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'''Original Editor '''- [[User:Henry Tan|Henry Tan]], [[User:Kevin Lam|Kevin Lam]], [[User:Alex Mong|Alex Mong]], [[User:Andrew To|Andrew To]]  
'''Original Editor '''- [[User:Henry Tan|Henry Tan]], [[User:Kevin Lam|Kevin Lam]], [[User:Alex Mong|Alex Mong]], [[User:Andrew To|Andrew To]]  as part of the [[Queen's University Neuromotor Function Project]]


'''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}} &nbsp;  
'''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}} &nbsp;  
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== Introduction  ==
Lewy body disease or Lewy body [[dementia]] (LBD), is a [[Neurodegenerative Disease|neurodegenerative disease]] (specifically a [[synucleinopathy]]) related to [[Parkinson's|Parkinson disease]].<ref name=":0">Radiopedia [https://radiopaedia.org/articles/dementia-with-lewy-bodies Dementia with Lewy bodies] Available:https://radiopaedia.org/articles/dementia-with-lewy-bodies (accessed 13.9.2022)</ref>[[File:Dementia 3.jpg|thumb|Dementia]]
There are 2 types of LBD generally described in the literature:<ref name="Mckeith">McKeith IG, Galasko D, Kosaka K, Perry EK, Dickson DW, Hansen LA, Salmon DP, Lowe J, Mirra SS, Byrne EJ, Lennox G. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB) Report of the consortium on DLB international workshop. Neurology. 1996 Nov 1;47(5):1113-24. [https://www.ncbi.nlm.nih.gov/pubmed/8909416 PMID:8909416]</ref>


# Dementia with Lewy Bodies: dementia occurring first or within one year of movement disorder.
# Parkinson Disease Dementia: dementia occurring in a patient who receives a diagnosed of Parkinson's disease and then develops dementia symptoms after one year or more of the diagnosis.


== Definition ==
People with either type generally develop similar symptoms as the disease progresses. Distinct Parkinsonian symptoms such as slowness of movement, rigidity, REM [[Sleep Deprivation and Sleep Disorders|sleep]] behaviour disorder and visual hallucinations can help to distinguish DLB from Alzheimer’s disease.<ref name="National">National Institute on Aging. Lewy Body Dementia: Information for Patients, Families, and Professionals. (Accessed 4 May 2017). https://www.nia.nih.gov/alzheimers/publication/lewy-body-dementia/basics-lewy-body-dementia</ref>
== Epidemiology ==


Lewy body disease or Lewy body dementia (LBD) has been found to be strongly linked to a protein called alpha-synuclein. Abnormal accumulation of this protein in certain regions of the brain cause dramatic cognitive and motor deficits affecting behavior, mood, movement, and thinking. LBD is one of the most common causes of dementia along with [[Alzheimer's Disease|Alzheimers disease]]<ref name="National">National Institute on Aging. Lewy Body Dementia: Information for Patients, Families, and Professionals. (Accessed 4 May 2017). https://www.nia.nih.gov/alzheimers/publication/lewy-body-dementia/basics-lewy-body-dementia</ref>.
* Occurs in older patients (onset typically in 50-70 years of age), and is sporadic. However a family history of LBD and Parkinson disease will increase a person's risk.<ref name=":2">Haider A, Spurling BC, Sánchez-Manso JC. [https://www.ncbi.nlm.nih.gov/books/NBK482441/ Lewy body dementi]a. InStatPearls [Internet] 2022 Oct 23. StatPearls Publishing.Available:https://www.ncbi.nlm.nih.gov/books/NBK482441/ (accessed 6.10.2023)</ref>
* LBD is one of the most common causes of dementia (accounting for up to  20% to 30% of cases<ref name=":2" />) along with [[Alzheimer's Disease|Alzheimers disease (AD)]]<ref name="National" />.  


