Abstract
Neurocognitive disorder (NCD) with Lewy bodies is a leading cause of non-Alzheimer disease-related NCDs. It is frequently underdiagnosed and misdiagnosed as other NCDs, Parkinson disease-related NCD, or major depressive disorder. It should be considered in the differential of new-onset cognitive impairment or parkinsonian symptoms in the older adults. It is marked by a combination of motor, sleep, and autonomic dysfunction and neurocognitive and neuropsychiatric symptoms. Definitive diagnosis can only be made on autopsy. However, the DSM-5 and the Lewy body NCD Consortium have developed a set of criteria for diagnosis based on core and suggestive features of the disease. Development of these symptoms within 1 year of each other strongly suggests a diagnosis of NCD with Lewy bodies. Treatment with cholinesterase inhibitors may help slow the progression of cognitive deterioration. Low-potency antipsychotics may be considered for treatment of hallucinations if benefits outweigh the risks of antipsychotic sensitivity.
Notes
- 1.
AD-NCD, neurocognitive disorder due to Alzheimer disease; LB-NCD, neurocognitive disorder with Lewy bodies; PD-NCD, neurocognitive disorder due to Parkinson disease.
References
Hugo J, Ganguli M. Dementia and cognitive impairment epidemiology, diagnosis, and treatment. Clin Geriatr Med. 2014;30(3):421–42.
Kim WS, Kågedal K, Halliday GM. Alpha-synuclein biology in Lewy body diseases. Alzheimers Res Ther. 2014;6(5):73.
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;47:1113–24.
McKeith I, Perry E, Perry R. Report of the second dementia with Lewy body international workshop: diagnosis and treatment. Neurology. 1999;53:902–5.
McKeith I, Dickson D, Lowe J, et al. Diagnosis and management of dementia with Lewy bodies: third report of the DLB consortium. Neurology. 2005;65:1863–72.
American Psychiatric Association. Diagnostic and statistical manual of mental disorders. 5th ed. Arlington: American Psychiatric Publishing; 2013. p. 618.
Litvan I, Goldman JG, Tröster AI, et al. Diagnostic criteria for mild cognitive impairment in Parkinson’s disease: movement disorder society task force guidelines. Mov Disord. 2012;24:489–95.
Emre M, Aarsland D, Brown R, et al. Clinical diagnostic criteria for dementia associated with Parkinson’s disease. Mov Disord. 2007;22(12):1689–707.
Walker Z, Possin K, Boeve B, Aarsland D. Lewy body dementias. Lancet. 2015;38(6):1683–97.
Zupancic M, Mahajan A, Handa K. Dementia with Lewy bodies: diagnosis and management for primary care providers. Prim Care Companion CNS Disord. 2011;13(5):PCC.11r01190.
Yamada T, Hattori H, Miura A, et al. Prevalence of Alzheimer’s disease, vascular dementia and dementia with Lewy bodies in a Japanese population. Psychiatry Clin Neurosci. 2001;55(1):21–5.
Stevens T, Livingston G, Kitchen G, et al. Islington study of dementia subtypes in the community. Br J Psychiatry. 2002;180:270–6.
De Silva HA, Gunatilake SB, Smith AD. Prevalence of dementia in a semi-urban population in Sri Lanka: report from a regional survey. Int J Geriatr Psychiatry. 2003;18(8):711–5.
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.
Rahkonen T, Eloniemi-Sulkava U, Rissanen S, et al. Dementia with Lewy bodies according to the consensus criteria in a general population aged 75 years or older. J Neurol Neurosurg Psychiatry. 2003;74(6):720–4.
Miech RA, Breitner JC, Zandi PP, et al. Incidence of AD may decline in the early 90s for men, later for women: the Cache County study. Neurology. 2002;58:209–18.
Savica R, Grossardt BR, Bower JH, et al. Incidence of dementia with Lewy bodies and Parkinson disease dementia. JAMA Neurol. 2013;70(11):1396–402.
Geser F, Wenning GK, Poewe W, McKeith IG. How to diagnose dementia with Lewy bodies: state of the art. Mov Disord. 2005;20:S11–20.
Savica R, Grossardt BR, Bower JH, et al. Incidence and time trends of drug-induced parkinsonism: a 30-year population-based study. Mov Disord. 2017;32(2):227–34.
McKeith IG. Dementia with Lewy bodies. Br J Psychiatry. 2002;180(2):144–7.
