Dementia with Lewy bodies

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Treatment is symptomatic. The main goal of treatment is to improve or stabilize cognition, behavior, and activities of daily living, and to maintain safety. Evidence on treatments for DLB has increased in recent years, and a number of reviews and meta-analyses are available.[32][38][39] Data from related conditions such as Alzheimer disease (AD) and Parkinson disease can also inform treatment of DLB.[32]

Patients with DLB have a deficiency of both acetylcholine and dopamine, so treatments should be initiated and titrated in small increments and low doses to minimize adverse effects. The use of antipsychotic medication should be avoided as far as possible due to the increased sensitivity of patients with DLB to the adverse effects of these medications, including increased mortality.[32]

A prioritized problem-oriented approach should be adopted. A list should be made of all the symptoms that the patient, family members, and other caregivers wish to be treated (which may not be the same). Consensus should be reached about which symptoms to address and in what order, bearing in mind that improvement in one symptom may be accompanied by deterioration in another.[32] Cognitive, behavioral, psychiatric, sleep, motor, and autonomic issues should be considered. Limited response to some treatments or sensitivity to adverse effects should be identified and monitored.

Supportive care

Providing support and information is an essential part of care for patients with dementia.

Following diagnosis, education, support, and resources should be provided to the patient, their family, and other caregivers, focusing on the key behavioral and psychological symptoms of DLB and how these translate to care needs.[40] Planning should focus on meeting current needs and anticipating future problems. Family and caregivers should be empowered to assist the patient in making decisions regarding health and property, managing finances, taking medications, cooking meals, etc. Discussion of advanced directives and end-of-life care that may be anticipated requires sensitivity, based on a good patient-provider and family-provider relationship.[40][41][42] Legal counsel may be needed in cases requiring guardianship and the handling of finances, especially because DLB affects executive functions that are key to making responsible financial decisions. A social worker, psychologist, or other mental health professional should be made available to provide emotional support and psychosocial input.

Discussion of driving is an emotional topic, frequently bound up with a patient’s desire for maintaining autonomy. Driving privileges, especially in patients with visual hallucinations, should be recommended to be restricted. American Academy of Neurology guidelines suggest that patients with dementia are not accurate at judging their own abilities, and that on-road driving tests are to be utilized to best assess abilities.[37]

Environmental review

Special attention should be directed to maintaining patient safety at home, particularly for those living alone or whose family can provide limited support. A home safety evaluation should be undertaken by an occupational therapist, as well as an assessment of transport, driving, and self-care needs. Falls, in particular, may be limiting and changes may be required to assure home safety. Physical and occupational therapy consultations may provide useful strategies to prevent injuries. A safe sleeping environment should be ensured for patients with rapid eye movement (REM) sleep behavior disorder.[43]

Adherence to medications should be monitored at home, and medication lists reviewed at every visit.

Support for family and caregivers

Guiding and supporting caregivers is an integral part of the care plan for any patient with dementia. There is evidence that caregivers find the depression, apathy, cognitive fluctuation, and especially the psychotic symptoms associated with DLB particularly difficult to cope with.[44] Caregivers should be advised about coping techniques, and about local and national support organizations such as the Lewy Body Dementia Association. Lewy Body Dementia Association Opens in new window The Savvy Caregiver Program is a validated psychoeducational program for caregivers, which has been adapted for racially and ethnically diverse communities as an online “tele-savvy” program.[45][46][47]

In-home assistance, respite services, and residential care

Many patients require professional help in the home to provide respite to the family, as well as supervision and assistance to the patient. Daycare services can offer respite to caregivers and patients, and may be used in combination with in-home care.

In many cases, continued home care is no longer possible due to the nature of the care situation (e.g., a spouse who cannot retire) or to problem behaviors. Patients who require residential care should be cared for in a specialist dementia unit.