There are 2 types of LBD generally described in the literature:<ref name="Mckeith">McKeith IG, Galasko D, Kosaka K, Perry EK, Dickson DW, Hansen LA, Salmon DP, Lowe J, Mirra SS, Byrne EJ, Lennox G. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB) Report of the consortium on DLB international workshop. Neurology. 1996 Nov 1;47(5):1113-24. [https://www.ncbi.nlm.nih.gov/pubmed/8909416 PMID:8909416]</ref>
== Etiology ==
# Dementia with Lewy bodies (DLB) – Cognitive deficits appear within a year of onset of parkinsonism related deficits.  
The etiology of LBD is unknown. [[Genetics and Health|Genetics]], environmental factors, and changes linked to [[Older People Introduction|aging]], may have a role and research is still ongoing.<ref name=":1">Haider A, Spurling BC, Sánchez-Manso JC. Lewy body dementia. InStatPearls [Internet] 2021 Jul 12. StatPearls Publishing.Available:https://www.ncbi.nlm.nih.gov/books/NBK482441/ (accessed 13.9.2022)</ref>
# Parkinson’s with dementia (PDD) – Cognitive deficits appear beyond one year of parkinsonism.
People with either type generally develop similar symptoms as the disease progresses. Distinct Parkinsonian symptoms such as slowness of movement, rigidity, REM sleep behaviour disorder and visual hallucinations can help to distinguish DLB from Alzheimer’s disease.<ref name="National" /><br>  


{{#ev:youtube|U6kz-mcNNzo|300}}
LBD has been found to be strongly linked to a [[Proteins|protein]] called [[Alpha-Synuclein (α-syn)|alpha-synuclein]]. The abnormal accumulation of this [[Proteins|protein]] in certain regions of the [[Brain Anatomy|brain]] causes dramatic [[Cognitive Impairments|cognitive]] and motor deficits affecting behaviour, mood, movement, and thinking.  
== Epidemiology  ==
 
LBD is becoming increasingly common, affecting more than 1 million people in the US. Typically thought to be uncommon, it is now believed to be the second most common cause of dementia.<ref name="Mckeith" />&nbsp;Age seems to be the greatest risk factor, typically developing in individuals who are over the age of 50. The disease state on average lasts 5 to 7 years from onset to death with men being affected more than women.<ref name="National" />  A retrospective cohort study found cardiovascular diseases (CVD) as a significant cause of death in older people who have dementia with a relatively shorter survival approximately 4 years after the diagnosis of dementia<ref>Naharci MI, Buyukturan O, Cintosun U, Doruk H, Tasci I. [https://www.ncbi.nlm.nih.gov/pubmed/31114103 Functional status of older adults with dementia at the end of life: Is there still anything to do?.] Indian journal of palliative care. 2019 Apr;25(2):197.</ref>.
 
Other potential risk factors include:<ref name="National" />
* Parkinson’s
* REM sleep disorder
* Genetics (although only a small genetic link has been found)
* No association between lifestyle and LBD


== Clinical Presentation  ==
== Clinical Presentation  ==
[[File:Frontal lobe.jpeg|right|frameless]]
The clinical features of LBD are the consequence of the blockage of information transfer from the striatum to the [[Cerebral Cortex|cortex]], more notably the [[Frontal Lobe|frontal lobe]].


The clinical features of LBD are the consequence of the blockage of information transfer from the striatum to the cortex, more notably the frontal lobe.
Core features:<ref name="Gn">Gnanalingham KK, Byrne EJ, Thornton A, Sambrook MA, Bannister P. Motor and cognitive function in Lewy body dementia: comparison with Alzheimer's and Parkinson'ss. Journal of Neurology, Neurosurgery &amp; Psychiatry. 1997 Mar 1;62(3):243-52. http://jnnp.bmj.com/content/jnnp/62/3/243.full.pdf</ref>
 
Signs and symptoms include:<ref name="Gn">Gnanalingham KK, Byrne EJ, Thornton A, Sambrook MA, Bannister P. Motor and cognitive function in Lewy body dementia: comparison with Alzheimer's and Parkinson'ss. Journal of Neurology, Neurosurgery &amp; Psychiatry. 1997 Mar 1;62(3):243-52. http://jnnp.bmj.com/content/jnnp/62/3/243.full.pdf</ref>  


*Recurrent visual hallucinations
# Fluctuating cognitive impairment especially in executive function, attention and alertness
*Visuospatial impairment (Stroop test, Clockface test)
# Visuospatial impairment, including visual hallucinations (detailed and vivid)
*Executive function deficits (Nelson Card sort test)  
# Concurrent parkinsonian symptoms may be present but are less common, more frequently occurring years after the onset of dementia eg early [[Extrapyramidal and Pyramidal Tracts|extrapyramidal]] features (dystonia, akathisia, muscle rigidity, [[bradykinesia]], tremor, tardive dyskinesia
*Parkinsonian motor features (mild gait impairments, resting tremor)
Watch this 3 minute video showing a personal story "It happened little by little. First he would forget things, then he'd lose track of what he was doing. LBD took over the life of the man you're about to meet."<br>
*Early extrapyramidal features (dystonia, akathisia, muscle rigidity, bradykinesia, tremor, tardive dyskinesia  
*Fluctuating cognitive impairment (periods of coherence and alertness alternating with sequences of confusion and unresponsiveness)
*Attentional deficits
*Poor postural stability
*Neuroleptic sensitivity
*Orthostatic hypotension<br>


