Skip to main content
Article thumbnail
Location of Repository

Dementia with Lewy bodies: Definition, diagnosis, and pathogenic relationship to Alzheimer’s disease

By Robert E Mrak and W Sue T Griffin


Clinical dementia associated with the appearance of Lewy bodies in the cerebral cortex has been recognized for over 40 years. Until the 1990s, however, cortical Lewy body disease was thought to be a rare cause of dementia. At that time, the advent of sensitive and specific immunohistochemical techniques for highlighting these elusive structures led to the recognition of cortical Lewy body disease as a common substrate for clinical dementia. Current diagnostic criteria recognize dementia with Lewy bodies as a clinicopathological entity. Also recognized is the closely related (and perhaps biologically identical) entity of Parkinson’s disease dementia, which differs from dementia with Lewy bodies only in the temporal sequence of appearance of clinical symptoms. The generic term “Lewy body disease” encompasses both entities. There is frequent and extensive overlap, both clinically and pathologically, between dementia with Lewy bodies and Alzheimer’s disease. The two diseases share several genetic and environmental risk factors that have in common increased inflammatory states associated with increased disease risk. Moreover, pathological and experimental work has implicated the involvement of activated microglia and of microglia-derived interleukin-1 in the pathogenesis of the pathognomonic lesions of both diseases. Such neuroinflammatory processes may be the common link driving progression in both diseases and explaining the frequent overlap between the two diseases

Topics: Reviews
Publisher: Dove Medical Press
OAI identifier:
Provided by: PubMed Central
Download PDF:
Sorry, we are unable to provide the full text but you may find it at the following location(s):
  • http://www.pubmedcentral.nih.g... (external link)
  • Suggested articles


