Abstract
Dementia with Lewy bodies (DLB), the second most common cause of dementia, remains a difficult condition to accurately diagnose and manage. Variable involvement of motor and cognitive functions, plus psychiatric and behavioral symptoms, contributes to the difficulty in managing DLB. Additionally, DLB can cause severe sleep disruption through REM sleep behavior disorder, autonomic symptoms, disruptions of olfaction/taste and mood, hallucinations, and more. In this chapter, advances and remaining challenges in the diagnosis of DLB are discussed, including a review of the current consensus criteria for DLB. The spectrum of disorders with Lewy bodies (LBs) are described including their wide-range of clinical presentations and overlap with Alzheimer’s disease (AD) and Parkinson’s disease with and without dementia. Particular consideration is given to advancements in quantification of cognitive fluctuations through improved clinical instruments, EEG, and other advanced biomarkers. Detection of DLB has improved, but establishing the “primary” pathology in cases with concomitant LB andd AD remains difficult. Likelihood of a clinical DLB syndrome is thought to be a function of distribution of LBs and severity of AD-type pathology. Further work is needed to better understand LB disease subtypes and the underlying pathophysiological mechanisms to allow for more targeted and comprehensive therapies.
Highlights
Dementia with Lewy bodies (DLB) is generally accepted to be the second most common cause of dementia [1–4] accounting for approximately 20% of cases in the Western world, second in prevalence to only Alzheimer’s disease (AD) [5]
In patients who present with dementia and parkinsonism, the 1-year rule is recommended to differentiate between DLB and PDD
It should be noted that these advances have improved in the detection of DLB, but the attribution of which disease process is “primary” remains difficult in cases with concomitant LB and AD pathology
Summary
Dementia with Lewy bodies (DLB) is generally accepted to be the second most common cause of dementia [1–4] accounting for approximately 20% of cases in the Western world, second in prevalence to only Alzheimer’s disease (AD) (which is usually accompanied by some degree of cerebrovascular disease) [5]. DLB can cause severe disruption of sleep with REM sleep behavior disorder (RBD), autonomic symptoms, disruptions of olfaction/taste and mood, hallucinations, and more. These patients often have adverse effects of medications such as neuroleptic sensitivity, even to atypical antipsychotics, worsening of orthostatic hypotension by L-Dopa, and behavioral disinhibition to clonazepam and other benzodiazepines taken to treat RBD. After reviewing the composition and regional distribution of Lewy bodies, this chapter will focus on the advances and remaining challenges in the diagnosis of DLB, rather than therapies and management, for which the reader is referred to other chapters in this volume. The spectrum of disorders with Lewy bodies will be described, with their wide range of clinical presentations and overlap with AD, as well as Parkinson’s disease (PD) with and without dementia
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