Gå direkt till huvudinnehållet

Lewy body-demens

Senast uppdaterad: Senast reviderad:
Sakkunnig:

Definition:
Lewy body-demens är en synukleinopati, vilket innebär ett neurodegenerativt tillstånd med onormal ansamling av alfa-synuklein. Hög ålder, manligt kön och genetiska riskfaktorer predisponerar för sjukdomen.
Förekomst:
Lewy body-demens är det näst vanligaste neurodegenerativa tillståndet, efter Alzheimers sjukdom.
Symtom:
Kärnsymtomen är framträdande fluktuationer i uppmärksamhet och vakenhet, upprepade synhallucinationer, störd drömsömn (REM sömnstörning) och spontan parkinsonism.
Kliniska fynd:
Patienter med Lewy body-demens är ofta väl orienterade men långsamma i tanken och har svårt att bedöma avstånd och uppfatta saker tredimensionellt. Eventuella minnesproblem uppkommer ofta sent i förloppet.
Diagnostik:
Neuropsykologiska undersökningar kan eventuellt vara av värde i tidig fas vid Lewy body-demens för att fånga visuospatiala och exekutiva svårigheter, och påvisa förhållandevis bättre minnesfunktion. DT eller MR visar ofta bevarade temporallober, om inte en blandbild med Alzheimer föreligger. DAT scan eller Dopa PET visar ofta nedsatt dopamintransport, som vid Parkinsons sjukdom.
Behandling:
Huvudsaklig behandling är organiserande av stödåtgärder. Kolinesterashämmare är förstahandsval i medicineringen.
  1. Neef D, Walling AD. Dementia with Lewy bodies: An emerging disease. Am Fam Physician 2006; 73: 1223-9. PubMed  
  2. McKeith I, Mintzer J, Aarsland D, Burn D, Chiu H, Cohen-Mansfield J, et al. Dementia with Lewy bodies. Lancet Neurol 2004; 3: 19-28. PubMed  
  3. Sanford AM. Lewy Body Dementia. Clin Geriatr Med. 2018;34(4):603-615. PMID: 30336990 PubMed  
  4. Walker Z, Possin KL, Boeve BF, et al. Lewy body dementias. Lancet. 2015 Oct 24;386(10004):; 386, Issue 10004: 1683-97. PMID: 26595642 PubMed  
  5. Kosaka K, Yoshimura M, Ikeda K, Budka H. 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: 185-92. PubMed  
  6. McKeith IG, Dickson DW, Lowe J, et al. Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium published correction appears in Neurology. 2005 Dec 27;65(12):1992. Neurology. 2005;65(12):1863-1872. PMID: 16237129 PubMed  
  7. 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(3 Suppl):417-423. PMID: 16914880 PubMed  
  8. SveDem årsrapport 2018. www.ucr.uu.se  
  9. Nationella riktlinjer för vård och omsorg vid demenssjukdom – Stöd för styrning och ledning (2017). Socialstyrelsen. roi.socialstyrelsen.se  
  10. Trojanowski JQ, Lee VMY. Aggregation of neurofilaments and alfa-synuclein proteins in Lewy bodies. Arch Neurol 1998; 55: 151-2. PubMed  
  11. Galvin JE, Lee VM, Trojanowski JQ. Synucleinopathies: clinical and pathological implications. Arch Neurol 2001; 58: 186-90. PubMed  
  12. Årsland D. Demens med Lewy-legemer. Tidsskr Nor Lægeforen 2002; 122: 525-9. Tidsskrift for Den norske legeforening  
  13. Samuel W, Galasko D, Masliah E, Hansen LA. Neocortical Lewy body counts correlate with dementia in the Lewy body variant of Alzheimer's disease. J Neuropathol Exp Neurol 1996; 5: 44-52. PubMed  
  14. Bras J, Guerreiro R, Darwent L, Londos E, et al. Genetic analysis implicates APOE, SNCA and suggests lysosomal dysfunction in the etiology of Dementia with Lewy Bodies. Hum Mol Genet. 2014 Dec 1;23(23):6139-46. doi: 10.1093/hmg/ddu334. dx.doi.org  
