About

Introduction   

Lewy body dementia is one of the more common causes of dementia. Currently it is the second most common dementia disorder following Alzheimer’s disease [2]. This condition is shown to affect more than 1.4 million people in the United States [1] [2]. Of dementia cases in older adults, Lewy body dementia is said to make up 5% of people with dementia [2]. Lewy body dementia is a disorder that progresses over time [1]. The progression of the disease differs between individuals and the severity of the symptoms [1].  

On average an individual lives between five to eight years after diagnosis [1]. Currently there is not a cure for this disease [1]. This course will examine the causes of this disease, signs and symptoms patients might experience, diagnostic tests, types of management, and educational resources for family members. This course is designed to inform nurses about this common disease and to use this information in their daily practice to care for their patients.  

Definition

Lewy body is an umbrella term that includes two separate diagnoses: Dementia with Lewy bodies and Parkinson’s disease dementia [5]. As these diseases progress, they develop together and are seen as one entity, not two separate conditions [4]. Lewy body dementia is a condition that involves neurocognitive disorders that include hallucinations, memory loss, behavior changes, and parkinsonism features [2]. This disease can also affect intellectual abilities and cause individuals to act out dreams during REM (rapid eye movement) sleep [2]. REM sleep behavior disorder sometimes may be experienced before any other symptoms are exhibited [2].   

Lewy body dementia is known for a buildup of deposits of alpha- synuclein proteins called Lewy bodies [1]. Diagnosing this condition can be difficult because many neurological disorders have similar symptoms. Lewy body dementia and Parkinson disease dementia are very similar. For a diagnosis of Lewy body dementia, there must be a cognitive impairment with motor symptoms occuring in less than 12 months [3].  Parkinson’s disease dementia affects an individual’s movements; cognitive symptoms appear later (greater than one year) [5].  

Lewy body dementia is known to affect older adults generally between the ages of 50 and 85 [2]. This disease is said to be underdiagnosed due to a large number of diagnoses occuring post-death during autopsies [4]. Several medications used to treat neurocognitive and behavioral symptoms in other conditions can worsen the symptoms of Lewy body dementia [4]. Therefore, an accurate diagnosis can impact an individual’s quality of life.  

Epidemiology 

Lewy body dementia affects a significant number of individuals in the United States. This condition is found more often in men than women [4]. Age is thought to be the greatest risk factor for an individual developing this disease [4]. An individual who has a family history of Lewy body dementia and Parkinson’s disease is at a higher risk for developing this condition [3].

Lewy Body dementia is more widespread in European, Asian, and African ethnic groups [3].  In individuals with Parkinson’s disease, the incidence of Parkinson’s disease dementia is said to be around 25-30% [4]. The incidence of individuals with Parkinson disease developing this type of dementia after having Parkinson’s for more than 20 years increases to around 83% [4].  

Pathophysiology 

There is a buildup of alpha- synuclein proteins that causes neurons to die in Lewy body dementia [2] [5].  As mentioned above in this course, this buildup of proteins is called Lewy bodies. The death of neurons that produce dopamine result in problems with movement, cognitive impairment, a decline in cognition, and sleep disturbances [4].  In Lewy body dementia there is a deficiency of acetylcholine [3]. There is also a decrease in acetylcholine with Alzheimer’s disease, but the deficiency is greater with Lewy body dementia [3]. The decrease in neurons that produce acetylcholine causes memory loss and learning impairment [4].  

The mutation of synuclein alpha and synuclein beta genes can cause dementia with Lewy bodies [2]. Mutations in apolipoprotein E and GBA genes are potential risk factors for developing the disease [2]. There have been cases where a buildup of alpha-synuclein was found during an autopsy, but the individual did not show any clinical signs of Lewy Body dementia when alive [4]. The function of these proteins in this condition is still undetermined [5]. 

