-29
3. American Psychiatric Association. Diagnostic and statistical manual of mental disorders. 4th ed. (1994), Author, Washington, DC
4. Arvanitakis Z., Schneider J.A., Wilson R.S., Bienias J.L., Kelly J.F., Evans D.A., et al. Statins, incident Alzheimer disease, change in cognitive function, and neuropathology. Neurology 70 (2008) 1795-1802
5. Aylward E.H., Burt D.B., Thorpe L.U., Lai F., and Dalton A. Diagnosis of dementia in individuals with intellectual disability. Journal of Intellectual Disability Research 41 (1997) 152-164
6. Baddeley A. The episodic buffer: A new component of working memory. Trends in Cognitive Science 4 (2000) 417-423
7. Barcikowska M., Silverman W., Zigman W.B., Kozlowski P.B., Kujawa M., Rudelli R., et al. Alzheimer-type neuropathology and clinical symptoms of dementia in mentally retarded people without Down syndrome. American Journal on Mental Retardation 93 (1989) 551-557
8. Beery K., and Buktenica N. Developmental test of visual-motor integration (1989), Modern Curriculum Press, Cleveland, OH
9. Berg L. Clinical Dementia Rating (CDR). Psychopharmacology Bulletin 24 (1988) 637-639
10. Bittles A.H., Petterson B.A., Sullivan S.G., Hussain R., Glasson E.J., and Montgomery P.D. The influence of intellectual disability on life expectancy. Journals of Gerontology Series A, Biological Sciences and Medical Sciences 57 (2002) M470-472
11. Blennow K., and Hampel H. CSF markers for incipient Alzheimer's disease. Lancet Neurology 2 (2003) 605-613
12. Blessed G., Tomlinson B.E., Roth M., et al. The association between quantitative measures of dementia and of senile change in the cerebral grey matter of elderly subjects. British Journal of Psychiatry 114 (1968) 797-811
13. Brookmeyer R., Johnson E., Ziegler-Graham K., and Arrighi H.M. Forecasting the global burden of Alzheimer's disease (2007), Berkeley Electronic Press, Baltimore
14. Burt D.B., and Aylward E.H. Test battery for the diagnosis of dementia in individuals with intellectual disability. Working group for the establishment of criteria for the diagnosis of dementia in individuals with intellectual disability. Journal of Intellectual Disability Research 44 (2000) 175-180
15. Burt D.B., Loveland K.A., Primeaux-Hart S., Chen Y.W., Phillips N.B., Cleveland L.A., et al. Dementia in adults with down syndrome: Diagnostic challenges. American Journal on Mental Retardation 103 (1998) 130-145
16. Buschke H. Selective reminding for analysis of memory and learning. Journal of Verbal Learning and Verbal Behavior 12 (1973) 543-550
17. Buschke H. Cued recall in amnesia. Journal of Clinical Neuropsychology 6 (1984) 433-440
18. Capone G.T. Down syndrome: Advances in molecular biology and the neurosciences. Journal of Developmental and Behavioral Pediatrics 22 (2001) 40-59
19. Carter G., and Jancar J. Mortality in the mentally handicapped: A 50 year survey at the stoke park group of hospitals (1930-1980). Journal of Mental Deficiency Research 27 (1983) 143-156
20. Chace C., Pang D., Ferin M., Mayeux R., Zigman W.B., Silverman W., et al. The long TTTA repeat length in CYP19 is associated with early age at onset of Alzheimer's disease in women with Down syndrome. Paper presented at the Hormones, Cognitive Function and Dementia: Expert Meeting (2007)
21. Collacott R.A., Cooper S.A., and Ismail I.A. Multi-infarct dementia in Down's syndrome. Journal of Intellectual Disability Research 38 (1994) 203-208
22. Corder E.H., Saunders A.M., Strittmatter W.J., Schmechel D.E., Gaskell P.C., Small G.W., et al. Gene dose of apolipoprotein E type 4 allele and the risk of Alzheimer's disease in late onset families. Science 261 (1993) 921-923
23. Corwin J., and Snodgrass J.G. The picture memory and fragmented pictures tests: Use with cognitively impaired populations. Psychopharmacology Bulletin 23 (1987) 286-290
24. Crome L. Morphological aspects of mental retardation. Proceedings of the Royal Society of Medicine 65 (1972) 587-588
25. de-Andrade F.M., Larrandaburu M., Callegari-Jacques S.M., Gastaldo G., and Hutz M.H. Association of apolipoprotein E polymorphism with plasma lipids and Alzheimer's disease in a Southern Brazilian population. Brazilian Journal of Medical and Biological Research 33 (2000) 529-537
26. Deb S., Braganza J., Norton N., Williams H., Kehoe P.G., Williams J., et al. APOE epsilon 4 influences the manifestation of Alzheimer's disease in adults with Down's syndrome. British Journal of Psychiatry 176 (2000) 468-472
