Dementias and Mild Cognitive Impairment in Adults



Dementia does not describe a specific disease entity, but rather describes a clinical syndrome characterized by a loss of previously acquired cognitive functions that adversely affects an individual’s ability to complete day to day activities. The decline in cognitive functioning is greater than what occurs during the normal aging process. This chapter will review prominent definitions of dementia and a number of the etiologies of this syndrome. The prodromal phase between dementia and normal aging, Mild Cognitive Impairment (MCI), is reviewed later in this chapter. Readers interested in a more detailed review of dementia syndromes and conditions presenting as dementia are referred to Mendez and Cummings (Dementia: a clinical approach, 3rd edn, Butterworth Heinemann, Philadelphia, 2003).


Mild Cognitive Impairment Dementia With Lewy Body Progressive Supranuclear Palsy Normal Pressure Hydrocephalus Semantic Dementia 


  1. American Psychiatric Association.(2000). Diagnostic and statistical manual of mental disorders, 4th ed., text revision ed. Washington DC: American Psychiatric Association.Google Scholar
  2. Antinori, A., Arendt, G., Becker, J. T., et al. (2007). Updated research nosology for HIV-associated neurocognitive disorders. Neurology, 69, 1789–1799.PubMedCrossRefGoogle Scholar
  3. Ballard, C., O’Brien, J., Gray, A., et al. (2001). Attention and fluctuating attention in patients with dementia with Lewy bodies and Alzheimer disease. Archives of Neurology, 58, 977–982.PubMedCrossRefGoogle Scholar
  4. Baltes, P. B., & Mayer, K. U. (1999). The Berlin aging study: Aging from 70 to 100. Cambridge, England: Cambridge University Press.Google Scholar
  5. Barnes, D. E., Covinsky, K. E., Whitmer, R. A., Kuller, L. H., Lopez, O. L., & Yaffe, K. (2009). Predicting risk of dementia in older adults. The late-life dementia risk index. Neurology, 73, 173–179.PubMedCrossRefGoogle Scholar
  6. Beatty, W. W., Ryder, K. A., Gontkovsky, S. T., Scott, J. G., McSwan, K. L., & Bharucha, K. J. (2003). Analyzing the subcortical dementia syndrome of Parkinson’s disease using the RBANS. Archives of Clinical Neuropsychology, 18, 509–520.PubMedGoogle Scholar
  7. Berent, S., Giordani, B., Gilman, S., et al. (2002). Patterns of neuropsychological performance in multiple system atrophy compared to sporadic and hereditary olivopontocerebellar atrophy. Brain and Cognition, 50, 194–206.PubMedCrossRefGoogle Scholar
  8. Bhidayasiri, R., & Ling, H. (2008). Multiple system atrophy. The Neurologist, 14, 224–237.PubMedCrossRefGoogle Scholar
  9. Boeve, B. F. (2007). Links between frontotemporal lobar degeneration, corticobasal degeneration, progressive supranuclear palsy, and amyotrophic lateral sclerosis. Alzheimer Disease and Associated Disorders, 21, S31–38.PubMedCrossRefGoogle Scholar
  10. Bozeat, S., Gregory, C. A., Ralph, M. A., & Hodges, J. R. (2000). Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer’s disease? Journal of Neurology, Neurosurgery and Psychiatry, 69, 178–186.CrossRefGoogle Scholar
  11. Busse, A., Hensel, A., Gühne, U., Angermeyer, M. C., & Riedel-Heller, S. G. (2006). Mild cognitive impairment: Long-term course of four clinical subtypes. Neurology, 67, 2176–2185.PubMedCrossRefGoogle Scholar
  12. Carnahan, R. M., Lund, B. C., Perry, P. J., Pollock, B. G., & Culp, K. R. (2006). The anticholinergic drug scale as a measure of drug-related anticholinergic burden: Associations with serum anticholinergic activity. Journal of Clinical Pharmacology, 46, 1481–1486.PubMedCrossRefGoogle Scholar