{{#ev:youtube|RSRbR1R4mz0|300}}  
{{#ev:youtube|RSRbR1R4mz0|300}}  


== Pathophysiology  ==
== Pathophysiology  ==  


The distinguishing feature of LDB is the aggregation of Lewy bodies inside neurons of the cerebral cortex. Aarsland et al, found that an increase in Lewy bodies in the temporal lobe is associated with early occurrences of visual hallucinations - a hallmark of DLB.<ref name="Dick" /> Additionally, increased Lewy body densities in the limbic and frontal lobes also correlate strongly with the severity of dementia. Although much of DLB remains unsolved, recent research has shed light on potential density-location relationships that have assisted clinicians in diagnosing and managing DLB.<ref name="Dick" />
The characteristic feature of dementia with Lewy bodies is the accumulation of Lewy bodies throughout the brain. These intracellular inclusions result from the aggregation of misfolded α-synuclein. Neurofibrillary tangles are also present, however they lack an amyloid core, as seen in AD<ref name=":0" />.[[Image:Lewybody.png|alt=|center|thumb|620x620px|Regions of the [[Brain Anatomy|brain]] affected by LBD include: cerebral cortex, [[Limbic System|limbic]] cortex, [[hippocampus]], [[midbrain]], [[brainstem]].]]


The alpha-synuclein protein has been proven to be important in many functions in the brain, particularly at synapses. Alpha-synuclein aggregate into clumps called Lewy Bodies within neurons. These Lewy Bodies alter chemicals in the brain and can damage neurons which can eventually lead to the death of those neurons.<ref name="National" />


Postmortem studies have shown that the development of Lewy Bodies can occur in the substantia nigra, locus coeruleus, dorsal raphe, substantia innominate and dorsal motor nucleus of cranial nerve X.<ref name="Crystal">Crystal, H. A. Dementia With Lewy Bodies. (Accessed 4 May 2017). http://emedicine.medscape.com/article/1135041-overview?pa=cDgRhU%2BPIzR23Dql%2BM7%2B565jaAQt6LN6YC%2BY9393F2Za2VPhjJr4I4RTzfItGaGxWFsk2h6wPa3yDctZJ2JfeiwhCTQq25Ki1mL6i64Z7Vg%3D#showall</ref>[[Image:Lewybody.png|right]]Regions of the brain affected by LBD include: 
* Cerebral cortex
* Limbic cortex
* Hippocampus
* Midbrain
* Brain stem
Source (http://labiotech.eu/major-cns-disease-milestones-in-biotech-2015/)  
Source (http://labiotech.eu/major-cns-disease-milestones-in-biotech-2015/)  


== Diagnostic Procedures  ==
== Diagnostic Procedures  ==


It is difficult to diagnose LBD and due to similarities with Alzheimer's and Parkinson’s disease symptoms, patients are often misdiagnosed.<ref name="Lewy">Lewy Body Dementia Association. What is LBD? Available from: https://www.lbda.org/category/3437/what-is-lbd.htm [Accessed May 5, 2017]</ref> Best practice in the diagnosis of LBD should include a thorough subjective examination, physical examination and neurological evaluation.
It is important to realise that there is a significant overlap between many neurodegenerative diseases, and that a clear-cut distinction between entities is not always possible. No precise test can accurately diagnose LBD. A thorough assessment is useful to reach an alternative working diagnosis (or rules out similar conditions):


Core features should be identified including fluctuations in cognition, visual hallucinations and any motor parkinsonism. If at least two of these symptoms are present, there is a greater likelihood an individual has DLB&nbsp;<ref name="Mckeith" />
* Detailed history and examination
* Assessment of [[Cognitive Impairments|cognitive]] function
* [[Blood Tests|Blood tests]] (e.g., [[Vitamin B12 Deficiency|vitamin B12]] levels, chemistry panel, [[Thyroid Gland|thyroid]] profile, [[syphilis]], [[Human Immunodeficiency Virus (HIV)|HIV]]) to rule out other causes of dementia