27. Del Bo R., Comi G.P., Bresolin N., Castelli E., Conti E., Degiuli A., et al. The apolipoprotein E epsilon4 allele causes a faster decline of cognitive performances in Down's syndrome subjects. Journal of the Neurological Sciences 145 (1997) 87-91
28. Devenny D., Hill A.L., Patxot O., Silverman W.P., and Wisniewski K.E. Ageing in higher functioning adults with Down's syndrome: An interim report in a longitudinal study. Journal of Intellectual Disability Research 36 (1992) 241-250
29. Devenny D., Krinsky-McHale S., and Kittler P. Cognitive processes in early-stage dementia in adults with Down syndrome. Paper presented at the 9th World Down Syndrome Congress (2006)
30. Devenny D., Krinsky-McHale S.J., Sersen G., and Silverman W.P. Sequence of cognitive decline in dementia in adults with Down's syndrome. Journal of Intellectual Disability Research 44 (2000) 654-665
31. Devenny D., Silverman W.P., Hill A.L., Jenkins E., Sersen E.A., and Wisniewski K.E. Normal ageing in adults with Down's syndrome: A longitudinal study. Journal of Intellectual Disability Research 40 (1996) 208-221
32. Devenny D., Wegiel J., Schupf N., Jenkins E., Zigman W.B., Krinsky-McHale S.J., et al. Dementia of the Alzheimer's type and accelerated aging in Down syndrome. Journal 2005 14 (2005). DN1. Retrieved from http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed &dopt=Citation&list_uids=15814818
33. Devenny D., Zimmerli E.J., Kittler P., and Krinsky-McHale S.J. Cued recall in early-stage dementia in adults with Down's syndrome. Journal of Intellectual Disability Research 46 (2002) 472-483
34. Dupuy A.M., Mas E., Ritchie K., Descomps B., Badiou S., Cristol J.P., et al. The relationship between apolipoprotein E4 and lipid metabolism is impaired in Alzheimer's disease. Gerontology 47 (2001) 213-218
35. Edland S.D., Rocca W.A., Petersen R.C., Cha R.H., and Kokmen E. Dementia and Alzheimer disease incidence rates do not vary by sex in Rochester, Minn. Archives of Neurology 59 (2002) 1589-1593
36. Evans D.A., Funkenstein H.H., Albert M.S., Scherr P.A., Cook N.R., Chown M.J., et al. Prevalence of Alzheimer's disease in a community population of older persons. Higher than previously reported. Journal of the American Medical Association 262 (1989) 2551-2556
37. Evans R.M., Emsley C.L., Gao S., Sahota A., Hall K.S., Farlow M.R., et al. Serum cholesterol, APOE genotype, and the risk of Alzheimer's disease: A population-based study of African Americans. Neurology 54 (2000) 240-242
38. Evenhuis H. Manual of the dementia questionnaire for persons with mental retardation (DQMR). 2nd ed. (1995), Hooge Burch Centre for People With Intellectual Disability, Zwammerdam, the Netherlands
39. Evenhuis H.M. Evaluation of a screening instrument for dementia in ageing mentally retarded persons. Journal of Intellectual Disability Research 36 (1992) 337-347
40. Evenhuis H.M. Further evaluation of the Dementia Questionnaire for Persons with Mental Retardation (DMR). Journal of Intellectual Disability Research 40 (1996) 369-373
41. Folstein M.F., Folstein S.E., and McHugh P.R. "Mini-mental state". A practical method for grading the cognitive state of patients for the clinician. Journal of Psychiatric Research 12 (1975) 189-198
42. Fraser J., and Mitchell A. Kalmuc idiocy: Report of a case with autopsy with notes on sixty-two cases. Journal of Mental Science 22 (1876) 169-179
43. Freeman S.B., Allen E.G., Oxford-Wright C.L., Tinker S.W., Druschel C., Hobbs C.A., et al. The National Down syndrome project: Design and implementation. Public Health Reports 122 (2007) 62-72
44. Gardner R.J.M., and Sutherland G.R. Chromosome abnormalities and genetic counseling (2004), Oxford University Press, USA
45. Glasson E.J., Sullivan S.G., Hussain R., Petterson B.A., Montgomery P.D., and Bittles A.H. The changing survival profile of people with Down's syndrome: Implications for genetic counselling. Clinical Genetics 62 (2002) 390-393
46. Green R.C., Jayakumar P., Benke K., and Farrer L. Statin use is associated with reduced risk of Alzheimer's disease. Neurobiology of Aging 23 (2002) S273-274
47. Hardy J., and Allsop D. Amyloid deposition as the central event in the aetiology of Alzheimer's disease. Trends in Pharmacological Sciences 12 (1991) 383-388
48. Haxby J.V. Neuropsychological evaluation of adults with Down's syndrome: Patterns of selective impairment in non-demented old adults. Journal of Mental Deficiency Research 33 (1989) 193-210
