Dementia with Lewy Bodies Jennifer Rose V. Molano, MD1

University of Cincinnati College of Medicine, Cincinnati, Ohio Semin Neurol 2013;33:330–335.

Abstract Keywords

► Lewy bodies ► synucleinopathy ► REM sleep behavior disorder ► dysautonomia ► parkinsonism

Address for correspondence Jennifer Rose V. Molano, MD, Department of Neurology and Rehabilitation Medicine, The University of Cincinnati College of Medicine, P.O. Box 670525, 260 Stetson St., Suite 2300, Cincinnati, OH 45267-0525 (e-mail: [email protected]).

Dementia with Lewy bodies (DLB) is a synucleinopathy that is clinically distinct from Alzheimer’s disease, associated with cognitive decline, fluctuations in alertness and cognition, visual hallucinations, and parkinsonism. Other clinical symptoms that can occur with DLB include dysautonomia and sleep disorders such as rapid-eye-movement sleep behavior disorder (RBD). The pathological criteria of DLB are associated with the location of Lewy body pathology and the extent of Alzheimer’s pathology seen. Treatment is symptomatic. The genetic basis of DLB is being explored, and future studies will investigate ways to identify those most at risk for DLB prior to the onset of cognitive symptoms.

Dementia with Lewy bodies (DLB) is associated clinically with cognitive decline, fluctuations in attention, visual hallucinations, and parkinsonism assumed to be due to abnormal processing of the protein α-synuclein.1 It is considered to be a common cause of neurodegenerative dementia, with a prevalence of 4.2 to 7.5% and incidence of 3.8% in those newly diagnosed with dementia.2 Because DLB may also be associated with increased financial and caregiver burden compared with Alzheimer’s disease (AD),3,4 awareness of the clinical features of DLB is essential in treating patients who present with cognitive changes.

Clinical Features Patients with DLB can have a plethora of symptoms that differ from patients with AD. In the approach to those with DLB, cognitive, motor, neuropsychiatric features, sleep issues, and autonomic symptoms should be addressed. ►Table 1 summarizes the clinical features of DLB, and ►Table 2 compares these features to those commonly associated with AD.

Cognitive Although AD patients classically present with short-term memory loss, those with DLB tend to have impairments in visuospatial skills, attention, and executive functioning.5–7 Fluctuations in alertness and cognition can be seen and can

Issue Theme Neurodegenerative Dementias; Guest Editor, Brandy R. Matthews, MD

be challenging to define, though caregivers may report disturbed arousal and disorganized speech.8,9 Questionnaires, such as the Mayo Fluctuations Scale,9 the Clinician Assessment of Fluctuations,10 and the One Day Fluctuation Assessment Scale,10 can be used to determine whether fluctuations have occurred.

Motor Motor features are less common in AD than DLB. Parkinsonism is often seen in patients with DLB and may be associated with higher functional disability and subsequent need for further resources.11 As a result, it is essential in the evaluation of these patients to assess for extrapyramidal signs, such as tremor, rigidity, bradykinesia, and postural instability. Compared with idiopathic Parkinson’s disease, parkinsonian features in DLB are more likely to occur bilaterally instead of unilaterally, and with a postural rather than rest tremor.

Neuropsychiatric Features Neuropsychiatric features are also more common in DLB compared with AD and can include visual hallucinations, depression, apathy, and delusions.12 The visual hallucinations in DLB tend to occur within the first 5 years of a dementia diagnosis compared with patients with AD, who tend to experience visual hallucinations later in their course.13 In DLB, visual hallucinations are typically well formed, and

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DOI http://dx.doi.org/ 10.1055/s-0033-1359315. ISSN 0271-8235.

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1 Department of Neurology and Rehabilitation Medicine, The

Dementia with Lewy Bodies

Dementia with Lewy bodies Areas of cognitive impairment

Attention-executive functioning Visuospatial skills




Visual hallucinations Fluctuations in attention Apathy Depression Anxiety


Rapid-eye-movement sleep behavior disorder


Orthostatic hypotension Constipation Urinary incontinence

associated with synucleinopathies such as DLB, Parkinson’s disease, and multiple systems atrophy, the presence of dream enactment behavior may provide an important clue to the underlying neuropathology in patients presenting for evaluation of cognitive symptoms. Obtaining polysomnography to confirm a lack of muscle atonia during REM sleep is advised to confirm RBD, as respiratory events associated with obstructive sleep apnea also may trigger dream enactment behavior. Other causes for RBD may include selective serotonin reuptake inhibitors and autoimmune disorders.16

Autonomic Symptoms Autonomic features such as orthostatic hypotension, constipation, and urinary incontinence are common in DLB.17 Autonomic symptoms in Alzheimer’s disease are more variable. Dysautonomia may be associated with sympathetic and cardiac vagal denervation, detrusor overactivity, delayed gastrointestinal motility, and abnormal sweat response with emotion.18

