Clinical Diagnostic Criteria for Dementia with Lewy Bodies



Clinical diagnostic concepts and methods for the dementias associated with Lewy body disease have evolved over the last three decades to incorporate previously used terminologies including diffuse LB disease (DLBD) [Kosaka et al., Clin Neuropathol 3(5):185–192, 1984; Dickson et al., Acta Neuropathol 75:8–15, 1987; Lennox et al., Lancet 8633(1):323–324, 1989], LB dementia (LBD) [Gibb et al., Brain 110:1131–1153, 1987], dementia associated with cortical Lewy bodies (DCLB) [Byrne et al., Dementia 2:283–284, 1991], the LB variant of Alzheimer’s disease (LBVAD) [Hansen et al., Neurology 40:1–8, 1990; Förstl et al., Br J Psychiatry 162:385–392, 1993] and senile dementia of LB type (SDLT) [Perry et al. J Neurol Sci 95:119–139, 1990]. Dementia with Lewy bodies (DLB) was eventually agreed as a term to include all of these within one set of operationalised consensus criteria [McKeith et al., Neurology 47(5):1113–1124, 1996; McKeith et al. Neurology 65(12):1863–1872, 2005]. These in turn formed the basis for the inclusion of neurocognitive disorder with Lewy bodies (NCDLB) in the latest revision of the DSM5 [APA. Diagnostic and statistical manual of mental disorders: DSM-5. American Psychiatric Association, Washington, DC, 2013]. This formal recognition of DLB as a diagnostic category equivalent to others such as Alzheimer’s disease, vascular disease and frontotemporal disorder will greatly assist recognition and diagnosis of the disorder with benefit for patients and families. It will also enable clinical and research practice and impact upon reimbursement and regulatory authorities. Criteria for early and prodromal diagnosis remain in development.


Clinical Diagnosis Lewy Dementia 


  1. 1.
    Kosaka K, Yoshimura M, Ikeda K, et al. Diffuse type of Lewy body disease: progressive dementia with abundant cortical Lewy bodies and senile changes of varying degree – a new disease? Clin Neuropathol. 1984;3(5):185–92.PubMedGoogle Scholar
  2. 2.
    Dickson DW, Davies P, Mayeux R, et al. Diffuse Lewy body disease. Neuropathological and biochemical studies of six patients. Acta Neuropathol. 1987;75:8–15.CrossRefPubMedGoogle Scholar
  3. 3.
    Lennox G, Lowe J, Byrne EJ, et al. Diffuse Lewy body disease. Lancet. 1989;8633(1):323–4.CrossRefGoogle Scholar
  4. 4.
    Gibb WRG, Esiri MM, Lees AJ. Clinical and pathological features of diffuse cortical Lewy body disease (Lewy body dementia). Brain. 1987;110:1131–53.CrossRefPubMedGoogle Scholar
  5. 5.
    Byrne EJ, Lennox G, Godwin-Austen RB, et al. Dementia associated with cortical Lewy bodies. Proposed diagnostic criteria. Dementia. 1991;2:283–4.Google Scholar
  6. 6.
    Hansen L, Salmon D, Galasko D, et al. The Lewy body variant of Alzheimer's disease: a clinical and pathologic entity. Neurology. 1990;40:1–8.CrossRefPubMedGoogle Scholar
  7. 7.
    Förstl H, Burns A, Luthert P, et al. The Lewy-body variant of Alzheimer’s disease. Clinical and pathological findings. Br J Psychiatry. 1993;162:385–92.CrossRefPubMedGoogle Scholar
  8. 8.
    Perry RH, Irving D, Blessed G, et al. Senile dementia of Lewy body type. A clinically and neuropathologically distinct form of Lewy body dementia in the elderly. J Neurol Sci. 1990;95:119–39.CrossRefPubMedGoogle Scholar
  9. 9.
    McKeith IG, Galasko D, Kosaka K, et al. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop. Neurology. 1996;47(5):1113–24.CrossRefPubMedGoogle Scholar
  10. 10.
    McKeith I, Dickson D, Emre M, et al. Dementia with Lewy bodies: diagnosis and management: third report of the DLB consortium. Neurology. 2005;65(12):1863–72.CrossRefPubMedGoogle Scholar
  11. 11.
    APA. Diagnostic and statistical manual of mental disorders: DSM-5. Washington, DC: American Psychiatric Association; 2013.Google Scholar
  12. 12.
    Okazaki H, Lipkin LE, Aronson SM. Diffuse intracytoplasmic ganglionic inclusions (Lewy type) associated with progressive dementia and quadriparesis in flexion. J Neuropathol Exp Neurol. 1961;20:237–44.CrossRefPubMedGoogle Scholar
  13. 13.
    Burkhardt CR, Filley CM, Kleinschmidt-DeMasters BK, et al. Diffuse Lewy body disease and progressive dementia. Neurology. 1988;38:1520–8.CrossRefPubMedGoogle Scholar
  14. 14.
    Crystal HA, Dickson DW, Lizardi JE, et al. Antemortem diagnosis of diffuse Lewy body disease. Neurology. 1990;40:1523–8.CrossRefPubMedGoogle Scholar
  15. 15.
    Hansen LA, Galasko D. Lewy body disease. Curr Opin Neurol Neurosurg. 1992;5:889–94.PubMedGoogle Scholar
  16. 16.
    Byrne EJ, Lennox G, Lowe J, et al. Diffuse Lewy body disease: clinical features in 15 cases. J Neurol Neurosurg Psychiatry. 1989;52:709–17.CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    McKeith IG, Perry RH, Fairbairn AF, et al. Operational criteria for senile dementia of Lewy body type (SDLT). Psychol Med. 1992;22:911–22.CrossRefPubMedGoogle Scholar
  18. 18.
    McKeith I, Fairbairn A, Perry R, et al. Neuroleptic sensitivity in patients with senile dementia of Lewy body type. Br Med J. 1992;305:673–8.CrossRefGoogle Scholar
  19. 19.
    McKeith IG. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop. J Alzheimers Dis. 2006;9:417–23.PubMedGoogle Scholar
  20. 20.
    Emre M, Aarsland D, Brown R, et al. Clinical diagnostic criteria for dementia associated with Parkinson disease. Mov Disord. 2007;e-pub ahead of print.Google Scholar
  21. 21.
    Dubois B, Burn D, Goetz C, et al. Diagnostic procedures for Parkinson’s disease dementia: recommendations from the movement disorder society task force. Mov Disord. 2007;22(16):2314–24.CrossRefPubMedGoogle Scholar
  22. 22.
    Lippa C, Duda JE, Grossman M, et al. DLB and PDD boundary issues: diagnosis, treatment, molecular pathology, and biomarkers. Neurology. 2007;68:812–9.CrossRefPubMedGoogle Scholar
  23. 23.
    Salmon D, Galasko D. Neuropsychological aspects of Lewy body dementia. In: Perry R, McKeith I, Perry E, editors. Dementia with Lewy bodies. New York: Cambridge University Press; 1996. p. 99–113.CrossRefGoogle Scholar
  24. 24.
    Salmon DP, Galasko D, Hamilton JM, et al. Cognitive profiles differ across disease course in autopsy-proven dementia with Lewy bodies and Alzheimer’s disease. Neurobiol Aging. 2002;23:S130.Google Scholar
  25. 25.
    McKeith IG, Ballard CG, Perry RH, et al. Prospective validation of consensus criteria for the diagnosis of dementia with Lewy bodies. Neurology. 2000;54:1050–8.CrossRefPubMedGoogle Scholar
  26. 26.
    Litvan I, Bhatia KP, Burn DJ, et al. SIC Task Force appraisal of clinical diagnostic criteria for Parkinsonian disorders. Mov Disord. 2003;18(5):467–86.CrossRefPubMedGoogle Scholar
  27. 27.
    McKeith IG, Perry EK, Perry RH. Report of the second dementia with Lewy body international workshop. Neurology. 1999;53(5):902–5.CrossRefPubMedGoogle Scholar
  28. 28.
    Boeve B, Silber M, Ferman T, et al. Association of REM sleep behavior disorder and neurodegenerative disease may reflect an underlying synucleinopathy. Mov Disord. 2001;16:622–30.CrossRefPubMedGoogle Scholar
  29. 29.
    Walker Z, Costa DC, Walker RWH, et al. In vivo demonstration of dopaminergic degeneration in dementia with Lewy bodies using 123I-FP-CIT and SPET. Joint meeting of international Psychogeriatric Association and Royal College of Psychiatrists’, Faculty of Old Age on Non-Alzheimer Cognitive Impairment, 4–7 April 2000; 2000:23.Google Scholar
  30. 30.
    Aarsland D, Rongve A, Nore SP, et al. Frequency and case identification of Dementia with Lewy bodies using the revised consensus criteria. Dement Geriatr Cogn Disord. 2008;26(5):445–52.CrossRefPubMedGoogle Scholar
  31. 31.
    Weisman D, Cho M, Taylor C, et al. In dementia with Lewy bodies, Braak stage determines phenotype, not Lewy body distribution. Neurology. [Article]. 2007;69(4):356–9.Google Scholar
  32. 32.
    Tiraboschi P, Attems J, Thomas A, et al. Clinicians’ ability to diagnose dementia with Lewy bodies is not affected by beta-amyloid load. Neurology. 2015;84(5):496–9.CrossRefPubMedPubMedCentralGoogle Scholar
  33. 33.
    Kosaka K. Lewy body disease and dementia with Lewy bodies. Proc Jpn Acad Series B Phys Biol Sci. 2014;90(8):301–6.CrossRefGoogle Scholar
  34. 34.
    Donaghy PC, McKeith IG. The clinical characteristics of dementia with Lewy bodies and a consideration of prodromal diagnosis. Alzheimer's Res Ther 2014; (6)4. doi: 10.1186/alzrt274.

Copyright information

© Springer Japan KK 2017

Authors and Affiliations

  1. 1.Newcastle University Institute for AgeingNewcastle upon TyneUK

Personalised recommendations