Nonpharmacologic and behavioral interventions

Evidence for the effectiveness of nonpharmacologic and behavioral interventions for patients with DLB is limited due to a lack of suitable trials. However, such interventions have been shown to be of value in other types of dementia (e.g., AD) and for psychosis.[48]

Interventions with some evidence of effectiveness for dementia include the following:

Psychological interventions (e.g., cognitive behavioral therapy and cognitive training) may improve cognition and behavioral disturbance, and reduce symptoms of anxiety and depression in people with dementia.[49][50][51] [ ]

Exercise may improve cognitive decline, activities of daily living, and motor disturbances.[48]

Multifactorial interventions, including physical activity, occupational therapy, and music therapy, may be effective for psychosis in dementia.[48]

Music-based interventions were reported to moderately improve symptoms of depression, and possibly behavior, emotional wellbeing, and anxiety, in patients with dementia, but with little or no effect on cognition, agitation, or aggression.[51][52]

Massage therapy, animal-assisted interventions, and personally tailored interventions may reduce agitation in dementia.[53]

Cholinesterase inhibitors and memantine for cognitive and behavioral symptoms

Similar to AD, first-line treatment for cognitive impairment and behavioral symptoms in DLB is a cholinesterase inhibitor.[1][54][55] [ ] The three commonly used cholinesterase inhibitors are donepezil, rivastigmine, and galantamine.

Donepezil and rivastigmine have been shown to improve both cognition and behavioral symptoms, without significant exacerbation of motor symptoms in most cases; evidence for the efficacy of galantamine is limited.[32][38][39] However, nausea, vomiting, hypersalivation, vivid dreams, sleepiness, orthostatic hypotension, and syncope may occur.[56] Adverse effects of chronic cholinesterase inhibitor use include weight loss, runny nose, and muscle cramps.

The N-methyl-D-aspartate (NMDA) receptor antagonist memantine is well tolerated, although evidence for effectiveness in DLB (either as monotherapy or combined with a cholinesterase inhibitor) is mixed and limited.[32][38][54][57][58]

Symptoms of psychosis

Nonpharmacologic approaches are first-line treatment for psychosis in dementia. These include providing a comfortable environment with adequate lighting, correcting vision, and decreasing visual triggers. Increasing social engagement and ongoing activities may help mask psychotic symptoms.

It is important to identify and adequately manage potential precipitating factors for psychosis; for example, underlying infections, sleep deprivation, dehydration, and severe pain.

Antipsychotic drugs

The use of antipsychotic drugs for the acute management of behavioral disturbance in patients with DLB should be avoided as far as possible because of the risk of increased adverse effects and mortality.[1][32] Possible complications include increased rigidity, immobility, confusion, sedation, postural falls, weight gain, diabetes, and increased mortality risk.[56] Severe antipsychotic sensitivity reactions have been reported in up to 50% of patients with DLB.[1][32]

The American Psychiatric Association recommends reserving antipsychotics for symptoms that are considered severe, dangerous, and/or cause significant distress, and assessing efficacy and adverse effects to continuously balance the risk-benefit ratio in each individual patient.[59] The use of antipsychotics in patients with dementia is not a registered indication of the Food and Drug Administration or similar regulatory agencies.

Low-dose atypical antipsychotics may be considered in acute situations or for severe symptoms that do not respond to other treatments (nonpharmacologic treatments and cholinesterase inhibitors), where a balanced decision is made that benefits outweigh risks.[1][32] Typical antipsychotics should not be used because of their tendency to worsen parkinsonism. The patient and family should be involved in decision making before starting an antipsychotic. Specialist advice should be sought about drug choice and dosage, and drugs titrated in very small increments. Screening for cardiac disease and a baseline ECG are recommended.