49. Head E., Lott I.T., Patterson D., Doran E., and Haier R.J. Possible compensatory events in adult Down syndrome brain prior to the development of Alzheimer disease neuropathology: Targets for nonpharmacological intervention. Journal of Alzheimer's Disease 11 (2007) 61-76
50. Hoshino T., Kamino K., and Matsumoto M. Gene dose effect of the APOE-epsilon4 allele on plasma HDL cholesterol level in patients with Alzheimer's disease. Neurobiology of Aging 23 (2002) 41-45
51. Hoskin E.K., Tang M.X., Manly J.J., and Mayeux R. Elevated sex-hormone binding globulin in elderly women with Alzheimer's disease. Neurobiology of Aging 25 (2004) 141-147
52. Hyman B.T., West H.L., Rebeck G.W., Lai F., and Mann D.M. Neuropathological changes in Down's syndrome hippocampal formation. Effect of age and apolipoprotein E genotype. Archives of Neurology 52 (1995) 373-378
53. Isbir T., Agachan B., Yilmaz H., Aydin M., Kara I., Eker E., et al. Apolipoprotein-E gene polymorphism and lipid profiles in Alzheimer's disease. American Journal of Alzheimer's Disease and Other Dementias 16 (2001) 77-81
54. In: Janicki M., and Wisniewski H.M. (Eds). Aging and developmental disabilities: Issues and approaches (1985), Paul H. Brookes, Baltimore
55. Jenkins E., Velinov M., Ye L., Gu H., Pang D., Devenny D., et al. Telomere shortening in T lymphocyte metaphases and interphases and individual chromosomes 21 and 1, from older individuals with Down syndrome and dementia. Paper presented at the American Society of Human Genetics Annual Meeting from (2006). www.ashg.org/genetics/ashg06s
56. Jenkins E., Ye L., Gu H., Ni S., Velinov M., Pang D., et al. Shorter telomeres may indicate dementia status in older individuals with Down syndrome. Neurobiology of Aging (2008 accepted)
57. Jenkins E., Ye L., Gu H., Pang D., Devenny D., Zigman W., et al. Telomere shortening and/or "absence" may indicate dementia/AD status in older individuals with Down syndrome. Journal of the Alzheimer's Association 3 (2007) S109-S110
58. Jenkins E.C., Velinov M.T., Ye L., Gu H., Li S., Jenkins Jr. E.C., et al. Telomere shortening in T lymphocytes of older individuals with Down syndrome and dementia. Neurobiology of Aging 27 (2006) 941-945
59. Jervis G.A. Early senile dementia in mongoloid idiocy. American Journal of Psychiatry 105 (1948) 102-106
60. Jick H., Zornberg G.L., Jick S.S., Seshadri S., and Drachman D.A. Statins and the risk of dementia. Lancet 356 (2000) 1627-1631
61. Jorm A. The epidemiology of Alzheimer's disease. Paper presented at the the First Shizuoka Forum on Health and Longevity (1996)
62. Kida E., Wisniewski K.E., and Wisniewski H.M. Early amyloid-beta deposits show different immunoreactivity to the amino- and carboxy-terminal regions of beta-peptide in Alzheimer's disease and Down's syndrome brain. Neuroscience Letters 193 (1995) 105-108
63. Kittler P., Krinsky-McHale S.J., and Devenny D.A. Semantic and phonological loop effects on verbal working memory in middle-age adults with mental retardation. American Journal on Mental Retardation 109 (2004) 467-480
64. Kittler P., Krinsky-McHale S.J., and Devenny D.A. Verbal intrusions precede memory decline in adults with Down syndrome. Journal of Intellectual Disability Research 50 (2006) 1-10
65. Kivipelto M., Helkala E.L., Laakso M.P., Hanninen T., Hallikainen M., Alhainen K., et al. Midlife vascular risk factors and Alzheimer's disease in later life: Longitudinal, population based study. British Medical Journal 322 (2001) 1447-1451
66. Korenberg J.R., Chen X.N., Schipper R., Sun Z., Gonsky R., Gerwehr S., et al. Down syndrome phenotypes: The consequences of chromosomal imbalance. Proceedings of the National Academy of Sciences of the United States of America 91 (1994) 4997-5001
67. Krinsky-McHale S.J., Devenny D.A., Gu H., Jenkins E.C., Kittler P., Murty V.V., et al. Successful aging in a 70-year-old man with down syndrome: A case study. Intellectual and Developmental Disabilities 46 (2008) 215-228
68. Krinsky-McHale S.J., Devenny D.A., Kittler P., and Silverman W. Implicit memory in aging adults with mental retardation with and without Down syndrome. American Journal on Mental Retardation 108 (2003) 219-233
69. Krinsky-McHale S.J., Devenny D.A., and Silverman W.P. Changes in explicit memory associated with early dementia in adults with Down's syndrome. Journal of Intellectual Disability Research 46 (2002) 198-208