Etiology 

The exact cause of Lewy body dementia is still unknown. While research is ongoing and new developments are occuring, the specific cause has not been determined. The accumulation of Lewy bodies cause cell death which causes symptoms, however, the reason for the buildup of Lewy bodies is still under research [5]. As mentioned earlier, there are specific gene mutations that have been shown to increase the likelihood of producing altered alpha- synuclein proteins, in turn causing them to clump together (forming the Lewy bodies) [2].  

The mutation of the GBA gene interferes with the function of lysosomes, which can affect the breakdown of the alpha- synuclein proteins, causing the proteins to accumulate [2]. The e4 allele type of the APOE gene has been shown to increase the risk of developing Lewy body dementia [2]. These clumps of Lewy bodies form inside and outside of neurons in different areas of the brain, where they can alter the function of the cell and can cause the cell to die [2].

The neurons that develop the neurotransmitter dopamine are especially impacted by these clumps of Lewy bodies, which was addressed earlier in this course [2]. Further research is required to find out why these Lewy bodies develop in certain individuals. Currently, age, genetics, and environmental factors are some of the greatest risk factors [3]. 

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Clinical Signs and Symptoms 

Lewy body dementia is a progressive disorder – the signs and symptoms worsen over time. The symptoms that are more common are sleep changes, impaired behavior, movement, and cognition [5]. Research shows that the location of Lewy body accumulation impacts the clinical signs and symptoms the individual experiences [3]. If Lewy bodies develop in the brainstem and cerebral cortex first, the condition is called dementia with Lewy bodies, and the onset of the dementia is early [3]. If Lewy bodies accumulate in the brain stem and then develop into the cerebral cortex as time passes, the onset of dementia appears later, and this condition is called Parkinson’s disease dementia [3].  

Rapid Eye Movement Sleep Behavior Disorder 

Rapid eye movement (REM) sleep behavior disorder is sometimes the first clinical sign of dementia with Lewy bodies [2]. Individuals with this disorder move and talk while dreaming in their sleep [2]. The movements can be violent and cause the individual to fall out of bed [5]. Individuals may kick, punch, and scream in REM sleep (the second half of their sleep) [4]. REM sleep behavior disorder is seen in 76% of patients with dementia with Lewy bodies [4].   

This disorder can cause fractures and contusions in some individuals resulting from falling out of bed [4]. This can not only affect the individual, but also the sleep partner of the patient [4]. In some cases, separate sleeping arrangements are needed for the safety of the individual and their sleeping partner. A questionnaire by the patient and sleep partner is part of the diagnosis of REM sleep behavior disorder [14]. If the individual does sleep next to someone, this questionnaire can be helpful as most of the time the patient cannot recall the events while asleep [14]. Video polysomnography is required for a complete diagnosis of this disorder [14]. These events while asleep must be repeated to meet the diagnostic criteria [14]. 

Other Sleep Disorders 

Other disorders of sleep include sleepiness in the daytime, restless leg syndrome, confusion when awakened, and obstructive sleep apnea [4]. 

Visual Hallucinations 

Visual hallucinations are present in about 80% of individuals with Lewy body dementia [1]. Visual hallucinations are a core clinical symptom of dementia with Lewy bodies [4]. They are more common in women than in men [4]. Individuals are aware of these hallucinations and can tell others what they experienced [4]. Visual hallucinations are vivid to individuals and have been said to range from people walking around the house to seeing people that have died sitting next to them [6]. During the beginning stages of the disease, the hallucinations do not seem to affect the patient as much as when the disease progresses [6]. Patients are said to be afraid of these hallucinations in the later stages of the disease [6]. Nonvisual hallucinations are less common, however can occur in some patients [1]. These hallucinations include smelling or hearing something that is not in their surroundings [1]. 

Fluctuation in Cognition 

Fluctuation in cognition is also a clinical sign that is associated with dementia with Lewy bodies [4]. This symptom includes changes in attention, concentration, and alertness [5]. These changes are random and can differ day-to-day [1]. Symptoms can include delirium, and mimic symptoms that are caused by metabolic diseases, which can further the difficulty with identifying the correct diagnosis [4]. To diagnose dementia with Lewy bodies, one of the episodes must be confirmed [4]. These fluctuations can be present in other forms of dementia in their later stages but when present in earlier stages, they point to dementia with Lewy bodies [4].  

Memory loss that impacts activities of daily living can be found in later stages of Lewy body dementia [1]. Memory loss early on is more often a characteristic sign of Alzheimer’s dementia [1]. Confusion about the individual’s whereabouts, and inability to multitask can also occur in dementia with Lewy bodies [4]. 

Problems with Movement 

Problems with movement are signs of Lewy body dementia. Bradykinesia (slow movements) and rigidity occur in about 85% of individuals with dementia with Lewy bodies [4]. Tremor at rest is less common in individuals with this condition [4]. Loss of coordination and difficultly swallowing can occur [1]. Problems with movement greatly increase the risk of falls for these individuals [4]. This can place strain on the individual’s caregivers [4]. 

Autonomic Dysfunction 

Autonomic dysfunction can be present in dementia with Lewy bodies and Parkinson’s disease dementia. This symptom is seen in about 90% of patients with Lewy body dementia [4]. The symptoms that result from autonomic dysfunction can be constipation, urinary incontinence, orthostatic hypotension, erectile dysfunction, and dizziness [1] [4]. Orthostatic hypotension appears as early as five years prior to the diagnosis of Lewy body dementia [4]. Syncope and falls are usually the result of orthostatic hypotension [4]. Constipation can also occur earlier in the disease process [4]. 

Diagnostic Tests and Evaluations  

Throughout this course, it has been mentioned that Lewy body dementia is significantly underdiagnosed. Individuals are usually diagnosed as the disease progresses due to the symptoms that overlap with other forms of dementia and other neurological and psychiatric disorders [3]. An autopsy of the brain after death is one of the only ways to have a conclusive diagnosis of Lewy body dementia [16]. There are certain diagnostic criteria and diagnostic tests that are used to diagnose an individual with Lewy body dementia. 

Diagnosis by Symptoms 

Lewy body dementia is probable when an individual experiences dementia and two main features of the disease. Lewy body dementia is a potential diagnosis if the individual experiences progressive dementia and one main feature of the disease [3]. As discussed in the clinical signs and symptoms section of this course, key features of Lewy body dementia are cognitive fluctuations, dementia that progresses, problems with movement (signs of parkinsonism), REM sleep behavior disorder, and visual hallucinations [3] [16].  

Timing of symptoms is relevant for distinguishing between the two forms of Lewy body dementia [3]. Currently healthcare providers use the time span of one year to distinguish the two forms [3]. If dementia occurs within one year of the appearance of movement problems, then a diagnosis of dementia with Lewy bodies is used [3]. If an individual is diagnosed with Parkinson’s disease and starts experiencing symptoms of dementia more than one year after their Parkinson’s diagnosis, then Parkinson’s disease dementia is used [3]. Some indicative biomarkers in addition to clinical symptoms are used in diagnosis [4]. Some of these biomarkers can be found in cerebral spinal fluid (CSF) and are still under research [4].  

Cognitive Tests 

Cognitive testing can be used to show the cognitive impairment of patients with Lewy body dementia [3]. The Mini-Mental State Examination can be used as an initial screening test [4]. This exam tests cognitive function by focusing on concentration, orientation, and memory [15]. This test can be limited since symptoms of these patients can fluctuate day to day [3]. Another cognitive function test is the Montreal Cognitive Assessment (MoCA) [15]. Providers do not usually diagnose based on a single test; instead, they use the results to look for other signs and symptoms of Lewy body dementia [4]. 

Imaging Tests 

There are certain imaging tests that can help with diagnosis and distinguishing between other dementia disorders. A single-photon emission computerized tomography (SPECT) scan can help support a diagnosis [16]. This is a nuclear scan that can sense radioactivity [16]. If the SPECT scan shows a reduced dopamine transporter uptake in the basal ganglia, this can be a sign of Lewy body dementia [16]. This will separate the diagnosis between Lewy body dementia and Alzheimer’s disease [4]. Performing this scan alone will not lead to a possible diagnosis of Lewy body dementia; however, in combination with other diagnostic tests, the scan can lead to a more certain diagnosis [4]. Results from these scans can appear normal initially, and the scan may need to be repeated [4].  

An iodine- MIBG myocardial scintigraphy can be performed to support Lewy body dementia [16]. This would show decreased communication of cardiac nerves [16]. The results may be skewed by heart disease or certain drugs [4]. A CT or MRI may be used but these imaging tests can present mixed results [4]. With Alzheimer’s disease, significant atrophy is seen in the medial temporal lobes [4]. There is normally minimal atrophy in Lewy body dementia [4].  

As mentioned earlier in the course, video polysomnography is needed for the diagnosis of REM sleep behavior disorder [14]. This sleep study without the loss of muscle tone can also point towards a diagnosis of Lewy body dementia as REM sleep behavior disorder has now moved to a key feature of this disease [14]. 

Case Studies 

Case Study #1 

A 74-year-old male presents to his primary care provider after his wife reports abnormal behavior over the past several months. His wife reports the patient kicks and screams during sleep. The patient reports seeing little people walking around the living room during the day. The wife states the patient some days will fall asleep throughout the day while completing activities.  The patient states difficulty walking and muscle stiffness.  

The wife states last week the patient was supposed to go to the local grocery store to buy milk. After two hours passed, the wife called her husband as she was worried about him. He states he got lost finding the grocery store and did not know where he was. The wife said she had to drive to find her husband and bring him home. The patient also reports dizziness when standing. After the nurse obtained an orthostatic blood pressure, the patient was positive for orthostatic hypotension. 

• Which form of dementia is the patient most likely experiencing? 
• What type of symptoms is the patient experiencing that would point to that diagnosis? 
• What diagnostic tests or evaluations should the patient undergo? 
• What types of supportive treatment should the healthcare provider include in the treatment plan for this patient? 

Case Study #2 

A 70-year-old female presents to the emergency department via EMS after falling at home. The patient’s daughter called 911 after finding her on the floor when going to visit her. Upon arrival at the emergency department the patient is oriented to self. The patient does not know where she is or what happened to precipitate the fall. The patient has a past medical history of hypertension, diabetes type II, and Parkinson’s disease.  

The patient was diagnosed with Parkinson’s disease two years prior. The daughter states the patient has been forgetful lately and not acting like herself. The daughter reports that her mom’s behavior is different from day-to-day. An MRI and the National Institutes of Health Stroke Scale (NIHSS) are used to rule out a cerebrovascular accident. A complete blood count (CBC), a complete metabolic count (CMP), and urinalysis are obtained. The patient suffered a contusion to her right cheek and a right radius fracture. The patient states that she sees figures dancing in the room and smells popcorn. The patient appears to be frightened by the hallucinations. The patient’s daughter states for the last six months the patient has had difficulty swallowing and a reduced appetite.  

• Which form of dementia is the patient most likely experiencing? 
• What would the MRI of the patient most likely show? 
• What clinical signs of dementia is the patient exhibiting? 

Management 

Currently there is not a cure for Lewy body dementia, only supportive treatment. The management of this disease involves a multifaceted approach, including therapies, pharmacological treatments, and family support. 

Therapies 

Specific therapies can help with symptom management and help improve the individual’s quality of life. Occupational therapy can help improve a patient’s ability to complete activities of daily living. Speech therapy can help with swallowing coordination and improve the clarity and volume of speech [5]. Physical therapy can aid patients with problems with movement [5]. Mental health counseling can help individuals and their families with managing behaviors and their emotions [5]. 

Medications 

Pharmacotherapy can help with supportive treatment but can also worsen symptoms if certain medications are taken. Below are some examples of medications that are used by patients with Lewy body dementia. 

• Cholinesterase Inhibitors are used to help cholinergic activity to improve cognitive function [6].  

• Rivastigmine was one of the first of these drugs to be tested [6]. Patients were noted to have improved on their cognitive exams [6]. It is also shown to reduce hallucinations and lessen anxiety [6]. This class of drugs has been said to improve the quality of life for some patients [4].   

• Donepezil and Galantamine are also used to reduce dementia symptoms of hallucinations [6]. These drugs were initially targeted for patients with Alzheimer dementia, however, they are effective for individuals with Lewy body dementia as well [3]. A study was done stating even if there is not a sign of cognitive improvement, this should not be the criteria to stop the medication as this medication has been proven to protect the individual from further impairment of cognition [4]. 

• Atypical Antipsychotics are prescribed to patients that are not seeing a reduction of symptoms while on cholinesterase inhibitors [3]. These types of drugs are seen as controversial due to the many adverse effects that have been seen in patients [4]. Drugs such as haloperidol and olanzapine should be avoided in patients with Lewy body dementia as they can cause neuroleptic malignant syndrome (a life-threatening condition) [5]. Quetiapine, clozapine, pimavanserin, and aripiprazole are atypical antipsychotic drugs that can be used to improve agitation and help prevent cognitive fluctuations [5]. 

• Carbidopa-Levodopa can be used in patients to manage problems with movement [3]. This medication can cause side effects and can result in hallucinations, delusions, and increase confusion [3]. Providers should begin with low doses of this medication [3]. 

• Clonazepam is a benzodiazepine that can lessen the REM sleep behavior disorder that patients with dementia with Lewy bodies can experience [5]. Between 33-65% of patients with REM sleep behavior disorder can experience an injury while sleeping [5]. This medication has been proven to decrease injuries that occur during sleep [5]. Clonazepam can adversely affect individuals with gait disorders or sleep apnea [5]. 

• Melatonin is a hormone that can be used for patients that are affected by REM sleep behavior disorder (5). Studies have shown that the use of melatonin lessened the frequency and the severity of symptoms associated with REM sleep behavior disorder [5]. Melatonin can have side effects such as headaches in the morning, sleepiness during the day, and hallucinations [5]. 

• Memantine is used to treat dementia symptoms [5]. This medication is an NMDA receptor antagonist that stops effects of glutamate in the brain [5]. Memantine has been shown to improve symptoms of patients early in the disease [5]. 

Nursing Care 

As mentioned before, there is not a cure for Lewy body dementia. Caring for patients with Lewy body dementia includes supportive treatment. Nurses can play a significant role in caring for these patients and providing the family with support. Home health nurses can help with frequent assessment of the patient and their environment [3]. Environmental changes may be needed to protect the patient from falls and other accidents. Home health nurses can assess the type of assistance the patient would benefit from.  

Nurses can aid the family by providing education to assist in how to care for the patient. Family members and caregivers must be aware of the changes in behavior, fluctuations in cognition, and hallucinations that the individual might experience [3]. Nurses must also provide education to the caregiver of the patient on the side effects of certain medications, as they can affect an individual with this disease [7]. 

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Family Support 

Lewy body dementia is growing in recognition; however, many people might not be aware of this condition and the disease process. Family members need support from health care professionals to better care for their loved ones. Support can come in the form of education and preparing the family for the symptoms the individual may experience. The cognitive function of patients with this disease can be very limited [3]. Family members must be educated on monitoring the individual closely to promote safety [3]. These individuals are at a high risk for falling and developing aspiration pneumonia (due to swallowing difficulties) [3]. Family members should be educated in preparing for an emergency.  

Individuals with Lewy body dementia may need care and the family needs to know how to inform health care providers of their specific needs. It is important to educate family members that their roles in their past relationship with the patient will likely change due to the disease process. To prevent caregiver burnout, family members must be aware of their limitations and know when they need help [7]. Modifying the patient’s home may be needed for patient safety [3]. Each patient may have specific needs and family members should know what modifications may be necessary [3].  

