Semin Neurol 2014; 34(02): 182-188
DOI: 10.1055/s-0034-1381741
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Dementia with Lewy Bodies

Mary Catherine Mayo
1   Department of Neurology, David Geffen School of Medicine at UCLA, Los Angeles, California
,
Yvette Bordelon
2   Movement Disorders Program, Department of Neurology, David Geffen School of Medicine at UCLA, Los Angeles, California
› Author Affiliations
Further Information

Publication History

Publication Date:
25 June 2014 (online)

Abstract

Dementia with Lewy bodies (DLB) is the second most common diagnosis of dementia after Alzheimer disease (AD). The essential pathologic feature is the Lewy body, a neuronal inclusion containing α-synuclein, found in brainstem nuclei and the neocortex. Clinical features include early fluctuations in attention, hallucinations, and parkinsonism, with progression to a combined cortical and subcortical dementia. To distinguish it from Parkinson disease dementia, a time course of one year from cognitive changes to motor feature onset has been established. There is more severe impairment of verbal fluency, executive function, and visuospatial abilities in DLB patients. Both rapid eye movement sleep behavior disorder and neuroleptic sensitivity are notable in this patient group. Treatment is aimed at symptom management. Cholinesterase inhibitors can be beneficial for behavioral and cognitive issues, whereas dopaminergic agents may help motor symptoms. Survival is equivalent to AD when measured from symptom onset, though diagnosis in DLB may be delayed.