  13. Chamberlain, S. R., & Sahakian, B. J. (2006). The neuropsychology of mood disorders. Current Psychiatry Reports, 8, 458–463.PubMedCrossRefGoogle Scholar
  14. Chen, P., Ratcliff, G., Belle, S. H., Cauley, J. A., DeKosky, S. T., & Ganguli, M. (2000). Cognitive tests that best discriminate between presymptomatic AD and those who remain nondemented. Neurology, 55, 1847–1853.PubMedCrossRefGoogle Scholar
  15. Christensen, H., Henderson, A. S., Jorm, A. F., Mackinnon, A. J., Scott, R., & Korten, A. E. (1995). ICD-10 mild cognitive disorder: epidemiological evidence on its validity. Psychological Medicine, 25, 105–120.PubMedCrossRefGoogle Scholar
  16. Crook, T., Bartus, R. T., Ferris, S. H., Whitehouse, P., Cohen, G. D., & Gershon, S. (1986). Age-associated memory impairment: Proposed diagnostic criteria and measures of clinical change. Report of a National Institute of Mental Health Work Group. Developmental Neuropsychology, 2, 261–276.CrossRefGoogle Scholar
  17. Dawes, S., Suarez, P., Casey, C. Y., et al. (2008). Variable patterns of neuropsychological performance in HIV-1 infection. Journal of Clinical and Experimental Neuropsychology, 30, 613–626.PubMedCrossRefGoogle Scholar
  18. de Leon, M. J., Convit, A., Wolf, O. T., et al. (2001). Prediction of cognitive decline in normal elderly subjects with 2-[(18)F]fluoro-2-deoxy-D-glucose/positron-emission tomography (FDG/PET). Proceedings of the National Academy of Sciences of the United States of America, 98, 10966–10971.PubMedCrossRefGoogle Scholar
  19. DeCarli, C., Mungas, D., Harvey, D., et al. (2004). Memory impairment, but not cerebrovascular disease, predicts progression of MCI to dementia. Neurology, 63, 220–227.PubMedCrossRefGoogle Scholar
  20. Devito, E. E., Pickard, J. D., Salmond, C. H., Iddon, J. L., Loveday, C., & Sahakian, B. J. (2005). The neuropsychology of normal pressure hydrocephalus (NPH). British Journal of Neurosurgery, 19, 217–224.PubMedCrossRefGoogle Scholar
  21. Diehl, J., Monsch, A. U., Aebi, C., Wagenpfeil, S., Krapp, S., Grimmer, T., et al. (2005). Frontotemporal dementia, semantic dementia, and Alzheimer’s disease: the contribution of standard neuropsychological tests to differential diagnosis. Journal of Geriatric Psychiatry and Neurology, 18, 39–44.PubMedCrossRefGoogle Scholar
  22. Dubois, B., Slachevsky, A., Pillon, B., Beato, R., Villalponda, J. M., & Litvan, I. (2005). “Applause sign” helps to discriminate PSP from FTD and PD. Neurology, 64, 2132–2133.PubMedCrossRefGoogle Scholar
  23. Duff, K., Beglinger, L. J., Schultz, S. K., et al. (2007). Practice effects in the prediction of long-term cognitive outcome in three patient samples: A novel prognostic index. Archives of Clinical Neuropsychology, 22, 15–24.PubMedCrossRefGoogle Scholar
  24. Emre, M., Aarsland, D., Brown, R., et al. (2007). Clinical diagnostic criteria for dementia associated with Parkinson’s disease. Movement Disorders, 22, 1689–1707. quiz 1837.PubMedCrossRefGoogle Scholar
  25. Esper, C. D., Weiner, W. J., & Factor, S. A. (2007). Progressive supranuclear palsy. Reviews in Neurological Diseases, 4, 209–216.PubMedGoogle Scholar
  26. Fleisher, A. S., Sowell, B. B., Taylor, C., Gamst, A. C., Petersen, R. C., & Thal, L. J. (2007). Clinical predictors of progression to Alzheimer disease in amnestic mild cognitive impairment. Neurology, 68, 1588–1595.PubMedCrossRefGoogle Scholar