Diagnostic Criteria patients often state that they are not frightened to see animals and people. Although the physiological mechanism has not been well established, visual hallucinations in DLB may be associated with decreased cortical acetylcholine.1 Alterations in serotonergic transmission may also be involved.12 Extracampine hallucinations, associated with the sensation of someone being near, may also be experienced.14 Delusions may be associated with misidentifications such as Capgras syndrome or may be associated with ideas of theft or infidelity.12

Sleep Issues Sleep symptoms also tend to occur more frequently in those with DLB compared with those with AD.15 Symptoms may include insomnia, excessive daytime sleepiness, and dream enactment behavior. Dream enactment behavior is a clinical feature of rapideye-movement sleep behavior disorder (RBD). When dream enactment behavior occurs, the actions tend to mimic the content of dreams and may be associated with injuries to patients or their bed partners. Because RBD is strongly

Both clinical and pathological diagnostic criteria for DLB have been established and revised.1

Clinical The central feature of DLB is the presence of dementia. Core features include fluctuations in cognition, recurrent, wellformed visual hallucinations, and parkinsonism. Suggestive features of DLB include RBD, severe neuroleptic sensitivity, and decreased uptake of the dopamine transporter in the basal ganglia on single photon emission computed tomography (SPECT) or positron emission tomography (PET) scans. Supportive features include recurrent falls and syncope, autonomic dysfunction, depression, delusions, among others.1 Clinically probable DLB is associated with dementia with at least two core features or dementia with at least one core feature and one suggestive feature. Clinically possible DLB is associated with dementia with one or more suggestive features of DLB. Although supportive features include recurrent falls and syncope, transient loss of consciousness, and severe autonomic dysfunction, they are not specific enough to contribute to the formal diagnosis of DLB.1

Table 2 Comparison of features: dementia with Lewy bodies and Alzheimer’s disease

Area(s) of cognitive impairment


Dementia with Lewy bodies

Alzheimer’s disease

Attention-executive functioning Visuospatial skills

Prominent memory impairment



Less common

Neuropsychiatric features


Less common early in the disease

Sleep Issues


Less common




Structural neuroimaging

Dorsal mesopontine gray matter atrophy

Hippocampal atrophy

Pathological features

Lewy bodies

Amyloid plaques Neurofibrillary tangles

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Table 1 Common clinical features of dementia with Lewy bodies


Dementia with Lewy Bodies




Although DLB is a clinically distinct entity from AD, patients may have mixed neuropathology. The pathological criteria for DLB include the location of Lewy bodies (pathological aggregates of α-synuclein) and the extent of AD pathology observed to determine the likelihood of DLB.1 Areas of Lewy body pathology considered in these criteria include the brainstem, limbic system, and neocortex. Alzheimer’s pathology is categorized as low, intermediate, or high based on the presence of amyloid plaques and neurofibrillary tangles from National Institutes on Aging-Reagan criteria and the Braak staging schemes.1 High-likelihood of DLB is associated with diffuse neocortical Lewy body pathology with either low or intermediate AD pathology, or limbic Lewy body pathology with low AD pathology. Intermediate-likelihood of DLB is associated with limbic-predominant Lewy body pathology and intermediate AD pathology, or diffuse neocortical Lewy body pathology with high AD pathology. Low-likelihood of DLB is associated with brainstem-predominant Lewy body pathology and any AD pathology category, or limbic-predominant Lewy body pathology with high AD pathology.1 Validation of the pathological DLB criteria has shown that those with high-likelihood of DLB were more likely to have the core clinical features of DLB than those with low-likelihood DLB.19 In those with high-likelihood DLB, the following clinical features were reported within 3 years of dementia onset: parkinsonism in 93%, fluctuations in 83%, visual hallucinations in 62%, and RBD in 90%.19 It is also notable that limbic Lewy body pathology has been associated with visuospatial impairment, while neocortical Lewy body pathology has been associated with more rapid global cognitive decline and fluctuations in memory.20

Because DLB patients may have dream enactment behavior, polysomnography may be a clinically useful adjunctive study to confirm the diagnosis of REM sleep behavior disorder. In a study of 78 patients, polysomnographic features of DLB included increased spontaneous arousals and decreased sleep efficiency.27 These patients had mild sleep disordered breathing and no significant periodic limb movements of sleep. The majority of these patient had confirmation of REM sleep behavior disorder, with 96% of those studied having REM sleep without atonia.27

Routine Evaluation Routine evaluation in those with DLB includes neuropsychological testing, structural neuroimaging, and polysomnography. Laboratory workup is also advised to exclude other potentially reversible causes of cognitive decline such as vitamin B12 deficiency or thyroid disease.21

