Skip to main content
SpringerLink
Log in

Dementia with Lewy Bodies

  • Chapter
Book cover Neuropathology and Genetics of Dementia

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 487))

Abstract

An increasing variety of neurological syndromes and brain disorders have been associated with Lewy body pathology in various parts of the nervous system’. The major dementia syndromes in which Lewy bodies are frequently identified are Dementia with Lewy bodies (DLB), dementia arising in Parkinson’s disease, and Alzheimer’s disease. Within each of these clinico-pathological umbrellas many patients can be allocated to the core syndromes based on a combination of clinical features and the pathological changes demonstrable at autopsy. However, there is a substantial, and currently unquantified, overlap between them such that some authors have proposed a continuum of Lewy body and Alzheimer-type pathologies2. The paradigms of Parkinson’s disease and Alzheimer’s disease have been absorbed into clinical practice, pathological interpretation at autopsy, and models of pathogenesis, to such an extent that DLB has been difficult to assimilate. DLB is currently defined in terms of a clinical syndrome and prospective pathological studies suggest that this syndrome predicts the presence of Lewy body pathology at least as well as a diagnosis of AD can predict the pathology of that disorder3. Similar considerations apply to Parkinson’s disease so that for each of the three syndromes the currently accepted clinical criteria for their diagnosis is likely to be verified at autopsy in at least 4 out of 5 cases. The clinical features of DLB are defined in the published consensus criteria from an International Workshop held in 19954. Many studies published subsequently have turned the concept of DLB

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. .Ince PG, Perry EK, Morris CM (1998) Dementia with Lewy bodies. A distinct non-Alzheimer dementia syndrome? Brain Pathol 8: 299–324.

    Article  PubMed  CAS  Google Scholar 

  2. Perl DP, Olanow CW, Caine D (1998) Alzheimer’s disease and Parkinson’s disease: distinct entities or extremes of a spectrum of neurodegeneration. Ann Neuro 144 (Suppl 1): S19–S31.

    Google Scholar 

  3. McKeith I, Ballard C, Perry R et al. (2000) Prospective validation of Consensus criteria for the diagnosis of Dementia with Lewy bodies. Neurology 54: 1050–1058.

    Article  PubMed  CAS  Google Scholar 

  4. McKeith IG, Galasko D, Kosaka K et al. (1996) Consensus guidelines for the clinical and pathological diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB International Workshop. Neurology 47: 1113–1124.

    Article  PubMed  CAS  Google Scholar 

  5. Lantos PL, Ovenstone IM, Johnson J et al. (1994) Lewy bodies in the brains of two members of a family with the 717 (Val to Ile) mutation of the amyloid precursor protein gene. Neurosci Lett 172: 77–79.

    Article  PubMed  CAS  Google Scholar 

  6. Hamilton RL (2000) Lewy bodies in Alzheimer’s disease: a neuropathological review of 145 cases using a-synuclein immunohistochemistry. Brain Pathol 10: 378–384.

    Article  PubMed  CAS  Google Scholar 

  7. Kosaka K (1978) Lewy bodies in the cerebral cortex. Report of three cases. Acta Neuropathol 42: 127–134.

    Article  PubMed  CAS  Google Scholar 

  8. Kosaka K, Mehraein P (1979) Dementia-parkinsonism with numerous Lewy bodies and senile plaques in cerebral cortex. Arch Psychiatr Nervenkr 226: 241–250.

    Article  PubMed  CAS  Google Scholar 

  9. Kosaka K, Matsushita M, Oyanagi S et al. (1980) A clinicopathological study of the “Lewy Body Disease”. Pschiat Neurol Japon 82: 292–311.

    CAS  Google Scholar 

  10. Kosaka K, Yoshimura M, Ikeda K et al. (1984) Diffuse type of Lewy body disease: progressive dementia with abundant cortical Lewt bodies and senile changes of varying degree - A new disease? Clin Neuropathol 3: 185–192.

    PubMed  CAS  Google Scholar 

  11. Cummings J (1997) Changes in neuropsychiatric symptoms as outcome measures in. clinical trials with cholinergic therapies for Alzheimer’s disease. Alz Dis Assoc Disord 11: 51–59.