Motor Parkinsonism features include rigidity, bradykinesia, masked face, stopped posture and shuffling gait<ref name="Mckeith" />.&nbsp;With changes in cognition, a patient can experience variations in attention and alertness. They can also experience confusion from time to time and have fluctuations in mental abilities and behaviour&nbsp;<ref name="Mckeith" />. If a patient experiences motor parkinsonism more than a year after a diagnosis of DLB, it is more appropriate to diagnose the patient with Parkinson's with dementia instead <ref name="Mckeith" />
* [[Medical Imaging|Imaging]] studies (e.g., CT scan, MRI scan, SPECT scan, PET scan)
* [[CSF Cerebrospinal Fluid|Cerebrospinal fluid]] examinations have no significant role when conducted in these patients
* [[Sleep Deprivation and Sleep Disorders|Sleep evaluation]] for REM sleep behavior disorder<ref name=":1" />


Ultimately, the most definitive evidence of LBD is obtained via post mortem autopsies.<ref name="Lewy" />. Cortical and subcortical atrophy can be observed as well as atrophy of the grey matter of the temporal, frontal, and parietal lobes and the insular cortex&nbsp;with diagnostic imaging of patients with LBD<ref name="Morra">Morra LF, Donovick PJ. Clinical presentation and differential diagnosis of dementia with Lewy bodies: a review. International journal of geriatric psychiatry. 2014 Jun 1;29(6):569-76. [http://onlinelibrary.wiley.com/doi/10.1002/gps.4039/abstract DOI: 10.1002/gps.4039]</ref>. There can also be volume loss in the hippocampus, amygdala and the basal ganglia, specifically the substantia nigra&nbsp;<ref name="Morra" />. There can be atrophy of the nigrostriatal system and compromised dopamine transporters. This is more commonly seen in PDD&nbsp;<ref name="Morra" />.
== Management    ==
== Outcome Measures  ==


The Unified Parkinson’s Disease Rating Scale (UPDRS) was used in a study in the assessment of parkinsonism in patients with Lewy Body Disease.&nbsp;The study suggests that use of the 5-item sub-scale of the UPDRS “provides a reliable and generally applicable instrument for the assessment of parkinsonism in patients with Lewy Body Disease”.<ref name="Boot" />
There is no effective treatment for LBD and the condition is progressive. Pharmacological Management includes cholinesterase inhibitors. They treat the cognitive symptoms of LBD and are central to treatment. eg rivastigmine, galantamine, and donepezil. Acetylcholine is an important neurotransmitter for memory. People with eg LBD have reduced levels present in their brains. Cholinesterase inhibitors inhibit this enzyme , improving brain network communication.<ref name=":2" />.


== Management    ==
The other pharmacological agents are used to treat behavioural symptoms.


A four-stage approach to the management of Lewy Body Disease has been described, which includes: accurate diagnosis, identification of target symptoms with patient and caregiver, medical interventions; and non-medical interventions.&nbsp;<ref name="Boot">Boot B. P. Comprehensive treatment of dementia with Lewy bodies. Alzheimer’s Research Therapy, 2015 May 29; 7:45. [https://alzres.biomedcentral.com/articles/10.1186/s13195-015-0128-z DOI: 10.1186/s13195-015-0128-z]</ref><br>
* Home care nurses play a crucial role in regularly assessing the patient and providing support services.
* Education of the caregiver is essential, the loved ones needing to be aware of the behavior changes, hallucinations, and fluctuations in cognition. Caregivers have to monitor the patient closely as they have a love level of functioning, with most unable to perform ADLs and are prone to falls and aspiration pneumonia.
* The pharmacist  needs to educate caregivers that no medical therapy will cure the cognitive changes and the drugs simply manage behaviour and motor deficits ( and many have adverse effects).
* A mental health nurse is often needed as depression is common. Close communication between members of the interprofessional team is vital to improve outcomes.<ref name=":1" />  