Neuropsychological Testing Although a screening mental status examination such as the Mini Mental State Examination, Montreal Cognitive Assessment, and the Short Test of Mental Status may be used in the clinic setting,22–24 formal neuropsychological testing may further characterize the impaired cognitive domains. As previously discussed, formal neuropsychological testing may show more prominent impairments in attention-executive and visuospatial functioning, with variable impairments in memory and language.5–7

Structural Neuroimaging With brain MRI, focal atrophy may be observed in the dorsal mesopontine gray matter compared with AD, in which hippocampal atrophy is more prominent.25,26 Seminars in Neurology

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Other Ancillary Studies Other ancillary studies may be used in the evaluation for DLB but typically are not used in routine clinical practice.

Functional Neuroimaging On fluorodeoxyglucose positron emission tomography (FDGPET), hypometabolism in the parietal and occipital cortex can be seen.28 Functional MRI studies also have shown blood oxygen-level dependent (BOLD) occipitoparietal abnormalities in the brain.29 Studies using single photon emission computed tomography have shown decreased or absent putaminal dopamine transporter uptake in DLB patients (with unilateral or bilateral findings), compared with those with dementias due to other causes, such as Alzheimer’s disease and vascular dementia30 Pittsburgh compound B (PiB) is a positron emission tomography ligand that binds to aggregated amyloid-β and is considered to be a possible biomarker in AD.31 However, because some studies have shown PiB binding may also be found in DLB patients, multimodal neuroimaging including structural MRI, PET, and PiB studies, concurrently may be useful in determining which patients are more likely to have DLB versus AD.32

Autonomic Testing Autonomic studies may be useful in distinguishing between DLB and AD.18 In those with DLB, there may be abnormal cardiac 123I-meta-iodobenzylguanidine (MIBG) uptake, cardiovagal dysfunction, and abnormal skin reflexes on sudomotor testing. Pupillary sympathetic and cholinergic denervation may also be seen.18

Cerebrospinal Fluid Studies Cerebrospinal fluid studies are not routinely ordered in the evaluation of DLB, but the presence of α-synuclein may be useful in distinguishing a patient with a synucleinopathy from a patient with AD.33

Genetics Recent research suggests that genetics may be factor in the development of DLB, especially because there is overlap in disorders with known genetic mutations, such as AD and Parkinson’s disease with dementia.34,35 Although the biological process is unclear, mutations in the amyloid precursor protein

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Dementia with Lewy Bodies

Treatment and Management There are currently no FDA-approved treatments for DLB; treatment and management are primarily symptomatic (►Table 3).

Cognitive Cognitive features may benefit from acetylcholinesterase inhibitors and memantine,1,39,40 though the effect of these medications in DLB patients needs further study. Although there are no FDA-approved treatments for DLB, rivastigmine has been approved for the treatment of dementia associated with idiopathic Parkinson’s disease.41

Motor For parkinsonism, carbidopa-levodopa can be considered.1 The medication may improve motor function, but is typically not as effective in DLB as in Parkinson’s disease or Parkinson’s disease with dementia.42 Worsening of neuropsychiatric symptoms with the use of dopaminergic agents is often a significant clinical concern.

Neuropsychiatric Symptoms Given neuroleptic sensitivity that can occur in DLB patients, conventional antipsychotics should be avoided.1 In those with visual hallucinations, acetylcholinesterase inhibitors or atypi-

Table 3 Potential symptomatic pharmacological treatments for dementia with Lewy bodies Cognitive impairment

Cholinesterase inhibitors




Serotonin-reuptake inhibitors Stimulants Atypical antipsychotics Note: Typical antipsychotics are contraindicated in DLB.


Melatonin or clonazepam for rapid-eye-movement sleep behavior disorder


cal antipsychotics can be considered.1 If an atypical antipsychotic is prescribed, then patients and caregivers should be aware of the risks of increased cardiac morbidity and mortality with this class of medications.43 Passive observation of visual hallucinations and delusions can be considered if these symptoms are not causing significant disturbance to the patient or the household. Although cholinesterase inhibitors and stimulants may improve apathy, and selective serotonin reuptake inhibitors may be effective in treating anxiety and depression, more research needs to be performed on the safety of these medications in DLB patients.12

Sleep Sleep disruption may lead to diminished daytime function, such that educating patients and caregivers on proper sleep hygiene is essential in optimizing the sleep–wake cycle. Maintaining a daily routine, exposure to natural light during the daytime hours, eliminating daytime naps, and cessation of afternoon caffeine consumption are important recommendations.44 If RBD is present, melatonin or clonazepam can be used to treat dream enactment behavior.45 Low-dose quetiapine may also be considered.1 Nonpharmacological treatments of RBD are associated with increasing safety for both the patient and caregiver, with strategies including the placement of a mattress on the floor, padding the corners of furniture, and removing potentially dangerous objects from the bedroom. In some cases, the bed partner also may need to sleep in a separate room.45