Prognosis 

The prognosis of Lewy body dementia can be viewed as poor. As mentioned briefly earlier in this course, this disease is progressive and after diagnosis, the life expectancy is five to eight years [3]. The range of expectancy has also been attributed to delay in diagnosis, which can further delay supportive treatment to improve quality of life for the individual [3]. Patients can die from complications from the disease. Complications can include cardiac complications, falls, adverse effects from medications, pneumonia, and suicide [3].  

Compared to Alzheimer’s dementia, the risk of hospitalization or death due to respiratory infections is higher in patients with Lewy body dementia [8]. The median age at death is said to be similar between patients with Alzheimer’s dementia and Lewy body dementia [8]. The life expectancy from diagnosis to death is shorter in patients with Lewy body dementia [8]. The patient’s environment has been shown to play a role in the increased risk of mortality [8]. Patients in nursing homes have been shown to have a higher risk of mortality [8]. Caregivers can decrease the risk of complications by educating themselves on this disease and keeping their loved ones safe. 

Resources for Family Support  

Lewy body dementia is a diagnosis that can affect all aspects of an individual’s life and their family members lives. As nurses we must provide support for family members so they can better care for their loved ones and improve their quality of life. As recognition of this condition grows, family support resources are increasing. The Lewy Body Dementia Association is a nonprofit organization that raises awareness and provides support for families with individuals that suffer from Lewy body dementia [10]. Support groups can be found on their website to help families across the country in their local area [10].  

The Lewy Body Dementia Association was started by caregivers of individuals with this condition. They also focus on education and research into the disease. This association is a resource for family members [10]. 

Another resource for family members is The Lewy Body Dementia Resource Center. This is a nonprofit charitable organization that gives assistance and support to those who care for someone with Lewy body dementia [9]. This organization was founded by caregivers of individuals with Lewy body dementia. They have a support phone line that is available seven days a week to answer questions [9]. They also promote research and early diagnosis of this disease [9]. 

Research Programs 

Lewy body dementia is the second most common form of dementia in the United States [4]. This illness is thought to be underdiagnosed and commonly mistaken for other neurological disorders [3]. Research on Lewy body dementia can decrease the time it takes to diagnose a patient, and can help with management of the condition.  

The National Institute of Neurological Disorders and Stroke provides support for a variety of research endeavors for Lewy body dementia [11]. In 2021 The National Institute of Health spent $93 million dollars on Lewy Body dementia research [11]. One program is the Biomarkers for Lew body dementias program. This program aims to increase clinical data collection from patients with this condition, find biomarkers to expand further research, and allow access to the science community to help with further studies [11]. Another program is the Parkinson’s Disease Biomarkers Program. This program’s purpose is to collectively research with healthcare professionals, patients and family members, and technology professionals to increase biomarker research [11]. 

Biomarker research has been increasing in Lewy body dementia. A biomarker is a feature that can specifically indicate a certain disease [12]. For quite some time there were not any identified biomarkers for Lewy body dementia. There are certain biomarkers that aid in distinguishing Alzheimer’s disease from Lewy Body dementia [13]. These biomarkers can be assessed through imaging or in cerebral spinal fluid [13]. Currently these biomarkers are only helpful if another disease is doubtful [13].  

New biomarkers are needed to separate Alzheimer’s dementia from Lewy Body dementia and other neurological disorders [13]. Biomarkers that can help with early diagnosis would be beneficial for early treatment [13]. 

Conclusion  

Lewy Body dementia affects 1.4 million Americans [2]. The disease is underdiagnosed and often diagnosed incorrectly. Incorrect diagnoses can lead to worsening of symptoms and the administration of drugs that can lead to adverse effects.  

Educating healthcare providers and the community about Lewy body dementia can improve quality of life for individuals with the disease. As nurses, we must be informed about this disease to better educate our patients and their caregivers, and to know how to advocate for our patients.  

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