=== Medical Management ===
== Prognosis ==
<br>A number of medication types have been shown to provide some benefit, however, it is very individualised and may work better for one individual than another..&nbsp;<ref name="Lewy" />  Treatment can be categorized based on symptoms: autonomic, cognitive, movement, neuropsychiatric and sleep.
LBD has a poor prognosis, with an average life expectancy from initial diagnosis to death 5-8 years. Death usually occurs from one of the many complications eg falls, immobility, cardiac complications, medication side effects, pneumonia, swallowing problems, suicide.<ref name=":2" />
# '''Treatment for Autonomic Symptoms<ref name="Boot" />'''
#*Reduction or cessation of antihypertensive medication
#*Salt supplementation
#*Fludrocortisone
#*Domperidone
#*Cholinesterase inhibitors
#*Pyrodostigmine
#'''Treatment for Cognitive Symptoms'''
#*Cholinesterase Inhibitors: Considered the “standard” treatment for Lewy Body Disease. <ref name="Lewy" /> Cholinesterase inhibitors are used to treat cognitive symptoms that may be present for a patient suffering from LBD. This treatment compares favorably with similar treatment in Alzheimer’s disease, as DLB patients have profound cholinergic dysfunction. Precautions must be reviewed prior to prescribing cholinesterase inhibitors, as these medications may increase risk of bradycardia, nausea, vomiting, diarrhea, anorexia and weight loss. Rivastigmine has the widest evidence base for positive effect in individuals Lewy Body Disease.&nbsp;<ref name="Boot" />
#*Memantine
#'''Treatment of Movement Symptoms'''
#* Levodopa/Carbidopa: Lower doses of levodopa are prescribed, as motor symptoms experienced in Lewy Body Disease is less than uncomplicated Parkinson’s disease.&nbsp;<ref name="Dick">Aarsland, D., Burn, D., Chiu, H., Cohen-Mansfield, J., Dickson D, Dubois B, Duda J, Feldman H, Gauthier S, Halliday G, Lawlor B, Lippa C, Lopez O, Machado J, McKeith I, Mintzer J, O’Brien J, Playfer J, Reid W. Dementia with Lewy bodies. The Lancet Neurology, 2004 January; 3:19-28. [http://www.thelancet.com/journals/laneur/article/PIIS1474-4422(03)00619-7/fulltext DOI: http://dx.doi.org/10.1016/S1474-4422(03)00619-7]</ref>
# '''Treatment of Neuropsychiatric Symptoms<ref name="Boot" />'''
#*Cholinesterase Inhibitors
#*Antipsychotic medications (quetiapine, clozapine)
#'''Treatment of Sleep Symptoms'''&nbsp;
#*Caffeine (for patients without periodic limb movement disorder)
#*Methylphenidate
#*Dextroamphetamine
#*Modafinil
#*Armodafinil
#*Melatonin
#*Rivastigmine
#*Clonazepam<br>
Restless Leg Syndrome and Periodic Limb Movement Disorder: *
*Cardidopa/Levodopa,
*Benzodiazepines (i.e. Clonazepam)
*Alpha-2-delta calcium channel ligands (gabapentin, gabapentin enacarbil, and pregabalin)<br> <br>* - The above treatment for restless leg syndrome and periodic limb movement disorder are effective in patients with Parkinson’s Disease. There are no trials of these symptoms in the context of Lewy Body Disease.


=== Physiotherapy Management ===
=== Physiotherapy Management ===
<br>Physiotherapy for Lewy Body Disease is similar to that of Parkinson’s Disease. It can help manage parkinsonism that is prevalent in LBD by providing intervention which will encourage strengthening and flexibility exercises and gait training. Aerobic exercise should be included to optimise cardiovascular fitness.<ref name="Lewy" /> Physiotherapy is especially helpful in improving balance and postural stability to minimize the risk of falls. With the addition of exercise, non-motor symptoms such as cognitive decline, sleep problems and fatigue will also improve. As the disease progresses and the dementia increases, exercise can be hard to do. Therefore, it is important to incorporate exercise in the early and middle stages of Lewy Body Disease. A study<ref>Sondell A, Littbrand H, Holmberg H, Lindelöf N, Rosendahl E. [https://www.ncbi.nlm.nih.gov/pubmed/31781732 Is the Effect of a High-Intensity Functional Exercise Program on Functional Balance Influenced by Applicability and Motivation among Older People with Dementia in Nursing Homes?.] The journal of nutrition, health & aging. 2019 Dec 1;23(10):1011-20.</ref> suggests that a high-intensity functional exercise program has positive outcomes on balance in these patients.<br> <br>Tips to help make exercise easier to maintain:<ref name="Lewy" />  
[[File:Elderly-people-doing-balance-exercise.jpg|thumb|Balance training]]
Physiotherapy for Lewy Body Disease is similar to that of Parkinson’s Disease. It can help manage parkinsonism that is prevalent in LBD by providing intervention such as [[Strength Training|strengthening]] and [[flexibility]] exercises and [[gait]] training. [[Aerobic Exercise|Aerobic]] exercise should be included to optimise [[Cardiovascular System|cardiovascular]] fitness.<ref name="Lewy">Lewy Body Dementia Association. What is LBD? Available from: https://www.lbda.org/category/3437/what-is-lbd.htm [Accessed May 5, 2017]</ref> Physiotherapy is especially helpful in improving [[balance]] and [[Postural Control|postural]] stability to minimize the risk of [[falls]]. With the addition of exercise, non-motor symptoms such as cognition, sleep and fatigue will improve. As the disease progresses and the dementia increases, exercise can be hard to do. Therefore, it is important to incorporate exercise in the early and middle stages of Lewy Body Disease. A study<ref>Sondell A, Littbrand H, Holmberg H, Lindelöf N, Rosendahl E. [https://www.ncbi.nlm.nih.gov/pubmed/31781732 Is the Effect of a High-Intensity Functional Exercise Program on Functional Balance Influenced by Applicability and Motivation among Older People with Dementia in Nursing Homes?.] The journal of nutrition, health & aging. 2019 Dec 1;23(10):1011-20.</ref> suggests that a high-intensity functional exercise program has positive outcomes on balance in these patients.<br> <br>Tips to help make exercise easier to maintain:<ref name="Lewy" />  