  27. Forman, M. S., Farmer, J., Johnson, J. K., et al. (2006). Frontotemporal dementia: Clinicopatho­logical correlations. Annals of Neurology, 59, 952–962.PubMedCrossRefGoogle Scholar
  28. Graff-Radford, N. R. (2007). Normal pressure hydrocephalus. Neurologic Clinics, 25, 809–832. vii-viii.PubMedCrossRefGoogle Scholar
  29. Graham, J. E., Rockwood, K., Beattie, B. L., et al. (1997). Prevalence and severity of cognitive impairment with and without dementia in an elderly population. Lancet, 349, 1793–1796.PubMedCrossRefGoogle Scholar
  30. Griffith, H. R., Netson, K. L., Harrell, L. E., Zamrini, E. Y., Brockington, J. C., & Marson, D. C. (2006). Amnestic mild cognitive impairment: Diagnostic outcomes and clinical prediction over a two-year time period. Journal of the International Neuropsychological Society, 12, 166–175.PubMedCrossRefGoogle Scholar
  31. Gutierrez, R., Atkinson, J. H., & Grant, I. (1993). Mild neurocognitive disorder: needed addition to the nosology of cognitive impairment (organic mental) disorders. The Journal of Neuropsychiatry and Clinical Neurosciences, 5, 161–177.PubMedGoogle Scholar
  32. Hellstrom, P., Edsbagge, M., Archer, T., Tisell, M., Tullberg, M., & Wikkelso, C. (2007). The neuropsychology of patients with clinically diagnosed idiopathic normal pressure hydrocephalus. Neurosurgery, 61, 1219–1226. discussion 1227–1218.PubMedCrossRefGoogle Scholar
  33. Huey, E. D., Goveia, E. N., Paviol, S., Pardini, M., Krueger, F., Zamboni, G., et al. (2009). Executive dysfunction in frontotemporal dementia and corticobasal syndrome. Neurology, 72, 453–459.PubMedCrossRefGoogle Scholar
  34. Jack, C. R., Jr., Petersen, R. C., Xu, Y. C., et al. (1999). Prediction of AD with MRI-based hippocampal volume in mild cognitive impairment. Neurology, 52, 1397–1403.PubMedCrossRefGoogle Scholar
  35. Jack, C. R., Jr., Petersen, R. C., Xu, Y., et al. (2000). Rates of hippocampal atrophy correlate with change in clinical status in aging and AD. Neurology, 55, 484–489.PubMedCrossRefGoogle Scholar
  36. Jankovic, J. (2008). Parkinson’s disease: clinical features and diagnosis. Journal of Neurology, Neurosurgery and Psychiatry, 79, 368–376.CrossRefGoogle Scholar
  37. Johnson, D. K., Morris, J. C., & Galvin, J. E. (2005). Verbal and visuospatial deficits in dementia with Lewy bodies. Neurology, 65, 1232–1238.PubMedCrossRefGoogle Scholar
  38. Kawai, Y., Suenaga, M., Takeda, A., et al. (2008). Cognitive impairments in multiple system atrophy: MSA-C vs MSA-P. Neurology, 70, 1390–1396.PubMedCrossRefGoogle Scholar
  39. Kim, K. Y., Wood, B. E., & Wilson, M. I. (2005). Risk factors for Alzheimer’s disease: an overview for clinical practitioners. The Consultant Pharmacist, 20, 224–230.PubMedCrossRefGoogle Scholar
  40. Kral, V. A. (1962). Senescent forgetfulness: Benign and malignant. Canadian Medical Association Journal, 86, 257–260.PubMedGoogle Scholar
  41. Launer, L. J., Andersen, K., Dewey, M. E., et al. (1999). Rates and risk factors for dementia and Alzheimer’s disease: results from EURODEM pooled analyses. EURODEM Incidence Research Group and Work Groups. European Studies of Dementia. Neurology, 52, 78–84.PubMedCrossRefGoogle Scholar
  42. Levy, R. (1994). Aging-associated cognitive decline. International Psychogeriatrics, 6, 63–68.PubMedCrossRefGoogle Scholar
  43. Lubarsky, M., & Juncos, J. L. (2008). Progressive supranuclear palsy: a current review. The Neurologist, 14, 79–88.PubMedCrossRefGoogle Scholar