70. Krinsky-McHale S.J., Kittler P.K., Devenny D.A., and Silverman W. Is implicit memory resilient to the effects of dementia in adults with DS?. Paper presented at the 38th Annual Gatlinburg Conference on Research and Theory in Mental Retardation and Developmental Disabilities, Annapolis, MD (2005, March)
71. Lai F., Kammann E., Rebeck G.W., Anderson A., Chen Y., and Nixon R.A. APOE genotype and gender effects on Alzheimer disease in 100 adults with Down syndrome. Neurology 53 (1999) 331-336
72. Lakin K.C., and Stancliffe R.J. Residential supports for persons with intellectual and developmental disabilities. Mental Retardation and Developmental Disabilities Research Reviews 13 (2007) 151-159
73. Lambert J.C., Mann D., Goumidi L., Harris J., Amouyel P., Iwatsubo T., et al. Effect of the APOE promoter polymorphisms on cerebral amyloid peptide deposition in Alzheimer's disease. Lancet 357 9256 (2001) 608-609
74. Landesman-Dwyer S. Living in the community. American Journal of Mental Deficiency 86 (1981) 223-234
75. Launer L.J., White L.R., Petrovitch H., Ross G.W., and Curb J.D. Cholesterol and neuropathologic markers of AD: A population-based autopsy study. Neurology 57 (2001) 1447-1452
76. Lee J.H., Chulikavit M., Pang D., Zigman W.B., Silverman W., and Schupf N. Association between genetic variants in sortilin-related receptor 1 (SORL1) and Alzheimer's disease in adults with Down syndrome. Neuroscience Letters 425 (2007) 105-109
77. Lesko L.J., and Atkinson A.J. Use of biomarkers and surrogate endpoints in drug development and regulatory decision making: Criteria, validation, strategies. Annual Review of Pharmacology and Toxicology 41 (2001) 347
78. Leverenz J.B., and Raskind M.A. Early amyloid deposition in the medial temporal lobe of young Down syndrome patients: A regional quantitative analysis. Experimental Neurology 150 (1998) 296-304
79. Luckasson R., Borthwick-Duffy S., Buntix W.H.E., Coulter D.L., Craig E.M., Reeve A., et al. Mental retardation: Definition, classification and systems of supports. 10th Ed. (2002), American Association on Mental Retardation, Washington, DC:
80. Malamud N. Neuropathology of organic brain syndromes associated with aging. In: Gaitz C. (Ed). Aging and the brain (1972), Plenum, New York 63-87
81. Manly J.J., Schupf N., Tang M.X., and Stern Y. Cognitive decline and literacy among ethnically diverse elders. Journal of Geriatric Psychiatry and Neurology 18 (2005) 213-217
82. Mann D.M., and Esiri M.M. The pattern of acquisition of plaques and tangles in the brains of patients under 50 years of age with Down's syndrome. Journal of the Neurological Sciences 89 (1989) 169-179
83. Mausner J., and Kramer S. Mausner & Bahn epidemiology: An introductory text. 2nd ed. (1985), WB Saunders, Philadelphia
84. Mayeux R., Stern Y., Ottman R., Tatemichi T.K., Tang M.X., Maestre G., et al. The apolipoprotein epsilon 4 allele in patients with Alzheimer's disease. Annals of Neurology 34 (1993) 752-754
85. McCarthy D. Scales of children's abilities (1972), Psychological Corporation, San Antonio, TX
86. Miniño A., Heron M., and Smith B. Deaths: Preliminary data for 2004: Health E-stats (2006), National Center for Health Statistics, Hyattsville
87. Murakami N., Xie W., Lu R.C., Chen-Hwang M.C., Wieraszko A., and Hwang Y.W. Phosphorylation of amphiphysin I by minibrain kinase/dual-specificity tyrosine phosphorylation-regulated kinase, a kinase implicated in Down syndrome. Journal of Biological Chemistry 281 (2006) 23712-23724
88. Naslund J., Haroutunian V., Mohs R., Davis K.L., Davies P., Greengard P., et al. Correlation between elevated levels of amyloid beta-peptide in the brain and cognitive decline. Journal of the American Medical Association 283 (2000) 1571-1577
89. Nihira K., Foster R., Shellhaas M., and Leland H. AAMD adaptive behavior scale (1974), American Association on Mental Deficiency, Washington, DC
90. Olson L.E., Roper R.J., Sengstaken C.L., Peterson E.A., Aquino V., Galdzicki Z., et al. Trisomy for the Down syndrome 'critical region' is necessary but not sufficient for brain phenotypes of trisomic mice. Human Molecular Genetics 16 (2007) 774-782
91. Panossian L.A., Porter V.R., Valenzuela H.F., Zhu X., Reback E., Masterman D., et al. Telomere shortening in T cells correlates with Alzheimer's disease status. Neurobiology of Aging 24 (2003) 77-84
92. Park J., Yang E.J., Yoon J.H., and Chung K.C. Dyrk1A overexpression in immortalized hippocampal cells produces the neuropathological features of Down syndrome. Molecular and Cellular Neurosciences 36 (2007) 270-279
93. Patel B.N., Pang D., Stern Y., Silverman W., Kline J.K., Mayeux R., et al. Obesity enhances verbal memory in postmenopausal women with Down syndrome. Neurobiology of Aging 25 (2004) 159-166
94. Patel B.N., Seltzer G.B., Wu H.S., and Schupf N. Effect of menopause on cognitive performance in women with Down syndrome. Neuroreport 12 (2001) 2659-2662
95. Penrose L.S. The incidence of Mongolism in the general population. Journal of Mental Science 95 (1949) 685-688
96. Perneczky R., Pohl C., Sorg C., Hartmann J., Tosic N., Grimmer T., et al. Impairment of activities of daily living requiring memory or complex reasoning as part of the MCI syndrome. International Journal of Geriatric Psychiatry 21 (2006) 158-162
97. Polvikoski T., Sulkava R., Haltia M., Kainulainen K., Vuorio A., Verkkoniemi A., et al. Apolipoprotein E, dementia, and cortical deposition of beta-amyloid protein. New England Journal of Medicine 333 19 (1995) 1242-1247
98. Popovitch E.R., Wisniewski H.M., Barcikowska M., Silverman W., Bancher C., Sersen E., et al. Alzheimer neuropathology in non-Down's syndrome mentally retarded adults. Acta Neuropathologica 80 (1990) 362-367
99. Prasher V., Sajith S., Rees S., Patel A., Tewari S., Schupf N., et al. Significant effect of APOE epsilon 4 genotype on the risk of dementia in Alzheimer's disease and mortality in persons with Down syndrome. International Journal of Geriatric Psychiatry 23 (2008) 1-7
100. Prasher V.P., Chowdhury T.A., Rowe B.R., and Bain S.C. ApoE genotype and Alzheimer's disease in adults with Down syndrome: Meta-analysis. American Journal on Mental Retardation 102 (1997) 103-110
101. Prasher V.P., and Chung M.C. Causes of age-related decline in adaptive behavior of adults with Down syndrome: Differential diagnoses of dementia. American Journal on Mental Retardation 101 (1996) 175-183
102. Prasher V.P., Chung M.C., and Haque M.S. Longitudinal changes in adaptive behavior in adults with Down syndrome: Interim findings from a longitudinal study. American Journal on Mental Retardation 103 (1998) 40-46
103. Prasher V.P., Farrer M.J., Kessling A.M., Fisher E.M., West R.J., Barber P.C., et al. Molecular mapping of Alzheimer-type dementia in Down's syndrome. Annals of Neurology 43 (1998) 380-383
104. Raz N., Torres I.J., Briggs S.D., Spencer W.D., Thornton A.E., Loken W.J., et al. Selective neuroanatomic abnormalities in Down's syndrome and their cognitive correlates: Evidence from MRI morphometry. Neurology 45 (1995) 356-366
105. Reisberg B., Ferris S.H., Shulman E., Steinberg G., Buttinger C., Sinaiko E., et al. Longitudinal course of normal aging and progressive dementia of the Alzheimer's type: A prospective study of 106 subjects over a 3.6 year mean interval. Progress in Neuro-Psychopharmacology and Biological Psychiatry 10 (1986) 571-578
106. Reiss S., Levitan G.W., and Szyszko J. Emotional disturbance and mental retardation: Diagnostic overshadowing. American Journal of Mental Deficiency 86 (1982) 567-574
107. Reiss S., and Valenti-Hein D. Development of a psychopathology rating scale for children with mental retardation. Journal of Consulting and Clinical Psychology 62 (1994) 28-33
108. Riley K.P., Snowdon D.A., Desrosiers M.F., and Markesbery W.R. Early life linguistic ability, late life cognitive function, and neuropathology: Findings from the Nun Study. Neurobiology of Aging 26 (2005) 341-347
109. Robakis N.K., Wisniewski H.M., Jenkins E.C., Devine-Gage E.A., Houck G.E., Yao X.L., et al. Chromosome 21q21 sublocalisation of gene encoding beta-amyloid peptide in cerebral vessels and neuritic (senile) plaques of people with Alzheimer disease and Down syndrome. Lancet 1 (1987) 384-385
110. Roe C.M., Xiong C., Miller J.P., and Morris J.C. Education and Alzheimer disease without dementia: Support for the cognitive reserve hypothesis. Neurology 68 (2007) 223-228
111. Rogaeva E., Meng Y., Lee J.H., Gu Y., Kawarai T., Zou F., et al. The neuronal sortilin-related receptor SORL1 is genetically associated with Alzheimer disease. Nature Genetics 39 (2007) 168-177
112. Romas S.N., Tang M.X., Berglund L., and Mayeux R. APOE genotype, plasma lipids, lipoproteins, and AD in community elderly. Neurology 53 (1999) 517-521
113. Ross M.H., Galaburda A.M., and Kemper T.L. Down's syndrome: Is there a decreased population of neurons. Neurology 34 (1984) 909-916