    Article  Google Scholar 

  12. Hutchinson M, Fazzini E (1996) Cholinesterase inhibition in Parkinson’s disease. J Neurol Neurosurg Psychiatry 61: 324–325.

    Article  PubMed  CAS  Google Scholar 

  13. McKeith IG, Fairbairn A, Perry RH et al. (1992) Neuroleptic sensitivity in patients with senile dementia of Lewy body type. BMJI 305: 673–678.

    Article  CAS  Google Scholar 

  14. Olichney JM, Galasko D, Salmon DP et al. (1998) Cognitive decline is faster in Lewy body variant than in Alzheimer’s disease. Neurology 51:351–357.

    Article  PubMed  CAS  Google Scholar 

  15. Perry RH, Irving D, Blessed G et al. (1990) Senile dementia of Lewy body type: a clinically and neuropatholgically distinct form of Lewy body dementia in the elderly. J Neurol Sci 95: 119–139.

    Article  PubMed  CAS  Google Scholar 

  16. Lennox G, Lowe J, Morrell K et al. (1989) Anti-ubiquitin immunocytochemistry is more sensitive than conventional techniques in the detection of diffuse Lewy body disease. J Neurol Neurosurg Psychiatry 52: 67–71.

    Article  PubMed  CAS  Google Scholar 

  17. Lowe J, Blanchard A, Morrell K et al. (1988) Ubiquitin is a common factor in intermediate filament inclusion bodies of diverse type in man, including those of Parkinson’s disease, Pick’s disease and Alzheimer’s disease as well as Rosenthal fibres in cerebellar astrocytomas, cytoplasmic bodies in muscle and Mallories bodies in alcoholic liver disease. J Pathol 155: 9–15.

    Article  PubMed  CAS  Google Scholar 

  18. Polymeropoulos MH, Lavedan C, Leroy E et al. (1997) Mutation in the a-synuclein gene identified in families with Parkinson’s disease. Science 276: 2045–2047.

    Article  PubMed  CAS  Google Scholar 

  19. Spillantini MG, Schmidt ML, Lee VM-Y et al. (1997) a-Synuclein in Lewy bodies. Nature 388: 839–840.

    Article  PubMed  CAS  Google Scholar 

  20. Spillantini MG, Crowther RA, Jakes R et al. (1998) a-synuclein in filamentous inclusions of Lewy bodies from Parkinson’s disease and Dementia with Lewy bodies. Proc Natl Acad Sci USA 95: 6469–6473.

    Article  PubMed  CAS  Google Scholar 

  21. Mirra SS, Heyman A, McKeel D et al. (1991) The consortium to establish a registry of Alzheimer’s disease (CERAD) Part II. Standardization of the neuropathologic assessment of Alzheimer’s disease. Neurology 41: 479–486.

    Article  PubMed  CAS  Google Scholar 

  22. Ince PG, McArthur FK, Bjertness E et al. (1995) Neuropathological diagnoses in elderly patients in Oslo: Alzheimer’s disease, Lewy body disease and vascular lesions. Dementia 6: 162–168.

    PubMed  CAS  Google Scholar 

  23. Braak H, Braak E, Yilmazer D et al. (1994) Amygdala pathology in Parkinson’s disease. Acta Neuropathol 88: 493–500.

    Article  PubMed  CAS  Google Scholar 

  24. Del Ser T, Hachinski V, Merskey H et al. (2000) Clinical and pathological features of two groups of dementia with Lewy bodies: effect of co-existing Alzheimer-type lesion load. Alz Dis Assoc Disorders: in press.

    Google Scholar 

  25. Dickson DW, Davies P, Mayeux R et al. (1987) Diffuse Lewy body disease: Neuropathological and biochemical studies of six patients. Acta Neuropathol 75: 8–15.

    Article  PubMed  CAS  Google Scholar 

  26. Galasko D, Katzman R, Salmon DP et al. (1996) Clinical features and neuropathological findings in Lewy body dementias. Brain and Cognition 31: 166–175.

    Article  PubMed  CAS  Google Scholar 

  27. Gentleman SM, Williams B, Royston MC et al. (1992) Quantification of ßA4 protein deposition in the medial temporal lobe: A comparison of Alzheimer’s disease and senile dementia of Lewy body type. Neurosci Lett 142: 9–12.