  44. Luis, C. A., Barker, W. W., Loewenstein, D. A., et al. (2004). Conversion to dementia among two groups with cognitive impairment: A preliminary report. Dementia and Geriatric Cognitive Disorders, 18, 307–313.PubMedCrossRefGoogle Scholar
  45. Lund, Manchester. (1994). Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups. Journal of Neurology, Neurosurgery and Psychiatry, 57, 416–418.CrossRefGoogle Scholar
  46. MacDonald, S.W.S, Hultsch, D.F., & Dixon, R.A. (2008). Predicting impending death: Inconsistency in speed is a selective and early marker. Psychology and Aging, 23, 595–607.Google Scholar
  47. Manly, J. J., Tang, M. X., Schupf, N., Stern, Y., Vonsattel, J. P., & Mayeux, R. (2008). Frequency and course of mild cognitive impairment in a multiethnic community. Annals of Neurology, 63, 494–506.PubMedCrossRefGoogle Scholar
  48. McKeith, I. G., Galasko, D., Kosaka, K., et al. (1996). Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop. Neurology, 47, 1113–1124.PubMedCrossRefGoogle Scholar
  49. Mendez, M. F. (1994). Huntington’s disease: update and review of neuropsychiatric aspects. International Journal of Psychiatry in Medicine, 24, 189–208.PubMedCrossRefGoogle Scholar
  50. Mendez, M. F., & Cummings, J. L. (2003). Dementia: A Clinical Approach (3rd ed.). Philadelphia: Butterworth Heinemann.Google Scholar
  51. Morris, J. C. (1997). Clinical dementia rating: a reliable and valid diagnostic and staging measure for dementia of the Alzheimer type. International Psychogeriatrics, 9(Suppl 1), 173–176. discussion 177–178.PubMedCrossRefGoogle Scholar
  52. Morris, J. C., & Cummings, J. (2005). Mild cognitive impairment (MCI) represents early-stage Alzheimer’s disease. Journal of Alzheimer’s Disease, 7, 235–239. discussion 255–262.PubMedGoogle Scholar
  53. Morris, J. C., Storandt, M., Miller, J. P., et al. (2001). Mild cognitive impairment represents early-stage Alzheimer disease. Archives of Neurology, 58, 397–405.PubMedCrossRefGoogle Scholar
  54. Murray, R., Neumann, M., Forman, M. S., et al. (2007). Cognitive and motor assessment in autopsy-proven corticobasal degeneration. Neurology, 68, 1274–1283.PubMedCrossRefGoogle Scholar
  55. Navia, B. A., Jordan, B. D., & Price, R. W. (1986). The AIDS dementia complex: I. Clinical features. Annals of Neurology, 19, 517–524.PubMedCrossRefGoogle Scholar
  56. Neary, D., Snowden, J. S., Gustafson, L., et al. (1998). Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology, 51, 1546–1554.PubMedCrossRefGoogle Scholar
  57. Paul, R., Moser, D., Cohen, R., Browndyke, J., Zawacki, T., & Gordon, N. (2001). Dementia severity and pattern of cognitive performance in vascular dementia. Applied Neuropsychology, 8, 211–217.PubMedGoogle Scholar
  58. Paulsen, J. S., & Conybeare, R. A. (2005). Cognitive changes in Huntington’s disease. Advances in Neurology, 96, 209–225.PubMedGoogle Scholar
  59. Paulsen, J. S., Ready, R. E., Hamilton, J. M., Mega, M. S., & Cummings, J. L. (2001). Neuropsychiatric aspects of Huntington’s disease. Journal of Neurology, Neurosurgery and Psychiatry, 71, 310–314.CrossRefGoogle Scholar
  60. Paulsen, J. S., Langbehn, D. R., Stout, J. C., et al. (2008). Detection of Huntington’s disease decades before diagnosis: the Predict-HD study. Journal of Neurology, Neurosurgery and Psychiatry, 79, 874–880.CrossRefGoogle Scholar
  61. Peters, N. L. (1989). Snipping the thread of life. Antimuscarinic side effects of medications in the elderly. Archives of Internal Medicine, 149, 2414–2420.PubMedCrossRefGoogle Scholar