Bach JP, Ziegler U, Deuschl G, et al. Projected numbers of people with movement disorders in the years 2030 and 2050. Mov Disord. 2011;26:2286–90.
Zahirovic I, Wattmo C, Torisson G, et al. Prevalence of dementia with Lewy body symptoms: a cross-sectional study in 40 Swedish nursing homes. J Am Med Dir Assoc. 2016;17(8):706–11.
Dickson DW, Brakk H, Duda JE, et al. Neuropathological assessment of Parkinsons disease: refining the diagnostic criteria. Lancet Neurol. 2009;8(12):1150–7.
Kasuga K, Nishizawa M, Ikeuchi T. α-Synuclein as CSF and blood biomarker of dementia with Lewy bodies. Int J Alzheimers Dis. 2012;2012:437025. https://doi.org/10.1155/2012/437025.
Meeus B, Theuns J, Van Broeckhoven C. The genetics of dementia with Lewy bodies. Arch Neurol. 2012;69(9):1113–8.
Butler B, Sambo D, Khoshbouei H. Alpha-synuclein modulates dopamine neurotransmission. J Chem Nueroanat. 2016;pii:S0891–618.
Qing H. Lrrk2 phosphorylates alpha synuclein at serine 129: Parkinson’s disease implications. Biochem Biophys Res Commun. 2009;387:149–52.
Mata IF. LRRK2 in Parkinson’s disease: protein domains and functional insights. Trends Neurosci. 2006;29:286–93.
Meesus B, Nuytemans K, Crosiers D, et al. Comprehensive genetic and mutation analysis of familial dementia with Lewy bodies linked to 2q35-q36. Arch Neurol. 2010;30(1):197–205.
Ogaki K, Koga S, Heckman M, et al. Mitochondrial targeting sequence variants of the CHCHD2 gene are a risk for Lewy body disorders. Neurology. 2015;85:2016–25.
Spano M, Spignorelli M, Vitaliani R, Aguglia E, Giometto B. The possible involvement of mitochondrial dysfunctions in Lewy body dementia: a systematic review. Funct Neurol. 2015;30(3):151–8.
Bras J, Guerreiro R, Darwent L, et al. Genetic analysis implicates APOE, SNCA and suggests lysosomal dysfunction in the etiology of dementia with Lewy bodies. Hum Mol Genet. 2014;23:6139–46.
Crews L, Spencer B, Desplats P, et al. Selective molecular alterations in the autophagy pathway in patients with Lewy body and in models of α-synucleinopathy. PLoS One. 2010;5(2):e9313.
Stemberger S, Scholz SW, Singleton AB, Wenning GK. Genetic players in multiple system atrophy: unfolding the nature of the beast. Neurobiol Aging. 2011;1924:e5–e14.
Alexander GE. Biology of Parkinson’s disease: pathogenesis and pathophysiology of a multisystem neurodegenerative disorder. Dialogues Clin Neurosci. 2004;6(3):259–80.
Collerton D, Burn D, McKeith IG, 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):229–37.
Nakatsuka T, Imabayashi E, Matsuda H, et al. Discrimination of dementia with Lewy bodies from Alzheimer’s disease using voxel-based morphometry of white matter by statistical parametric mapping 8 plus diffeomorphic anatomic registration through exponentiated lie algebra. Neuroradiology. 2013;55(5):559–66.
Fukui T, Hori K, Yoshimasu H. Onset patterns and initial symptoms of dementia with Lewy bodies: possible pathophysiological diversities deduced from a SPECT study. Dement Geriatr Cogn Disord Extra. 2011;1(1):237–48.
Boeve B. Clinical, diagnostic, genetic and management issues in dementia with Lewy bodies. Clin Sci. 2005;109:343–54.
Kanazawa M, Sanpei K, Toyoshima Y, et al. An autopsy case of dementia with Lewy bodies showing autonomic failure and dementia as the initial symptoms. Mov Disord. 2007;22:1212–3.
Kaufmann H, Nahm K, Purohit D, Wolfe D. Autonomic failure as the initial presentation of Parkinson disease and dementia with Lewy bodies. Neurology. 2004;63(6):1093–5.
Turner RS. Idiopathic rapid eye movement sleep behavior disorder is a harbinger of dementia with Lewy bodies. J Geriatr Psychiatry Neurol. 2002;15(4):195–9.
Dugger BN, Boeve BF, Murray ME, et al. Rapid eye movement sleep behavior disorder and subtypes in autopsy-confirmed dementia with Lewy bodies. Mov Disord. 2012;27(1):72–8.
Parkkinen L, Pirttila T, Alafuzoff I. Applicability of current staging/categorization of α-synuclein pathology and their clinical relevance. Acta Neuropathol. 2008;115:399–407.
Aarsland D. Cognitive impairment in Parkinson’s disease and dementia with Lewy bodies. Parkinsonism Relat Disord. 2016;22(1):S144–8.
Simard M, Van Reekum R, Cohen T. A review of the cognitive and behavioral symptoms in dementia with Lewy bodies. J Neuropsychiatry Clin Neurosci. 2000;12(4):425–50.
Classen DO, Parisi JE, Giannini C, et al. Frontotemporal dementia mimicking dementia with Lewy bodies. Cogn Behav Neurol. 2008;21(3):157–63.
Bradshaw J, Saling M, Hopwood M, Anderson V, Brodtmann A. Fluctuating cognition in dementia with Lewy bodies and Alzheimer’s disease is qualitatively distinct. J Neurol Neurosurg Psychiatry. 2004;75(3):382–7.
Delli Pizzi S, Franciotti R, Taylor JP, et al. Structural connectivity is differently altered in dementia with Lewy body and Alzheimer’s disease. Front Aging Neurosci. 2015;7:208.
Ferman TJ, Smith GE, Dickson DW, et al. Abnormal daytime sleepiness in dementia with Lewy bodies compared to Alzheimer’s disease using the multiple sleep latency test. Alzheimers Res Ther. 2014;6(9):76.
Taylor JP, Firbank M, O’Brien JT. Visual cortical excitability in dementia with Lewy bodies. Br J Psychiatry. 2015;208:497–8.
Khundakar AA, Hanson PS, Erskine D, et al. Analysis of primary visual cortex in dementia with Lewy bodies indicates GABAergic involvement associated with recurrent complex visual hallucinations. Acta Neuropathol Commun. 2016;4:66.
Thaipisutikul P, Lobach I, Zweig Y, Gurnani A, Galvin JE. Capgras syndrome in dementia with Lewy bodies. Int Psychogeriatr. 2013;25(5):843–9.
Marantz AG, Verghese J. Capgras’ syndrome in dementia with Lewy bodies. J Geriatr Psychiatry Neurol. 2002;15(4):239–41.
Kobayashi K, Nakano H, Akiyama N, et al. Pure psychiatric presentation of the Lewy body disease is depression—an analysis of 60 case verified with myocardial meta-iodobenzylguanidine study. Int J Geriatr Psychiatry. 2015;30(6):663–8.
Walker Z, Possin K, Boeve B, Aarsland D. Lewy body dementias. Lancet. 2015;386:1683–97.
Boeve BF, Silber MH, Ferman TJ, Lucas JA, Parisi JE. Association of REM sleep behavior disorder and neurodegenerative disease may reflect an underlying synucleinopathy. Mov Disord. 2001;16:622–30.
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. 2012;7(10):e45451.
Thaisetthawatkul P, Boeve BF, Benarroch EE, et al. Autonomic dysfunction in dementia with Lewy bodies. Neurology. 2004;62:1804–9.
Postuma RB, Gagnon JF, Pelletier A, Montplaisir J. Prodromal autonomic symptoms and signs in Parkinson’s disease and dementia with Lewy bodies. Mov Disord. 2013;28:597–604.
Donaghy PC, McKeith IG. The clinical characteristics of dementia with lewy body and a consideration of prodromal diagnosis. Alzheimers Res Ther. 2014;6:46.
Walker MP, Ayer GA, Cummings JL, et al. Quantifying fluctuation in dementia with Lewy bodies, Alzheimer’s disease, and vascular dementia. Neurology. 2000;54(8):1616–25.
Ferman TJ, Smith GE, Boeve BF, et al. DLB fluctuations: specific features that reliably differentiate DLB from AD and normal aging. Neurology. 2004;62:181–7.
Lee DR, Taylor JP, Thomas AJ. Assessment of cognitive fluctuation in dementia: a systematic review of the literature. Int J Geriatr Psychiatry. 2012;27:989–98.
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):243–52.
Bibl M, Esselmann H, Wiltfang J. Neurochemical biomarkers in Alzheimer’s disease and related disorders. Ther Adv Neurol Disord. 2012;5(6):335–48.
Micanovic D, Pal S. The diagnostic utility of EEG in early-onset dementia: a systematic review of the literature with narrative analysis. J Neural Transm (Vienna). 2014;12(1):58–69.
McKeith IG, O’Brien J, Walker Z, et al. Sensitivity and specificity of dopamine transporter imaging with 123I-FP-CIT SPECT in dementia with Lewy bodies: a phase III, multicentere study. Lancet Neurol. 2007;6(4):305–13.
Filippi M, Kulisevsky J. Advances with MRI in Parkinson disease: from freezing to festination. Neurology. 2012;79:1–2.
Estorch M, Camacho V, Paredres P, et al. Carciac (123) I-metaiodobenzylguandinie imaging allows early identification of dementia with lewy bodies during life. Eur J Nucl Med Mol Imaging. 2008;35(9):1636–41.
Ferman TJ, Boeve BF. Dementia with Lewy bodies. Neurol Clin. 2007;25(3):741–60.
McKeith IG, 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):2031–6.
Mori E, Ikeda M, Kosaka K. On behalf of the donepezil-DLB study investigators. Donepezil for dementia with Lewy bodies: a randomized, placebo-controlled trial. Ann Neurol. 2012;72:41–52.
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. 2015;30(1):46–54.
Stinton C, McKeith IG, Taylor JP, et al. Pharmacological Management of Lewy body dementia: a systematic review and meta-analysis. Am J Psychiatry. 2015;172(8):731–42.
Chacko RC, Hyrley RA, Jonkovic J. Clozapine use in diffuse Lewy body disease. J Neuropsychiatry Clin Neurosci. 1993;5:206–8.
Dewey RJ, O’Suilleabhain P. Treatment of drug-induced psychosis with quetiapine and clozapine in Parkinson’s disease. Neurology. 2000;55:1753–4.
Leopold NA. Risperidone treatment of drug related psychosis in patients with parkinsonism. Mov Disord. 2000;15:301–4.
Takahashi H, Yoshida K, Sugita T, et al. Quetiapine treatment of psychotic symptoms and aggressive behavior in patients with dementia with Lewy bodies: a case series. Prog Neruopsychopharmacol Biol Psychiatry. 2003;27(3):549–53.
Cummings JL, Street J, Masterman D, Clark WS. Efficacy of olanzapine in the treatment of psychosis in dementia with Lewy bodies. Dement Geriatr Cogn Disord. 2002;13(2):67–73.
Schneider L, Dagerman K, Insel P. Efficacy and adverse effects of atypical antipsychotics for dementia: meta-analysis of randomized, placebo-controlled trials. Am J Geriatr Psychiatry. 2006;14:191–210.
Cummings J, Isaacson S, Mills R, et al. Pimavanserin for patients with Parkinson’s disease psychosis: a randomized, placebo-controlled phase 3 trial. Lancet. 2013;383(9916):533–44.
Molloy S, McKeith I, O’Brien J, Burn D. The role of levodopa in the management of dementia with Lewy bodies. J Neurol Neurosurg Psychiatry. 2005;76(9):1200–3.
Zellner M, Madersbacher H, Palmtag H, et al. Trospium chloride and oxybutynin hydrochloride in a German study of adults with urinary urge incontinence: results of a 12-week, multicenter, randomized, double-blind, parallel-group, flexible-dose non inferiority trial. Clin Ther. 2009;31(11):2519–39.
McGrane IR, Leung JG, St Louis EK, Boeve BF. Melatonin therapy for REM sleep behavior disorder: a critical review of evidence. Sleep Med. 2015;16(1):19–26.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2018 Springer International Publishing AG, part of Springer Nature
About this chapter
Cite this chapter
How, P.C., Lo, P., Xiong, G.L. (2018). Major or Mild Neurocognitive Disorders with Lewy Bodies. In: Hategan, A., Bourgeois, J., Hirsch, C., Giroux, C. (eds) Geriatric Psychiatry. Springer, Cham. https://doi.org/10.1007/978-3-319-67555-8_20
Download citation
DOI: https://doi.org/10.1007/978-3-319-67555-8_20
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-67554-1
Online ISBN: 978-3-319-67555-8
eBook Packages: MedicineMedicine (R0)