Autonomic For those with dysautonomia, compression stockings and salt tablets can be used for orthostatic hypotension. Stool softeners can be considered for constipation, and adult diapers can be used for urinary incontinence.18

Other Considerations In addition to symptomatic treatment, it is essential to discuss the importance of supervision of financial matters and medications. Given that those with cognitive impairment and parkinsonism are at higher risk for falls, providing strategies on fall prevention and home safety, such as removing obstacles from the floor and using an assistive device if necessary, is also advised.46 The cognitive impairment and parkinsonism associated with DLB, place patients at higher risk for driving impairment.47 A formal on-road driving evaluation should be considered in patients with DLB who wish to continue driving.

Future Directions: Toward an Earlier Diagnosis of DLB Recent changes in the AD diagnostic criteria incorporate biomarkers, in an attempt to determine a profile that will identify those at risk for developing the disease prior to the onset of dementia.48 A similar approach to identifying people earlier in the disease process is emerging for DLB. For example, RBD is increasingly identified as a critical clinical biomarker for DLB and other synucleinopathies.49,50Recent Seminars in Neurology

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and both presenilin genes have been associated with Lewy body pathology.34,35 Overexpression of the apolipoprotein E4 allele, which is considered to be a genetic risk factor in Alzheimer’s disease, may also be seen in those with the DLB phenotype.36 Mutations in the α-synuclein gene are associated with Parkinson’s disease, Parkinson’s disease with dementia, and DLB.34 There also is emerging evidence that mutations in β-synuclein have been associated with the DLB phenotype, potentially due to subsequent dysfunction in the autophagylysosomal pathway.34 Studies have also shown that gene mutations in glucocerebrosidase, an important enzyme associated with lysosomal storage, may be a factor in the development of neocortical Lewy bodies and DLB.37 With regard to the expression of clinical symptoms in DLB, polymorphisms in the serotonin transporter 5HTTLPR gene may be associated with delusions and hallucinations.38


Dementia with Lewy Bodies


research has shown that RBD may occur decades prior to the onset of cognitive and motor symptoms in those with DLB and other synucleinopathies such as Parkinson’s disease or multiple system atrophy.51 Neuropsychological profiles in those with RBD compared with those without RBD show a similar profile to those with DLB, with changes in visuospatial skills and attention-executive functioning.52 Patients with RBD and limbic or neocortical Lewy body disease on autopsy may present with only mild cognitive impairment, primarily affecting attention-executive function and visuospatial skills.53 In those with normal cognition, the presence of dream enactment behavior can be associated with the subsequent development of MCI.54 Rapid-eye-movement sleep behavior disorder may also be associated with autonomic dysfunction, such as orthostatic hypotension, erectile dysfunction, and constipation on an average of 5 years prior to the onset of cognitive or motor symptoms in those with DLB and Parkinson’s disease with dementia.55 When compared with those without RBD, functional neuroimaging findings in those with RBD have shown changes similar in areas affected DLB and PD.56 However, because RBD can be associated with other neuropathological causes, such as autoimmune disease, progressive supranuclear palsy, and rarely even in AD,49,57,58 more research needs to be performed to determine which patients with RBD will most likely develop DLB.

2 Vann Jones SA, O’Brien JT. The prevalence and incidence of










Conclusions Dementia with Lewy bodies is an important neurodegenerative dementia to accurately identify. Those with DLB have clinical features distinct from AD, including impairments in attention, executive functioning, and visuospatial skills, as well as neuropsychiatric symptoms, dysautonomia, and sleep disturbances. Both clinical and pathological criteria for DLB have been validated, with those with high-likelihood of DLB pathology manifesting more of the core clinical features of fluctuations in attention, visual hallucinations, and parkinsonism, as well as less AD neuropathology. Formal neuropsychological testing, neuroimaging, and laboratory workup is recommended as a part of the initial evaluation, though the presence of dream enactment behavior and RBD on polysomnogram may provide an important clue to underlying DLB pathology. Because RBD is emerging as a preclinical risk factor for DLB and other synucleinopathies, further research is needed to determine its predictive value in the context of other clinical and historical features on an individual patient basis. Although treatment is currently symptomatic and often very effective in the initial stages of the illness, the ultimate goal is the development of diseasemodifying therapies.



14 15



18 19


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Dementia with Lewy bodies.

Dementia with Lewy bodies (DLB) is a synucleinopathy that is clinically distinct from Alzheimer's disease, associated with cognitive decline, fluctuat...
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