  15. Duda JE, Lee VM, Trojanowski JQ. Neuropathology of synuclein aggregates. J Neurosci Res 2000; 61: 121-27. PubMed  
  16. Stubendorff K, Aarsland D, Minthon L, Londos E. The impact of autonomic dysfunction on survival in patients with dementia with Lewy bodies and Parkinson's disease with dementia. PLoS One. Published: October 01, 2012. DOI: 10.1371/journal.pone.0045451. DOI  
  17. McKeith IG, Boeve BF, Dickson DW, et al. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology 2017; 89(1): 88-100. pmid:28592453 PubMed  
  18. Walker MP, Ayre GA, Perry EK, Wesnes K, McKeith IG, Tovee M, et al. Quantification and characterization of fluctuating cognition in dementia with Lewy bodies and Alzheimer's disease. Dement Geriatr Cogn Disord 2000; 11: 327-35. PubMed  
  19. Ferman TJ, Smith GE, Boeve BF, Ivnik RJ, Petersen RC, Knopman D, et al. DLB fluctuations: specific features that reliably differentiate DLB from AD and normal aging. Neurology 2004; 62: 181-7. Neurology  
  20. Olson EJ, Boeve BF, Silber MH. Rapid eye movement sleep behaviour disorder: demographic, clinical and laboratory findings in 93 cases. Brain 2000; 123: 331-9. PubMed  
  21. Boeve BF, Silber MH, Ferman TJ. REM sleep behavior disorder in Parkinson's disease and dementia with Lewy bodies. J Geriatr Psychiatry Neurol 2004; 17: 146-57. PubMed  
  22. McKeith IG, Fairbairn A, Perry R, Thompson P, Perry E. Neuroleptic sensitivity in patients with senile dementia of Lewy body type. BMJ 1992; 305: 673-78. British Medical Journal  
  23. Knopman DS, Boeve BF, Petersen RC. Essentials of the proper diagnoses of mild cognitive impairment, dementia, and major subtypes of dementia. Mayo Clin Proc 2003; 78: 1290-308. PubMed  
  24. Mosimann UP, McKeith IG. Dementia with Lewy bodies-diagnosis and treatment. Swiss Med Wkly 2003; 133: 131-42. PubMed  
  25. Naqvi RM, Haider S, Tomlinson G, Alibhai S. Cognitive assessments in multicultural populations using the Rowland Universal Dementia Assessment Scale: a systematic review and meta-analysis. CMAJ. 2015 Mar 17;187(5):E169-75. PMID: 25691786 PubMed  
  26. SBU. Diagnostik och uppföljning av förstämningssyndrom. En systematisk litteraturöversikt. Stockholm: SBU, 2012. www.sbu.se  
  27. BMJ Best Practice. Lerner AJ: Dementia with Lewy bodies. Last reviewed and updated: August 2018. bestpractice.bmj.com  
  28. McCleery J, Morgan S, Bradley KM, Noel-Storr AH, Ansorge O, Hyde C. Dopamine transporter imaging for the diagnosis of dementia with Lewy bodies. Cochrane Database Syst Rev. 2015;1(1):CD010633. Published 2015 Jan 30. doi:10.1002/14651858.CD010633.pub2 DOI  
  29. Farlow MR. Prognosis and treatment of dementia with Lewy bodies. UpToDate, last updated Apr 15, 2020. www.uptodate.com  
  30. McKeith I, Ser TD, Spano PF, Emre M, Wesnes K, et al. Efficacy of rivastigmine in dementia with Lewy bodies: a randomised, double-blind, placebo-controlled international study. Lancet 2000; 356: 2031-36. PubMed  
  31. Emre M, Aarsland D, Albanese A et al. Rivastigmine for dementia associated with Parkinson's disease. N Engl J Med 2004; 351: 2509-18. New England Journal of Medicine  