There is no evidence supporting the use of particular atypical antipsychotics in patients with DLB.[1][32][60] Quetiapine is well tolerated and does not worsen motor function, but evidence for efficacy is lacking. Clozapine has been shown to be effective for treating psychosis associated with Parkinson disease, but efficacy and tolerability in DLB are not established. Risperidone may improve agitation in dementia but is associated with extrapyramidal symptoms. Pimavanserin (an antipsychotic medication with a specific inverse agonism and antagonism for the 5-HT2A receptor) has shown antipsychotic effects in patients with psychosis associated with Parkinson disease or dementia.[1][32][61]

Severe psychosis or affective disorders may require inpatient hospitalization, but most psychiatric comorbidities can be handled on an outpatient basis.

REM sleep behavior disorder (RBD)

RBD often accompanies DLB.

Clonazepam is an established treatment for RBD, and should be given in low doses.[1][22][23][32][43] About 90% of patients respond to treatment when given 30 minutes before bedtime; no tolerance has been noted.[62] Abrupt withdrawal must be avoided. Immediate-release melatonin has also been advocated as a first-line treatment for RBD, although evidence for efficacy is mixed. Melatonin may be safer than clonazepam for older patients, as clonazepam may be slowly metabolized, as well as increasing the risk of worsening cognition, gait impairment, and falls.[1][22][23][43]

Comorbid depression and/or anxiety

Depression and anxiety are common in patients with DLB. Nonpharmacologic interventions should be considered.[49][63]

Although there are no controlled studies specific to the drug treatment of depression in patients with DLB, options include selective serotonin-reuptake inhibitors (SSRIs) such as sertraline or citalopram, serotonin-norepinephrine reuptake inhibitors such as venlafaxine, and mirtazapine because of their limited adverse-effect profile and favorable pharmacokinetics. Treatment should be guided by individual patient tolerability and response.[1] [ ] Antidepressants with anticholinergic activity (e.g., tricyclics) should be avoided. Long-acting fluoxetine (an SSRI) may be appropriate in some cases where daily dosing is impractical. Patients with advanced dementia may need liquid preparations.

Anxiolytics (e.g., buspirone) may be useful. Cholinesterase inhibitors or memantine may have some efficacy in this regard.[64][65] Benzodiazepines may cause daytime sedation, and paroxysmal reactions may occur when new medications are started. Alprazolam may be used for anxiety control but can cause sedation, and withdrawal seizures have been reported.[66]

Acute behavioral disturbance and severe anxiety with or without agitation

For patients with acutely disturbed behavior, management is guided by whether behavioral changes are primary (due to the underlying dementia), secondary (e.g., due to comorbid medical illness, polypharmacy, dehydration, pain, lack of sleep), or mixed (primary exacerbated by secondary changes or vice versa). Addressing secondary causes usually alleviates the associated behavioral disturbances.

For severe primary behavioral changes, atypical antipsychotics may be used with caution for psychosis or mania-like symptoms with severe aggression, if a decision is made that benefits outweigh the risks.[1][32] A mood stabilizer such as valproate may also be considered alongside psychotropic medications to help alleviate aggression.[67]

Patients with significant motor symptoms

Because of potential adverse effects, motor symptoms should be treated with medication only if they are severe and interfere with activities of daily living. If needed, dopaminergic agents, typically levodopa, should be given in a small initial dose and titrated slowly.[1][32] Few controlled trials are available, and caution should be exercised due to possible worsening of cognition, hallucinations, and behavior. A significant motor response is seen in approximately one third of patients, with younger patients responding better. A limited duration of response is often seen.[68][69]

Palliative and end-of-life care

Late-/end-stage care includes palliative measures, end-of-life choices, and discussing goals of care with the family.[40][41][42]

The preferences of the patient and family regarding end-of-life interventions - including treatment, resuscitation, and prolonging life when treatable conditions arise - should be discussed early in the course of the illness.

End-of-life care is generally focused on providing comfort and basic needs (e.g., help with feeding and cleanliness, adequate pain control, good skin care, and prevention of injury through falls or misadventure). Overly aggressive care, such as enteral tube feeding, can worsen morbidity with no evidence of improvement in quality of life, survival, or caregiver outcomes.[70]

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