    Article  PubMed  CAS  Google Scholar 

  28. Hansen LA, Masliah E, Terry RD et al. (1989). A neuropathological subset of Alzheimer’s disease with concomitant Lewy body disease and spongiform change. Acta Neuropathol 78: 194–201.

    Article  PubMed  CAS  Google Scholar 

  29. Hansen L, Salmon D, Galasko D et al. (1990) The Lewy body variant of Alzheimer’s disease: a clinical and pathological entity. Neurology 40: 1–8.

    Article  PubMed  CAS  Google Scholar 

  30. Hansen LA, Masliah E, Galasko D et al. (1993) Plaque-only Alzheimer’s disease is usually the Lewy body variant and vice versa. J Neuropathol Expl Neurol 52: 648–654.

    Article  CAS  Google Scholar 

  31. Hansen LA, Samuel W (1997) Criteria for Alzheimer’s disease and the nosology of dementia with Lewy bodies. Neurology 48: 126–132.

    Article  PubMed  CAS  Google Scholar 

  32. Hansen LA (1997) The Lewy body variant of Alzheimer disease. J Neural Transm 51[Suppl]: 83–93.

    Article  CAS  Google Scholar 

  33. Ince P, Irving D, MacArthur F et al. (1991) Quantitative neuropathology in the hippocampus: comparison of senile dementia of Alzheimer type, senile dementia of Lewy body type, Parkinson’s disease and non-demented elderly control patients. J Neurol Sci 106: 142–152.

    Article  PubMed  CAS  Google Scholar 

  34. Jellinger KA, Bancher C (1996) Dementia with Lewy bodies: relationships to Parkinson’s and Alzheimer’s diseases. In: Dementia with Lewy Bodies (Perry RH, McKeith IG, Perry EK, eds), pp 268–286: Cambridge University Press, Cambridge.

    Chapter  Google Scholar 

  35. Kosaka K (1993) Dementia and neuropathology in Lewy body disease. Adv Neurol 60: 456–463.

    PubMed  CAS  Google Scholar 

  36. Samuel W, Crowder R, Hofstetter CR et al. (1997) Neuritic plaques in the Lewy body variant of Alzheimer’s disease lack paired helical filaments. Neurosci Lett 223: 73–76.

    Article  PubMed  CAS  Google Scholar 

  37. Samuel W, Alford M, Hofstetter CR et al. (1997) Dementia with Lewy bodies versus pure Alzheimer’s disease: differences in cognition, neuropathology, cholinergic dysfunction, and synaptic density. J Neuropathol Exp Neurol 56: 499–508.

    Article  PubMed  CAS  Google Scholar 

  38. Sima AAF, Clark AW, Stemberger NA et al. (1986) Lewy body dementia without Alzheimer changes. Can J Neurol Scis 13(Suppl)): 490–497.

    CAS  Google Scholar 

  39. Harrington CR, Perry RH, Perry EK et al. (1994) Senile dementia of Lewy body type and Alzheimer type are biochemically distinct in terms of paired helical filaments and hyperphosphorylated tau protein. Dementia 5: 216–228.

    Google Scholar 

  40. Strong C, Anderton BH, Perry RH et al. (1995) Abnormally phosphorylated tau protein in senile dementia of Lewy body type and Alzheimer’s disease: evidence that the disorders are distinct. Alz Dis Assoc Disorders 9: 218–222.

    CAS  Google Scholar 

  41. Dickson DW, Crystal H, Mattiace LA et al. (1989) Diffuse Lewy body disease: light and electron microscopic immunocytochemistry of senile plaques. Acta Neuropathol 78: 572–584.

    Article  PubMed  CAS  Google Scholar 

  42. Braak H, Braak E, Yilmazer D et al. (1996) Pattern of brain destruction in Parkinson’s disease and Alzheimer’s disease. J Neural Transm 103: 455–490.

    Article  PubMed  CAS  Google Scholar 

  43. Dickson DW, Schmidt ML, Lee VM et al. (1994) Immunoreactivity profile of hippocampal CA2/3 neuntes in diffuse Lewy body disease. Acta Neuropathol 87: 269–276.