  32. Ikeda M, Mori E, Matsuo K, Nakagawa M, Kosaka K. Donepezil for dementia with Lewy bodies: a randomized, placebo-controlled, confirmatory phase III trial. Alzheimers Res Ther. 2015;7(1):4. Published 2015 Feb 3. PMID: 25713599 PubMed  
  33. Mori E, Ikeda M, Kosaka K; Donepezil-DLB Study Investigators. Donepezil for dementia with Lewy bodies: a randomized, placebo-controlled trial. Ann Neurol. 2012;72(1):41-52. PMID: 22829268 PubMed  
  34. Wang HF, Yu JT, Tang SW, et al. Efficacy and safety of cholinesterase inhibitors and memantine in cognitive impairment in Parkinson's disease, Parkinson's disease dementia, and dementia with Lewy bodies: systematic review with meta-analysis and trial sequential analysis. J Neurol Neurosurg Psychiatry. 2015;86(2):135-143. PMID: 24828899 PubMed  
  35. Stinton C, McKeith I, Taylor JP, et al. Pharmacological Management of Lewy Body Dementia: A Systematic Review and Meta-Analysis. Am J Psychiatry. 2015;172(8):731-742. PMID: 26085043 PubMed  
  36. Bains J, Birks JS, Dening TR. The efficacy of antidepressants in the treatment of depression in dementia (Cochrane Review). In: The Cochrane Library, Issue 4, 2002. Oxford: Update Software. The Cochrane Library  
  37. Poewe W. Treatment of dementia with Lewy bodies and Parkinson's disease dementia. Mov Disord. 2005;20(suppl 12):S77-S82. PMID: 16092095 PubMed  
  38. Aarsland D, Perry R, Larsen JP, et al. Neuroleptic sensitivity in Parkinson's disease and parkinsonian dementias. J Clin Psychiatry. 2005 May;66(5):633-7. PMID: 15889951. PubMed  
  39. Piggot MA, Perry EK, McKeith IG, Marshall E, Perry RH. Dopamine D2 receptors in demented patients with severe neuroleptic sensitivity. Lancet 1994; 343: 1044-45. PubMed  
  40. Forbes D, Thiessen EJ, Blake CM, et al. Exercise programs for people with dementia. Cochrane Database Syst Rev. 2015 Apr 15;4:CD006489. PMID: 25874613. PubMed  
  41. Groot C, Hooghiemstra AM, Raijmakers PG, et al. The effect of physical activity on cognitive function in patients with dementia: A meta-analysis of randomized control trials. Ageing Res Rev. 2016 Jan;25:13-23.
  42. Larsson V, Torisson G, Bülow M, Londos E. Effects of carbonated liquid on swallowing dysfunction in dementia with Lewy bodies and Parkinson's disease dementia. Clin Interv Aging. 2017;12:1215-1222. Published 2017 Aug 8. PMID: 28848329 PubMed  
  43. Engedal K. Mortality in the elderly - a 3 year follow-up of an elderly community sample. Int J Geriatr Psychiatry 1996; 11: 467 - 71. PubMed  
  44. Williams MM, Xiong C, Morris JC, Galvin JE. Survival and mortality differences between dementia with Lewy bodies vs Alzheimer disease. Neurology 2006 Dec 12; 67(11): 1935-41. pmid:17159097 PubMed  
  45. Savica R, Grossardt BR, Bower JH, et al. Survival and Causes of Death Among People With Clinically Diagnosed Synucleinopathies With Parkinsonism: A Population-Based Study. JAMA Neurol. 2017;74(7):839-846. PMID: 2850526 PubMed  
  46. Larsson V, Torisson G, Londos E. Relative survival in patients with dementia with Lewy bodies and Parkinson's disease dementia. PLoS One. 2018;13(8):e0202044. Published 2018 Aug 10. PMID: 30096198 PubMed  
  • Elisabet Londos, professor och överläkare, Minneskliniken, Skånes universitetssjukhus
  • Anna Nager, med dr och specialist i allmänmedicin, Karolinska institutet

Tidigare sakkunniga

  • Peter D. Jost, spesialist allmennmedisin, redaktør NEL