  62. Petersen, R. C. (2003). Mild Cognitive Impairment. New York: Oxford Press.Google Scholar
  63. Petersen, R. C., Smith, G. E., Waring, S. C., Ivnik, R. J., Tangalos, E. G., & Kokmen, E. (1999). Mild cognitive impairment: clinical characterization and outcome. Archives of Neurology, 56, 303–308.PubMedCrossRefGoogle Scholar
  64. Petersen, R. C., Doody, R., Kurz, A., et al. (2001). Current concepts in mild cognitive impairment. Archives of Neurology, 58, 1985–1992.PubMedCrossRefGoogle Scholar
  65. Roman GC, Tatemichi TK, Erkinjuntti T, et al. Vascular dementia: diagnostic criteria for research studies. Report of the NINDS-AIREN International Workshop. Neurology 1993;43:250-260.Google Scholar
  66. Rosenstein, L. D. (1998). Differential diagnosis of the major progressive dementias and ­depression in middle and late adulthood: a summary of the literature of the early 1990s. Neuropsychology Review, 8, 109–167.PubMedCrossRefGoogle Scholar
  67. Salmon, D. P., & Filoteo, J. V. (2007). Neuropsychology of cortical versus subcortical dementia syndromes. Seminars in Neurology, 27, 7–21.PubMedCrossRefGoogle Scholar
  68. Salthouse, T. A. (2009). When does age-related cognitive decline begin? Neurobiology of Aging, 30, 507–514.PubMedCrossRefGoogle Scholar
  69. Starkstein, S. E., Sabe, L., Vazquez, S., et al. (1996). Neuropsychological, psychiatric, and cerebral blood flow findings in vascular dementia and Alzheimer’s disease. Stroke, 27, 408–414.PubMedCrossRefGoogle Scholar
  70. Storandt, M., Grant, E. A., Miller, J. P., & Morris, J. C. (2002). Rates of progression in mild cognitive impairment and early Alzheimer’s disease. Neurology, 59, 1034–1041.PubMedCrossRefGoogle Scholar
  71. Teng, E. L., & Chui, H. C. (1987). The modified mini-mental state (3MS) examination. The Journal of Clinical Psychiatry, 48, 314–318.PubMedGoogle Scholar
  72. Tuokko, H., Frerichs, R., Graham, J., et al. (2003). Five-year follow-up of cognitive impairment with no dementia. Archives of Neurology, 60, 577–582.PubMedCrossRefGoogle Scholar
  73. Williams-Gray, C. H., Foltynie, T., Lewis, S. J., & Barker, R. A. (2006). Cognitive deficits and psychosis in Parkinson’s disease: a review of pathophysiology and therapeutic options. CNS Drugs, 20, 477–505.PubMedCrossRefGoogle Scholar
  74. Woods, S. P., & Troster, A. I. (2003). Prodromal frontal/executive dysfunction predicts incident dementia in Parkinson’s disease. Journal of the International Neuropsychological Society, 9, 17–24.PubMedCrossRefGoogle Scholar
  75. Wright, S. L., & Persad, C. (2007). Distinguishing between depression and dementia in older persons: neuropsychological and neuropathological correlates. Journal of Geriatric Psychiatry and Neurology, 20, 189–198.PubMedCrossRefGoogle Scholar
  76. Yaari, R. (2007). Corey-Bloom J. Alzheimer’s disease. Seminars in Neurology, 27, 32–41.PubMedCrossRefGoogle Scholar
  77. Zaccai, J., McCracken, C., & Brayne, C. (2005). A systematic review of prevalence and incidence studies of dementia with Lewy bodies. Age and Ageing, 34, 561–566.PubMedCrossRefGoogle Scholar
  78. Zekry, D., Hauw, J. J., & Gold, G. (2002). Mixed dementia: epidemiology, diagnosis, and treatment. Journal of the American Geriatrics Society, 50, 1431–1438.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Departments of Psychiatry and Neurosciences, Neurology, and Neurological SurgeryUniversity of South Florida College of MedicineTampaUSA
  2. 2.Case Western Reserve University School of MedicineClevelandUSA

Personalised recommendations