    Article  PubMed  CAS  Google Scholar 

  44. Forstl H, Burns A, Luthert P et al. (1993) The Lewy body variant of Alzheimer’s disease: clinical and pathological findings. Br J Psychiat 162: 385–392.

    Article  CAS  Google Scholar 

  45. Galasko D, Hansen LA, Katzman R et al. (1994) Clinical-neuropathological correlation’s in Alzheimer’s disease and related disorders. Arch Neurol 51:888–895.

    Article  PubMed  CAS  Google Scholar 

  46. Heyman A, Fillenbaum GG, Gearing M et al. (1999) Comparison of Lewy body variant of Alzheimer’s disease with pure Alzheimer’s disease: Consortium to Establish a Registry for Alzheimer’s disease, Part XIX. Neurology 52: 1839–1844.

    Article  PubMed  CAS  Google Scholar 

  47. Kazee AM, Han LY (1995) Cortical Lewy bodies in Alzheimer’s disease. Arch Path Lab Med 119: 448–453.

    PubMed  CAS  Google Scholar 

  48. Victoroff J, Mack WJ, Leyness SA et al. (1995) Multicentre clinicopathological correlation in dementia. Am J Psychiat 152: 1476–1484.

    PubMed  CAS  Google Scholar 

  49. Khachaturian ZS (1985) The diagnosis of Alzheimer’s disease. Arch Neurol 42:10971105.

    Google Scholar 

  50. The National Institute on Aging and Reagan Institute Working Group on Diagnositic Criteria for the Neuropathological Assessment of Alzheimer’s disease (1997) Consensus recommendations for the postmortem diagnosis of Alzheimer’s disease. Neurobiol Aging 18 (Suppl 4): S1–27.

    Google Scholar 

  51. Lippa CF, Fujiwara H, Mann DM et al. (1998) Lewy bodies contain altered alphasynuclein in brains of many familial Alzheimer’s disease patients with mutations in presenilin and amyloid precursor protein genes. Am J Path 153: 1365–1370.

    Article  PubMed  CAS  Google Scholar 

  52. Lippa CF, Scmidt ML, Lee VM et al. (1994) Antibodies to alpha-synuclein detect Lewy bodies in many Down’s syndrome brains with Alzheimer’s disease. Ann Neurol 45: 353–357.

    Article  Google Scholar 

  53. McKhann G, Drachman D, Folstein M et al. (1984) Clinical diagnosis of Alzheimer’s Disease: report of the NINCDS-ADRDA Workgroup under the auspices of the Department of Health and Human Services Task Force on Alzheimer’s Disease. Neurology 34: 939–944.

    Article  PubMed  CAS  Google Scholar 

  54. Tomlinson BE, Blessed G, Roth M (1968) Observations on the brains of non-demented old people. J Neurol Sci 7: 331–356.

    Article  PubMed  CAS  Google Scholar 

  55. Kosaka K, Iseki E (1996) Diffuse Lewy body disease within the spectrum of Lewy body disease. In: Dementia with Lewy Bodies (Perry RH, McKeith IG, Perry EK, eds), pp 238–247: Cambridge University Press, Cambridge.

    Chapter  Google Scholar 

  56. Ince P, Perry R, Perry E (2000) Pathology of dementia with Lewy bodies. In: Dementia (O’Brien J, Burns A, eds), pp 699–717, 2nded: Arnold, London.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Neuropathology, Division of Genomic Medicine, Sheffield University Medical School, Sheffield, UK

    Paul Ince

Authors
  1. Paul Ince
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. University of Basel, Basel, Switzerland

    Markus Tolnay  & Alphonse Probst  & 

Rights and permissions

Reprints and permissions

Copyright information

© 2001 Springer Science+Business Media New York

About this chapter

Cite this chapter

Ince, P. (2001). Dementia with Lewy Bodies. In: Tolnay, M., Probst, A. (eds) Neuropathology and Genetics of Dementia. Advances in Experimental Medicine and Biology, vol 487. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-1249-3_12

Download citation

  • DOI: https://doi.org/10.1007/978-1-4615-1249-3_12

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-5461-1

  • Online ISBN: 978-1-4615-1249-3

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation