Behavioral and Neurophysiological Characteristics of Lewy Body Dementia Implications for Intervention
Behavioral and Neurophysiological Characteristics of Lewy Body Dementia Implications for Intervention
With the aging of the population, promotion of cognitive health has emerged as a priority in public health. Most notably, Alzheimer's disease (AD) alone remained one of the top 10 causes of mortality throughout the 20th century.1 The public health implications of cognitive status increase dramatically when it is recognized that AD is but one form of cognitive impairment, which, in addition to including several forms of dementia, also encompasses mild cognitive impairment,2 which is often, arguably, a predictor of the emergence of a dementing illness.
Another common form of dementia is Lewy body dementia (LBD). As its name suggests, LBD is characterized by the presence of Lewy bodies— eosinophilic inclusion bodies—in the brainstem and cerebral cortex and has been roughly estimated to comprise about 25% of all dementia cases.3 In this article we examine the behavioral and diagnostic characteristics and neuropathology of LBD, and summarize the latest findings concerning the treatment of this disease.
Studies selected for this review were identified by a search of PubMed for literature referenced to the keywords ‘Lewy body dementia’ or ‘dementia of the Lewy bodies’ and ‘epidemiology.’ In order to ensure the timeliness and clinical relevance of the investigations included, studies selected were limited to those published in the last 10 years featuring human subjects, which were written in English and appeared in core clinical journals (n=39). Furthermore, studies were generally limited to those that largely focused on LBD and provided definitional or diagnostic criteria and a specified interval of observation (n=24), with additional studies being added by expert input.
Results—Behavioral and Diagnostic Characteristics
Although AD is the most common form of dementia in older adults,4 investigators claim that LBD accounts for between 15 and 20% of lateonset cases of dementia.5,6 Part of the prevalence variance may stem from the use of different criteria for the diagnosis of LBD. Diagnostic criteria for LBD have evolved over the years. As summarized by Graham, Ballard, and Saad,7 the Nottingham Criteria for Dementia with Lewy Bodies included dementia having a gradual onset, pronounced deficits in attention or episodes of acute confusion, and parkinsonism (nervous disorders similar to those seen in Parkinson’s disease [PD]), slow or restricted body movements, postural problems, resting tremor, or rigidity.
Contemporary diagnostic criteria delineated in the third report of the Dementia with Lewy Bodies (DLB) consortium include progressive cognitive decline, prominent or persistent memory impairment (typically evident with disease progression), and prominent deficits in tests of attention, visuospatial ability, and executive function as central features essential for the diagnosis of possible or probable DLB. Cognitive fluctuation with marked variations in alertness and attention, recurrent visual hallucinations, and features of parkinsonism are considered core features of DLB, two of which must be present for a diagnosis of probable DLB. Suggestive features of DLB include, among others, rapid eye movement (REM) sleep behavior disorder (RBD) and severe neuroleptic sensitivity. Repeated falls and syncope, severe autonomic dysfunction, and transient, unexplained loss of consciousness are among an array of criteria comprising supportive features of DLB.8
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- Luck T, Riedel-Heller SG, Kaduszkiewicz H, et al., Mild cognitive impairment in general practice: Age-specific prevalence and correlate results from the German Study on Ageing, Cognition, and Dementia in Primary Care Parients (AgeCoDe), Dement Geriatr Cogn Disord, 2007;24:307 16.
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- Barker WW, Luis CA, Kashuba A, et al., 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. 2002;16:203 12.
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- Graham C, Ballard C, Saad K, Variables which distinguish patients fulfilling clinical criteria for Dementia with Lewy Bodies from those with Alzheimer's Disease, Int J Geriatr Psychiat, 1997;12:314 18.
- McKeith IG, Dickson DW, Lowe J, et al., Diagnosis and management of dementia with Lewy bodies: Third report of the DLB consortium, Neurology, 2005;65:1863 72.
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- Manford M, Andermann F, Complex visual hallucinations: Clinical and neurobiological insights, Brain, 1998;121:1819 40.
- Trojanowski JQ, Lee VM-Y, Aggregation of neurofilament and alpha-synuclein proteins in Lewy Bodies: Implications for the pathogenesis of Parkinson Disease and Lewy Body Dementia, Arch Neurol, 1998;55:151 2.
- Galvin JE, Malcom H, Johnson D, Morris JC, Personality traits distinguishing dementia with Lewy bodies from Alzheimer disease, Neurology, 2007;68:1895 1901.
- Serby M, Samuels SC, Diagnostic criteria for dementia with Lewy bodies reconsidered, Am J Geriatr Psychiatry, 2001;9:212 16.
- Ballard C, Ziabreva I, Perry R, et al., Differences in neuropathologic characteristics across the Lewy body dementia spectrum, Neurology, 2006;67:1931 4.
- Ballard CG, Jacoby R, Del Ser T, et al., Neuropathological substrates of psychiatric symptoms in prospectively studied patients with autopsy-confirmed dementia with Lewy bodies, Am J Psychiatry, 2004;161:843 9.
- Weisman D, Cho M, Taylor C, et al., In dementia with Lewy bodies, Braak stage determines phenotype, not Lewy body distribution, Neurology, 2007;69:356 9.
- Woodruff BK, Graff- Radford NR, Ferman TJ, et al., Family history of dementia is a risk factor for Lewy body disease, Neurology, 2006;66:1949 50.
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- Turner RS, D Amato CJ, Chervin RD, Blaivas M, The pathology of REM sleep behavior disorder with comorbid Lewy body dementia, Neurology, 2000;55:1730 32.
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- Bombois S, Maurage C-A, Gompel M, et al., Absence of betaamyloid deposits after immunization in Alzheimer disease with Lewy Body dementia, Arch Neurol, 2007;64:583 7.
- Conn DK, Cholinesterase inhibitors: Comparing the options for mild-to-moderate dementia, Geriatrics, 2001;56:56 7.
- Onofrj M, Thomas A, Severe worsening of parkinsonism in Lewy body dementia due to donepizil, Neurology, 2003;61:1452.
- Rozzini L, Ghianda D, Trabucchi M, et al., Severe worsening of parkinsonism in Lewy body dementia due to donepzil, Neurology, 2004;63:1543 4.
- McKeith I, Del Sar T, Spano P, et al., Efficacy of rivastigmine in dementia with Lewy bodies: A randomized, double-blind, placebocontrolled international study, Lancet, 2000;356:2031 6.