 
  • References

  • 1 Heidebrink JL. Is dementia with Lewy bodies the second most common cause of dementia?. J Geriatr Psychiatry Neurol 2002; 15 (4) 182-187
  • 2 Kosaka K. Diffuse Lewy body disease in Japan. J Neurol 1990; 237 (3) 197-204
  • 3 Chan SS, Chiu HF, Lam LC, Leung VP. Prevalence of dementia with Lewy bodies in an inpatient psychogeriatric population in Hong Kong Chinese. Int J Geriatr Psychiatry 2002; 17 (9) 847-850
  • 4 Shergill S, Mullan E, D'Ath P, Katona C. What is the clinical prevalence of Lewy body dementia?. Int J Geriatr Psychiatry 1994; 9 11: 907-912
  • 5 Grossardt BR, Bower JH, Boeve BF, Ahlskog JE, Rocca WA. Incidence of dementia with Lewy bodies and Parkinson disease dementia. JAMA Neurol 2013; 70 (11) 1396-1402
  • 6 McKeith IG, Fairbairn AF, Perry RH, Thompson P. The clinical diagnosis and misdiagnosis of senile dementia of Lewy body type (SDLT). Br J Psychiatry 1994; 165 (3) 324-332
  • 7 Galasko D, Katzman R, Salmon DP, Hansen L. Clinical and neuropathological findings in Lewy body dementias. Brain Cogn 1996; 31 (2) 166-175
  • 8 Gardner RC, Valcour V, Yaffe K. Dementia in the oldest old: a multi-factorial and growing public health issue. Alzheimers Res Ther 2013; 5 (4) 27
  • 9 Benjamin R, Leake A, Edwardson JA , et al. Apolipoprotein E genes in Lewy body and Parkinson's disease. Lancet 1994; 343 (8912) 1565
  • 10 Mata IF, Samii A, Schneer SH , et al. Glucocerebrosidase gene mutations: a risk factor for Lewy body disorders. Arch Neurol 2008; 65 (3) 379-382
  • 11 Nalls MA, Duran R, Lopez G , et al. A multicenter study of glucocerebrosidase mutations in dementia with Lewy bodies. JAMA Neurol 2013; 70 (6) 727-735
  • 12 Lewy F, In: Lewandowsky MH, Bumke O. , eds. Handbuch der Neurologie. Vol. 3. Berlin, Germany: Springer; 1912: 920-933
  • 13 Kosaka K. Lewy bodies in cerebral cortex, report of three cases. Acta Neuropathol 1978; 42 (2) 127-134
  • 14 Kosaka K. Diffuse Lewy body disease. Neuropathology 2000; 20 (Suppl): S73-S78
  • 15 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
  • 16 Galasko D, Kosaka K, Perry EK, Dickson DW, Hansen LA, Salmon DP , 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) 1113-1124
  • 17 Overk CR, Masliah E. Pathogenesis of synaptic degeneration in Alzheimer's disease and Lewy body disease. Biochem Pharmacol 2014; 88 (4) 508-516
  • 18 Zarranz JJ, Alegre J, Gómez-Esteban JC , et al. The new mutation, E46K, of α-synuclein causes Parkinson and Lewy body dementia. Ann Neurol 2004; 55 (2) 164-173
  • 19 Eriksen JL, Dawson TM, Dickson DW, Petrucelli L. Caught in the act: alpha-synuclein is the culprit in Parkinson's disease. Neuron 2003; 40 (3) 453-456
  • 20 Schulz-Schaeffer WJ. The synaptic pathology of alpha-synuclein aggregation in dementia with Lewy bodies, Parkinson's disease and Parkinson's disease dementia. Acta Neuropathol 2010; 120 (2) 131-143
  • 21 Masliah E, Mallory M, DeTeresa R, Alford M, Hansen L. Differing patterns of aberrant neuronal sprouting in Alzheimer's disease with and without Lewy bodies. Brain Res 1993; 617 (2) 258-266
  • 22 McKeith IG, Dickson DW, Lowe J , et al; Consortium on DLB. Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium. Neurology 2005; 65 (12) 1863-1872
  • 23 Ballard C, Aarsland D, Francis P, Corbett A. Lewy bodies: pathophysiology, clinical features, and pharmacological management. Drugs Aging 2013; 30 (8) 603-611
  • 24 Ballard C, Piggott M, Johnson M , et al. Delusions associated with elevated muscarinic binding in dementia with Lewy bodies. Ann Neurol 2000; 48 (6) 868-876
  • 25 Sharp SI, Ballard CG, Ziabreva I , et al. Cortical serotonin 1A receptor levels are associated with depression in patients with dementia with Lewy bodies and Parkinson's disease dementia. Dement Geriatr Cogn Disord 2008; 26 (4) 330-338
  • 26 Weisman D, McKeith I. Dementia with Lewy bodies. Semin Neurol 2007; 27 (1) 42-47
  • 27 Hickey C, Chisholm T, Passmore MJ, O'Brien JD, Johnston J. Differentiating the dementias. Revisiting synucleinopathies and tauopathies. Curr Alzheimer Res 2008; 5 (1) 52-60
  • 28 Camicioli R, Fisher N. Progress in clinical neurosciences: Parkinson's disease with dementia and dementia with Lewy bodies. Can J Neurol Sci 2004; 31 (1) 7-21
  • 29 Aarsland D, Perry R, Larsen JP , et al. Neuroleptic sensitivity in Parkinson's disease and parkinsonian dementias. J Clin Psychiatry 2005; 66 (5) 633-637
  • 30 Grace J, Daniel S, Stevens T , et al. Long-Term use of rivastigmine in patients with dementia with Lewy bodies: an open-label trial. Int Psychogeriatr 2001; 13 (2) 199-205
  • 31 Ballard CG, Rowan EN, Kenny RA. Incidence and prediction of falls in dementia: a prospective study in older people. PLoS ONE 2009; 4 (5) e5521
  • 32 Ropper AH, Samuels MA. Adams and Victor's Principles of Neurology. 9th ed. New York, NY: McGraw-Hills; 2009
  • 33 Shimomura T, Mori E, Yamashita H , et al. Cognitive loss in dementia with Lewy bodies and Alzheimer disease. Arch Neurol 1998; 55 (12) 1547-1552
  • 34 Collerton D, Burn D, McKeith I, O'Brien J. Systematic review and meta-analysis show that dementia with Lewy bodies is a visual-perceptual and attentional-executive dementia. Dement Geriatr Cogn Disord 2003; 16 (4) 229-237
  • 35 Salmon DP, Galasko D, Hansen LA , et al. Neuropsychological deficits associated with diffuse Lewy body disease. Brain Cogn 1996; 31 (2) 148-165
  • 36 Gnanalingham KK, Byrne EJ, Thornton A, Sambrook MA, Bannister P. Motor and cognitive function in Lewy body dementia: comparison with Alzheimer's and Parkinson's diseases. J Neurol Neurosurg Psychiatry 1997; 62 (3) 243-252
  • 37 Geser F, Wenning GK, Poewe W, McKeith I. How to diagnose dementia with Lewy bodies: state of the art. Mov Disord 2005; 20 (Suppl. 12) S11-S20
  • 38 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) 1-8
  • 39 Calderon J, Perry RJ, Erzinclioglu SW, Berrios GE, Dening TR, Hodges JR. Perception, attention, and working memory are disproportionately impaired in dementia with Lewy bodies compared with Alzheimer's disease. J Neurol Neurosurg Psychiatry 2001; 70 (2) 157-164
  • 40 Gill DP, Hubbard RA, Koepsell TD , et al. Differences in rate of functional decline across three dementia types. Alzheimers Dement 2013; 9 (5, Suppl) S63-S71
  • 41 Walker Z, McKeith I, Rodda J , et al. Comparison of cognitive decline between dementia with Lewy bodies and Alzheimer's disease: a cohort study. BMJ Open 2012; 2: e000380
  • 42 Hanyu H, Sato T, Hirao K, Kanetaka H, Sakurai H, Iwamoto T. Differences in clinical course between dementia with Lewy bodies and Alzheimer's disease. Eur J Neurol 2009; 16 (2) 212-217
  • 43 Ballard C, O'Brien J, Morris CM , et al. The progression of cognitive impairment in dementia with Lewy bodies, vascular dementia and Alzheimer's disease. Int J Geriatr Psychiatry 2001; 16 (5) 499-503
  • 44 Walker Z, Allen RL, Shergill S, Mullan E, Katona CL. Three years survival in patients with a clinical diagnosis of dementia with Lewy bodies. Int J Geriatr Psychiatry 2000; 15 (3) 267-273
  • 45 Nelson PT, Jicha GA, Kryscio RJ , et al. Low sensitivity in clinical diagnoses of dementia with Lewy bodies. J Neurol 2010; 257 (3) 359-366
  • 46 Barber R, Gholkar A, Scheltens P, Ballard C, McKeith IG, O'Brien JT. Medial temporal lobe atrophy on MRI in dementia with Lewy bodies. Neurology 1999; 52 (6) 1153-1158
  • 47 Barber R, Ballard C, McKeith IG, Gholkar A, O'Brien JT. MRI volumetric study of dementia with Lewy bodies: a comparison with AD and vascular dementia. Neurology 2000; 54 (6) 1304-1309
  • 48 Chow N, Aarsland D, Honarpisheh H , et al. Comparing hippocampal atrophy in Alzheimer's dementia and dementia with Lewy bodies. Dement Geriatr Cogn Disord 2012; 34 (1) 44-50
  • 49 Watson R, Blamire AM, Colloby SJ , et al. Characterizing dementia with Lewy bodies by means of diffusion tensor imaging. Neurology 2012; 79 (9) 906-914
  • 50 Bozzali M, Falini A, Cercignani M , et al. Brain tissue damage in dementia with Lewy bodies: an in vivo diffusion tensor MRI study. Brain 2005; 128 (Pt 7) 1595-1604
  • 51 Firbank MJ, Blamire AM, Teodorczuk A, Teper E, Mitra D, O'Brien JT. Diffusion tensor imaging in Alzheimer's disease and dementia with Lewy bodies. Psychiatry Res 2011; 194 (2) 176-183
  • 52 Mosconi L, Tsui WH, Herholz K , et al. Multicenter standardized 18F-FDG PET diagnosis of mild cognitive impairment, Alzheimer's disease, and other dementias. J Nucl Med 2008; 49 (3) 390-398
  • 53 Perneczky R, Haussermann P, Diehl-Schmid J , et al. Metabolic correlates of brain reserve in dementia with Lewy bodies: an FDG PET study. Dement Geriatr Cogn Disord 2007; 23 (6) 416-422
  • 54 Imamura T, Ishii K, Sasaki M , et al. Regional cerebral glucose metabolism in dementia with Lewy bodies and Alzheimer's disease: a comparative study using positron emission tomography. Neurosci Lett 1997; 235 (1-2) 49-52
  • 55 Zhao P, Zhang B, Gao S. 18F-FDG PET study on the idiopathic Parkinson's disease from several parkinsonian-plus syndromes. Parkinsonism Relat Disord 2012; 18 (Suppl. 01) S60-S62
  • 56 Lobotesis K, Fenwick JD, Phipps A , et al. Occipital hypoperfusion on SPECT in dementia with Lewy bodies but not AD. Neurology 2001; 56 (5) 643-649
  • 57 Bonanni L, Thomas A, Onofrj M. Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium. Neurology 2005; 65 (12) 1863-1872
  • 58 Yoshita M, Taki J, Yokoyama K , et al. Value of 123I-MIBG radioactivity in the differential diagnosis of DLB from AD. Neurology 2006; 66 (12) 1850-1854
  • 59 Kaerst L, Kuhlmann A, Wedekind D, Stoeck K, Lange P, Zerr I. Using cerebrospinal fluid marker profiles in clinical diagnosis of dementia with Lewy bodies, Parkinson's disease, and Alzheimer's disease. J Alzheimers Dis 2014; 38 (1) 63-73
  • 60 Mulugeta E, Londos E, Ballard C , et al. CSF amyloid β38 as a novel diagnostic marker for dementia with Lewy bodies. J Neurol Neurosurg Psychiatry 2011; 82 (2) 160-164
  • 61 Shea C, MacKnight C, Rockwood K. Donepezil for treatment of dementia with Lewy bodies: a case series of nine patients. Int Psychogeriatr 1998; 10 (3) 229-238
  • 62 Lanctôt KL, Herrmann N. Donepezil for behavioural disorders associated with Lewy bodies: a case series. Int J Geriatr Psychiatry 2000; 15 (4) 338-345
  • 63 Samuel W, Caligiuri M, Galasko D , et al. Better cognitive and psychopathologic response to donepezil in patients prospectively diagnosed as dementia with Lewy bodies: a preliminary study. Int J Geriatr Psychiatry 2000; 15 (9) 794-802
  • 64 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
  • 65 Maclean LE, Collins CC, Byrne EJ. Dementia with Lewy bodies treated with rivastigmine: effects on cognition, neuropsychiatric symptoms, and sleep. Int Psychogeriatr 2001; 13 (3) 277-288
  • 66 McKeith I, Del Ser T, Spano P , et al. Efficacy of rivastigmine in dementia with Lewy bodies: a randomised, double-blind, placebo-controlled international study. Lancet 2000; 356 (9247) 2031-2036
  • 67 Larsson V, Engedal K, Aarsland D, Wattmo C, Minthon L, Londos E. Quality of life and the effect of memantine in dementia with Lewy bodies and Parkinson's disease dementia. Dement Geriatr Cogn Disord 2011; 32 (4) 227-234
  • 68 Wesnes KA, Aarsland D, Ballard C, Londos E. Memantine improves attention and episodic memory in Parkinson's disease dementia and dementia with Lewy bodies. Int J Geriatr Psychiatry 2014;
  • 69 Fernandez HH, Trieschmann ME, Burke MA, Friedman JH. Quetiapine for psychosis in Parkinson's disease versus dementia with Lewy bodies. J Clin Psychiatry 2002; 63 (6) 513-515
  • 70 Goldmann Gross R, Siderowf A, Hurtig HI. Cognitive impairment in Parkinson's disease and dementia with Lewy bodies: a spectrum of disease. Neurosignals 2008; 16 (1) 24-34
  • 71 Nyth AL, Gottfries CG. The clinical efficacy of citalopram in treatment of emotional disturbances in dementia disorders. A Nordic multicentre study. Br J Psychiatry 1990; 157: 894-901
  • 72 Zak RS, Maganti RK, Auerbach SH, Casey KR, Chowdhuri S, Karippot A , et al. Best practice guide for the treatment of REM sleep behavior disorder (RBD). J Clin Sleep Med 2010; 6 (1) 85-95
  • 73 Goldman JG, Goetz CG, Brandabur M, Sanfilippo M, Stebbins GT. Effects of dopaminergic medications on psychosis and motor function in dementia with Lewy bodies. Mov Disord 2008; 23 (15) 2248-2250
  • 74 McKeith I, Fairbairn A, Perry R, Thompson P, Perry E. Neuroleptic sensitivity in patients with senile dementia of Lewy body type. BMJ 1992; 305 (6855) 673-678
  • 75 Hanagasi HA, Bilgiç B, Emre M. Neuroimaging, biomarkers, and management of dementia with Lewy bodies. Front Neurol 2013; 4: 151