Introduction
Methods
Search Strategy and Selection Criteria
Data Analysis
Ethical Considerations
Results
RCTs
Disease Course or Progression
Cognitive Decline
Survival and Mortality
Pharmaceutical Impact on Life Expectancy
Clinical Management
Consensus Guidelines
Organisation | Region | Reference details |
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BAP [45] | UK | O’Brien et al. Clinical practice with anti-dementia drugs: a revised (third) consensus statement from the British Association for Psychopharmacology. Journal of Psychopharmacology. 2017;31(2):147–168 |
DLBC [46] | International | McKeith et al. Diagnosis and management of dementia with Lewy bodies. Neurology. 2017;89(1):88–100 |
EFNS-ENS Joint Congress of European Neurology [47] | Europe | Sorbi et al. EFNS-ENS guidelines on the diagnosis and management of disorders associated with dementia. European Journal of Neurology. 2012;19(9):1159–1179 |
Diagnosis and Treatment Pathway
Clinical Diagnosis
Revised criteria for the clinical diagnosis of probable and possible DLB | |
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Essential for a diagnosis of DLB | |
Dementia, defined as a progressive cognitive decline of sufficient magnitude to interfere with normal social or occupational functions or with usual daily activities Prominent or persistent memory impairment may not necessarily occur in the early stages but is usually evident with progression Deficits on tests of attention, executive function, and visuoperceptual ability may be especially prominent and occur early | |
Clinical features | |
Core clinical features | Supportive clinical features |
Fluctuating cognition with pronounced variations in attention and alertness Recurrent visual hallucinations that are typically well formed and detailed REM sleep behaviour disorder, which may precede cognitive decline One or more spontaneous cardinal features of parkinsonism Bradykinesia (defined as slowness of movement and decrement in amplitude or speed) Rest tremor Rigidity Note: the first three typically occur early and may persist throughout the course of disease | Severe sensitivity to antipsychotic agents Postural instability Repeated falls Syncope or other transient episodes of unresponsiveness Severe autonomic dysfunction (e.g., constipation, orthostatic hypotension, urinary incontinence) Hypersomnia Hyposmia Hallucinations in other modalities Systematised delusions Apathy Anxiety Depression |
Biomarkers | |
Indicative biomarkers | Supportive biomarkers |
Reduced dopamine transporter uptake in basal ganglia demonstrated by SPECT or PET Abnormal (low uptake) 123iodine-MIBG myocardial scintigraphy Polysomnographic confirmation of REM sleep without atonia | Relative preservation of medial temporal lobe structures on CT/MRI scan Generalised low uptake on SPECT/PET perfusion/metabolism scan with reduced occipital activity ± the cingulate island sign on FDG-PET imaging Prominent posterior slow-wave activity on EEG with periodic fluctuations in the pre-alpha/theta range |
Diagnosis of DLB | |
Probable DLB can be diagnosed if (a) Two or more core clinical features of DLB are present, with or without the presence of indicative biomarkers, or (b) Only one core clinical feature is present, but with one or more indicative biomarkers Probable DLB should not be diagnosed on the basis of biomarkers alone Possible DLB can be diagnosed if (a) Only one core clinical feature of DLB is present, with no indicative biomarker evidence, or (b) One or more indicative biomarkers are present but there are no core clinical features DLB is less likely (a) In the presence of any other physical illness or brain disorder, including cerebrovascular disease, sufficient to account in part or in total for the clinical picture, although these do not exclude a DLB diagnosis and may serve to indicate mixed or multiple pathologies contributing to the clinical presentation, or (b) If parkinsonian features are the only core clinical feature and appear for the first time at a stage of severe dementia | |
Additional considerations | |
DLB should be diagnosed when dementia occurs before or concurrently with parkinsonism. The term PDD should be used to describe dementia that occurs in the context of well-established PD In a practice setting, the term that is most appropriate to the clinical situation should be used, and generic terms such as Lewy body disease are often helpful In research studies in which a distinction needs to be made between DLB and PDD, the existing 1-year rule between the onset of dementia and parkinsonism continues to be recommended |
Clinical Management
Symptom type | Recommendations |
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Non-pharmacological interventions | |
NA | Interventions can be patient- or caregiver-focused, or both Potential options based on preliminary evidence Exercise (both motor and cognitive benefits) Cognitive training Caregiver-oriented education and training to manage psychiatric symptoms including agitation and psychosis |
Pharmacological interventions | |
Cognitive symptoms | AChEIs Memantine |
Neuropsychiatric symptoms | AChEIs Antipsychotics Drugs targeting serotonergic system Newer drugs targeting the serotonergic system, such as pimavanserin, may be alternatives; however, controlled clinical trial data in patients with DLB are needed Although depressive symptoms are common in DLB, trial data are scant. In alignment with general advice on depression in dementia, SSRIs, SNRIs, and mirtazapine are options in DLB, with treatment guided by individual patient tolerability and response |
Motor symptoms | Levodopa Safety assessments Bone mineral density screening Assessment of vitamin D status |
Other symptoms | A wide range of other symptoms can occur in DLB, including autonomic and sleep/wakefulness disturbances, which have profound negative sequelae for QoL in both patients and their families In the absence of DLB-specific trial data for these symptoms, clinicians base their treatment decisions on clinical experience, expert opinion, or evidence-based recommendations developed in other diseases For example, cautious bedtime use of clonazepam may reduce the risk of sleep-related injuries in patients with DLB with REM sleep behaviour disorder. However, it carries a risk of worsening cognition and gait impairment; melatonin represents a possibly safer option |