    1. (2002). A meta-analysis of coffee drinking, cigarette smoking, and the risk of Parkinson’s disease.
    2. (2001). A polymorphism in the intronic region of the IL-1alpha gene and the risk for Parkinson’s disease.
    3. (2005). A systematic review of prevalence studies of dementia in Parkinson’s disease. Movement Disord,
    4. (2003). Age-dependent association between interleukin-1A (-889) genetic polymorphism and sporadic Alzheimer’s disease. A meta-analysis.
    5. (1998). Alzheimer’s disease and Parkinson’s disease: distinct entities or extremes of a spectrum of neurodegeneration? Ann Neurol,
    6. (1989). Anti-ubiquitin immunocytochemistry is more sensitive than conventional techniques in the detection of diffuse Lewy body disease.
    7. (2004). Association between the interleukin-1alpha gene and Alzheimer’s disease: a meta-analysis.
    8. (2002). Association of an interleukin 1B gene polymorphism (-511) with Parkinson’s disease in Finnish patients.
    9. (2002). Association of interleukin-1 beta polymorphisms with idiopathic Parkinson’s disease.
    10. (1980). Atypical senile dementia with widespread Lewy type inclusion in the cerebral cortex.
    11. (2005). Cognitive status correlates with neuropathologic stage in Parkinson disease.
    12. (1996). Consensus guidelines for the clinical and pathologic diagnosis of dementia with
    13. (1999). CYP2D6 is associated with Parkinson’s disease but not with dementia with Lewy bodies or Alzheimer’s disease.
    14. (1979). Dementia in Parkinson’s disease: A neuropathological study.
    15. (2003). Dementia with Lewy bodies: disease concept and genetics.
    16. (1961). Diffuse intracytoplasmic ganglionic inclusions (Lewy type) associated with progressive dementia and quadriparesis in fl exion.
    17. (1988). Diffuse Lewy body disease and progressive dementia.
    18. (1984). Extrapyramidal signs in Alzheimer’s disease.
    19. (2000). Fine mapping of the chromosome 12 late-onset Alzheimer disease locus: potential genetic and phenotypic heterogeneity.
    20. (2005). Genes involved in Alzheimer’s disease, a survey of possible candidates.
    21. (1988). Genetics, 68:381–404.Neuropsychiatric Disease and Treatment 2007:3(5) 625 Dementia with Lewy bodies Kuzuhara
    22. (1997). Glial-neuronal interactions in Alzheimer’s disease: Progressive association of IL-1alpha + microglia and S100beta + astrocytes with neurofi brillary tangle stages.
    23. (2003). Head trauma preceding PD: a case-control study.
    24. (1985). Heterogeneity in dementia of the Alzheimer type: evidence of subgroups.
    25. (1978). Idiopathic Parkinsonism with Lewy-type inclusions in cerebral cortex. A case report.
    26. (2000). Infl uence of interleukin-1beta gene polymorphisms on age-at-onset of sporadic Parkinson’s disease.
    27. (1995). Interleukin-1 expression in different plaque types in Alzheimer’s disease, signifi cance in plaque evolution.
    28. (2003). Interleukin-1 mediates pathological effects of microglia on tau phosphorylation and on synaptophysin synthesis in cortical neurons through a p38-MAPK pathway.
    29. (2000). Interleukin-1 promotes expression and phosphorylation of neurofi lament and tau proteins in vivo. Exp Neurol,
    30. (2001). Interleukin-1 promotion of MAPK-p38 overexpression in experimental animals and in Alzheimer’s disease: potential signifi cance for tau protein phosphorylation. Neurochem Int,
    31. (2004). Lack of association between the interleukin-1 alpha (-889) polymorphism and early-onset Parkinson’s disease.
    32. (1995). Microglial interleukin-1alpha expression in brain regions in Alzheimer’s disease: Correlation with neuritic plaque distribution.
    33. (2004). Molecular genetics of late-onset Alzheimer’s disease.
    34. (1997). NACP, a presynaptic protein, immunoreactivity in Lewy bodies in Parkinson’s disease.
    35. (1991). Neuropathological stageing of Alzheimer-related changes.
    36. (2001). No evidence of an association between CYP2D6 polymorphisms among Japanese and dementia with Lewy bodies. Psychiatry Clin Neurosci,
    37. (2003). Nonsteroidal antiinfl ammatory drugs and the risk of Parkinson disease.
    38. (1985). Parkinson’s disease and dementia.
    39. (1980). Parkinson’s disease, dementia, and Alzheimer’s disease: clinicopathological correlations. Annals Neurol,
    40. (2005). Physical activity and the risk of Parkinson disease.
    41. (1993). Plaque-only Alzheimer’s disease is usually the Lewy body variant, and vice versa.
    42. (2002). Polymorphisms in the interleukin-1 alpha and beta genes and the risk for Parkinson’s disease.
    43. (1978). Presenile dementia with Lewy bodies and neurofi brillary tangles.
    44. (1980). Progressive dementia with “diffuse Lewy-type inclusions” in cerebral cortex. A case report.
    45. (2002). Relative frequencies of Alzheimer disease, Lewy body, vascular and frontotemporal dementia, and hippocampal sclerosis in the state of Florida brain bank. Alzheimer Dis Assoc Disord,
    46. (2004). Risk and incidence of dementia in a cohort of older subjects with Parkinson’s disease in the United Kingdom. Movement Disord,
    47. (2003). Staging of brain pathology related to sporadic Parkinson’s disease. Neurobiol Aging,
    48. (1994). The apolipoprotein E allele epsilon 4 is overrepresented in patients with the Lewy body variant of Alzheimer’s disease.
    49. (1991). The Consortium to Establish a Registry for Alzheimer’s Disease (CERAD). Part II. Standardization of the neuropathologic assessment of Alzheimer’s disease.
    50. (1995). The CYP2D6B mutant allele is overrepresented in the Lewy body variant of Alzheimer’s disease.
    51. (1997). The National Institute on Aging, and Reagan Institute Working Group on Diagnostic Criteria for the Neuropathological Assessment of Alzheimer’s Disease
    52. (1966). The prevalence, natural history, and dementia of Parkinson’s disease.

    To submit an update or takedown request for this paper, please submit an Update/Correction/Removal Request.