114. Royston M.C., Mann D., Pickering-Brown S., Owen F., Perry R., Raghavan R., et al. Apolipoprotein E epsilon 2 allele promotes longevity and protects patients with Down's syndrome from dementia. Neuroreport 5 (1994) 2583-2585
115. Rubinsztein D.C., Hon J., Stevens F., Pyrah I., Tysoe C., Huppert F.A., et al. Apo E genotypes and risk of dementia in Down syndrome. American Journal of Medical Genetics 88 (1999) 344-347
116. Ryoo S.R., Jeong H.K., Radnaabazar C., Yoo J.J., Cho H.J., Lee H.W., et al. DYRK1A-mediated hyperphosphorylation of Tau. A functional link between Down syndrome and Alzheimer disease. Journal of Biological Chemistry 282 (2007) 34850-34857
117. Sadowski M., Wisniewski H.M., Tarnawski M., Kozlowski P.B., Lach B., and Wegiel J. Entorhinal cortex of aged subjects with Down's syndrome shows severe neuronal loss caused by neurofibrillary pathology. Acta Neuropathologica 97 (1999) 156-164
118. Saunders A.M., Strittmatter W.J., Schmechel D., George-Hyslop P.H., Pericak-Vance M.A., Joo S.H., et al. Association of apolipoprotein E allele epsilon 4 with late-onset familial and sporadic Alzheimer's disease. Neurology 43 (1993) 1467-1472
119. Scarmeas N., Brandt J., Blacker D., Albert M., Hadjigeorgiou G., Dubois B., et al. Disruptive behavior as a predictor in Alzheimer disease. Archives of Neurology 64 (2007) 1755-1761
120. Schmidt-Sidor B., Wisniewski K.E., Shepard T.H., and Sersen E.A. Brain growth in Down syndrome subjects 15 to 22 weeks of gestational age and birth to 60 months. Clinical Neuropathology 9 (1990) 181-190
121. Schupf N. Genetic and host factors for dementia in Down's syndrome. British Journal of Psychiatry 180 (2002) 405-410
122. Schupf N., Kapell D., Lee J.H., Ottman R., and Mayeux R. Increased risk of Alzheimer's disease in mothers of adults with Down's syndrome. Lancet 344 (1994) 353-356
123. Schupf N., Kapell D., Lee J.H., Zigman W.B., Canto B., Tycko B., et al. Onset of dementia is associated with apolipoprotein E epsilon4 in Down's syndrome. Annals of Neurology 40 (1996) 799-801
124. Schupf N., Kapell D., Nightingale B., Lee J.H., Mohlenhoff J., Bewley S., et al. Specificity of the fivefold increase in AD in mothers of adults with Down syndrome. Neurology 57 (2001) 979-984
125. Schupf N., Kapell D., Nightingale B., Rodriguez A., Tycko B., and Mayeux R. Earlier onset of Alzheimer's disease in men with Down syndrome. Neurology 50 (1998) 991-995
126. Schupf N., Pang D., Patel B.N., Silverman W., Schubert R., Lai F., et al. Onset of dementia is associated with age at menopause in women with Down's syndrome. Annals of Neurology 54 (2003) 433-438
127. Schupf N., Patel B., Pang D., Zigman W.B., Silverman W., Mehta P.D., et al. Elevated plasma beta-amyloid peptide Abeta42, incident dementia and mortality in Down syndrome. Archives of Neurology 64 (2007) 1007-1013
128. Schupf N., Patel B., Silverman W., Zigman W.B., Zhong N., Tycko B., et al. Elevated plasma amyloid beta-peptide 1-42 and onset of dementia in adults with Down syndrome. Neuroscience Letters 301 (2001) 199-203
129. Schupf N., Winsten S., Patel B., Pang D., Ferin M., Zigman W.B., et al. Bioavailable estradiol and age at onset of Alzheimer's disease in postmenopausal women with Down syndrome. Neuroscience Letters 406 (2006) 298-302
130. Schupf N., Zigman W.B., Kapell D., Lee J.H., Kline J., and Levin B. Early menopause in women with Down's syndrome. Journal of Intellectual Disability Research 41 (1997) 264-267
131. Seltzer G.B., Schupf N., and Wu H.S. A prospective study of menopause in women with Down's syndrome. Journal of Intellectual Disability Research 45 (2001) 1-7
132. Shumaker S.A., Legault C., Kuller L., Rapp S.R., Thal L., Lane D.S., et al. Conjugated equine estrogens and incidence of probable dementia and mild cognitive impairment in postmenopausal women: Women's health initiative memory study. Journal of the American Medical Association 291 (2004) 2947-2958
133. Silverman W., Devenny D.A., Krinsky-McHale S.J., Ryan R.S., and Zigman W.B. Aging, dementia and cognitive decline in adults with Down syndrome. Paper presented at the 39th Annual Gatlinburg Conference on Research & Theory in Intellectual and Developmental Disabilities (2006)
134. Silverman W., Popovitch E., Schupf N., Zigman W.B., Rabe A., Sersen E., et al. Alzheimer neuropathology in mentally retarded adults: Statistical independence of regional amyloid plaque and neurofibrillary tangle densities. Acta Neuropathologica 85 (1993) 260-266
135. Silverman W., Schupf N., Zigman W.B., Devenny D., Miezejeski C., Schubert R., et al. Dementia in adults with mental retardation: Assessment at a single point in time. American Journal on Mental Retardation 109 (2004) 111-125
136. Silverman W., Zigman W.B., Kim H., Krinsky-McHale S., and Wisniewski H.M. Aging and dementia among adults with mental retardation and Down syndrome. Topics in Geriatric Rehabilitation 13 (1998) 49-64
137. Smith D.J., and Rubin E.M. Functional screening and complex traits: Human 21q22.2 sequences affecting learning in mice. Human Molecular Genetics 6 (1997) 1729-1733
138. Smith D.J., Stevens M.E., Sudanagunta S.P., Bronson R.T., Makhinson M., Watabe A.M., et al. Functional screening of 2 Mb of human chromosome 21q22.2 in transgenic mice implicates minibrain in learning defects associated with Down syndrome. Nature Genetics 16 (1997) 28-36
139. Snowdon D.A., Kemper S.J., Mortimer J.A., Greiner L.H., Wekstein D.R., and Markesbery W.R. Linguistic ability in early life and cognitive function and Alzheimer's disease in late life. Findings from the nun study. Journal of the American Medical Association 275 (1996) 528-532
140. Sparks D.L., Sabbagh M.N., Connor D.J., Lopez J., Launer L.J., Browne P., et al. Atorvastatin for the treatment of mild to moderate Alzheimer disease: Preliminary results. Archives of Neurology 62 (2005) 753-757
141. In: Stegman L.K. (Ed). Stedman's medical dictionary. 28th ed. (2006), Lippincott, Williams & Wilkins, Baltimore
142. Strauss D., and Zigman W.B. Behavioral capabilities and mortality risk in adults with and without Down syndrome. American Journal on Mental Retardation 101 (1996) 269-281
143. Struwe F. Histopathologische Untersuchungen über Entstehung und Wesen der senilen Plaques. Z Ges Neurol Psychiatr 122 (1929) 291-307
144. Tang M.X., Jacobs D., Stern Y., Marder K., Schofield P., Gurland B., et al. Effect of oestrogen during menopause on risk and age at onset of Alzheimer's disease. Lancet 348 (1996) 429-432
145. Teng E., Lu P.H., and Cummings J.L. Neuropsychiatric symptoms are associated with progression from mild cognitive impairment to Alzheimer's disease. Dementia and Geriatric Cognitive Disorders 24 (2007) 253-259
146. Teri L., Truax P., Logsdon R., Uomoto J., Zarit S., and Vitaliano P.P. Assessment of behavioral problems in dementia: The revised memory and behavior problems checklist. Psychology and Aging 7 (1992) 622-631
147. Urv T.K., Zigman W.B., and Silverman W. Maladaptive behaviors related to adaptive decline in aging adults with mental retardation. American Journal on Mental Retardation 108 (2003) 327-339
148. Urv T.K., Zigman W.B., and Silverman W. Maladaptive behaviors related to dementia status in adults with Down syndrome. American Journal on Mental Retardation 113 (2008) 73-86
149. van Oijen M., Hofman A., Soares H.D., Koudstaal P.J., and Breteler M.M. Plasma Abeta(1-40) and Abeta(1-42) and the risk of dementia: A prospective case-cohort study. Lancet Neurology 5 (2006) 655-660
150. van Schrojenstein Lantman-de Valk H.M., Haveman M.J., and Crebolder H.F. Comorbidity in people with Down's syndrome: A criteria-based analysis. Journal of Intellectual Disability Research 40 (1996) 385-399
151. Victor M., and Ropper A.H. Adams and Victor's principals of neurology. 7th ed. (2001), McGraw-Hill, New York
152. Wechsler D. Wechsler intelligence scale for children-revised (1974), Psychological Corporation, New York, NY
153. Wegiel J., Dowjat K., Kaczmarski W., Kuchna I., Nowicki K., Frackowiak J., et al. Contribution of overexpressed Mnb/Dyrk1A protein to neurofibrillary degeneration in Down syndrome. Acta Neuropathologica (2008, submitted) (in press)
154. Wegiel J., Kuchna I., Nowicki K., Frackowiak J., Mazur-Kolecka B., Imaki H., et al. Intraneuronal Abeta immunoreactivity is not a predictor of brain amyloidosis-beta or neurofibrillary degeneration. Acta Neuropathologica 113 (2007) 389-402
155. Wegiel J., Wisniewski H.M., Dziewiatkowski J., Popovitch E.R., and Tarnawski M. Differential susceptibility to neurofibrillary pathology among patients with Down syndrome. Dementia 7 (1996) 135-141
156. Wehr H., Parnowski T., Puzynski S., Bednarska-Makaruk M., Bisko M., Kotapka-Minc S., et al. Apolipoprotein E genotype and lipid and lipoprotein levels in dementia. Dementia and Geriatric Cognitive Disorders 11 (2000) 70-73
157. Whalley L.J., Starr J.M., Athawes R., Hunter D., Pattie A., and Deary I.J. Childhood mental ability and dementia. Neurology 55 (2000) 1455-1459
158. Wisniewski H.M., and Wegiel J. The neuropathology of Alzheimer's disease. Neuroimaging Clinics of North America 5 (1995) 45-57
159. Wisniewski K., Dalton A.J., McLachlan C., Wen G.Y., and Wisniewski H.M. Alzheimer's disease in Down's syndrome: Clinicopathologic studies. Neurology 35 (1985) 957-961
160. In: Wisniewski K., and Hill A. (Eds). Clinical aspects of dementia in mental retardation and developmental disabilities (1985), Brookes, Baltimore, MD
161. Wisniewski K., Wisniewski H.M., and Wen G.Y. Occurrence of neuropathological changes and dementia of Alzheimer's disease in Down's
162. Wisniewski K.E. Down syndrome children often have brain with maturation delay, retardation of growth, and cortical dysgenesis. American Journal of Medical Genetics Supplement 7 (1990) 274-281
163. World Health Organization. International Statistical Classification of Diseases and Health Related Problems (The) ICD-10 Second Edition (2005), Author
164. World Health Organization. World Population Prospects: The 2004 Revision (2005), Author
165. Yang Q., Rasmussen S.A., and Friedman J.M. Mortality associated with Down's syndrome in the USA from 1983 to 1997: A population-based study. Lancet 359 (2002) 1019-1025
166. Yip A.G., Brayne C., and Matthews F.E. Risk factors for incident dementia in England and Wales: The Medical Research Council Cognitive Function and Ageing Study. A population-based nested case-control study. Age and Ageing 35 (2006) 154-160
167. Zandi P.P., Sparks D.L., Khachaturian A.S., Tschanz J., Norton M., Steinberg M., et al. Do statins reduce risk of incident Dementia and Alzheimer disease?: The cache county study. Archives of General Psychiatry 62 (2005) 217-224
168. Zigman W.B., Jenkins E., Mehta P., Schupf N., Urv T., Silverman W., et al. Alzheimer's disease, survival, and genetics in the "oldest old" with Down syndrome. Paper presented at the Down Syndrome Research Foundation (Canada)/National Down Syndrome Society (US) New Directions in Down Syndrome Research Conference (2000)
169. Zigman W.B., Jenkins E.C., Tycko B., Schupf N., and Silverman W. Mortality is associated with apolipoprotein E epsilon4 in nondemented adults with Down syndrome. Neuroscience Letters 390 (2005) 93-97
170. Zigman W.B., and Lott I.T. Alzheimer's disease in Down syndrome: Neurobiology and risk. Mental Retardation and Developmental Disabilities Research Reviews 13 (2007) 237-246
171. Zigman W.B., Schupf N., Devenny D.A., Miezejeski C., Ryan R., Urv T.K., et al. Incidence and prevalence of dementia in elderly adults with mental retardation without Down syndrome. American Journal on Mental Retardation 109 (2004) 126-141
172. Zigman W.B., Schupf N., Haveman M., and Silverman W. The epidemiology of Alzheimer disease in intellectual disability: Results and recommendations from an international conference. Journal of Intellectual Disability Research 41 (1997) 76-80
173. Zigman W.B., Schupf N., Jenkins E.C., Urv T.K., Tycko B., and Silverman W. Cholesterol level, statin use and Alzheimer's disease in adults with Down syndrome. Neuroscience Letters 416 (2007) 279-284
174. Zigman W.B., Schupf N., Lubin R.A., and Silverman W.P. Premature regression of adults with Down syndrome. American Journal of Mental Deficiency 92 (1987) 161-168
175. Zigman W.B., Schupf N., Sersen E., and Silverman W. Prevalence of dementia in adults with and without Down syndrome. American Journal on Mental Retardation 100 (1996) 403-412
176. Zigman W.B., Schupf N., and Silverman W. Cognitive reserve and dementia risk in adults with Down syndrome. Paper presented at the 38th Annual Gatlinburg Conference on Research & Theory in Intellectual & Developmental Disabilities (2005)
177. Zigman W.B., Schupf N., Urv T., Zigman A., and Silverman W. Incidence and temporal patterns of adaptive behavior change in adults with mental retardation. American Journal on Mental Retardation 107 (2002) 161-174
178. Zigman W., Schupf N., Zigman A., and Silverman W. Aging and Alzheimer disease in people with mental retardation. In: Bray N.W. (Ed). International Review of Research in Mental Retardation Vol. 19 (1993), Academic Press, New York 41-70
179. Zigman W.B., Silverman W., and Wisniewski H.M. Aging and Alzheimer's disease in Down syndrome: Clinical and pathological changes. Mental Retardation and Developmental Disabilities Research Reviews 2 (1996) 73-79