*Provide visual cues by demonstrating exercises  
*Provide visual cues by demonstrating exercises  
Line 116: Line 78:
*Make exercise fun and enjoyable
*Make exercise fun and enjoyable


== Resources  ==
== References ==
 
https://www.lbda.org/
 
http://www.parkinson.ca/site/c.kgLNIWODKpF/b.8647145/k.6D4A/Diffuse_Lewy_Body_Disease.htm
 
http://www.lewybodyjournal.org/
 
http://www.lewybodyjournal.org/links.html
== References ==
 
<references />


[[Category:Neurological - Conditions]]  
<references />
[[Category:Queen's_University_Neuromotor_Function_Project]]
[[Category:Queen's University Neuromotor Function Project]]
[[Category:Neurological - Conditions]]
[[Category:Older People/Geriatrics]]
[[Category:Older People/Geriatrics]]
[[Category:Dementia]]
[[Category:Dementia]]

Latest revision as of 07:52, 6 October 2023

Original Editor - Henry Tan, Kevin Lam, Alex Mong, Andrew To as part of the Queen's University Neuromotor Function Project

Top Contributors - Henry Tan, Andrew To, Alex Mong, Lucinda hampton, Kim Jackson, Kevin Lam, Vidya Acharya, Claire Knott, 127.0.0.1, Evan Thomas and Lauren Lopez  

Introduction[edit | edit source]

Lewy body disease or Lewy body dementia (LBD), is a neurodegenerative disease (specifically a synucleinopathy) related to Parkinson disease.[1]

Dementia

There are 2 types of LBD generally described in the literature:[2]

  1. Dementia with Lewy Bodies: dementia occurring first or within one year of movement disorder.
  2. Parkinson Disease Dementia: dementia occurring in a patient who receives a diagnosed of Parkinson's disease and then develops dementia symptoms after one year or more of the diagnosis.

People with either type generally develop similar symptoms as the disease progresses. Distinct Parkinsonian symptoms such as slowness of movement, rigidity, REM sleep behaviour disorder and visual hallucinations can help to distinguish DLB from Alzheimer’s disease.[3]

Epidemiology[edit | edit source]

  • Occurs in older patients (onset typically in 50-70 years of age), and is sporadic. However a family history of LBD and Parkinson disease will increase a person's risk.[4]
  • LBD is one of the most common causes of dementia (accounting for up to 20% to 30% of cases[4]) along with Alzheimers disease (AD)[3].

Etiology[edit | edit source]

The etiology of LBD is unknown. Genetics, environmental factors, and changes linked to aging, may have a role and research is still ongoing.[5]

LBD has been found to be strongly linked to a protein called alpha-synuclein. The abnormal accumulation of this protein in certain regions of the brain causes dramatic cognitive and motor deficits affecting behaviour, mood, movement, and thinking.

Clinical Presentation[edit | edit source]

Frontal lobe.jpeg

The clinical features of LBD are the consequence of the blockage of information transfer from the striatum to the cortex, more notably the frontal lobe.

Core features:[6]

  1. Fluctuating cognitive impairment especially in executive function, attention and alertness
  2. Visuospatial impairment, including visual hallucinations (detailed and vivid)
  3. Concurrent parkinsonian symptoms may be present but are less common, more frequently occurring years after the onset of dementia eg early extrapyramidal features (dystonia, akathisia, muscle rigidity, bradykinesia, tremor, tardive dyskinesia

Watch this 3 minute video showing a personal story "It happened little by little. First he would forget things, then he'd lose track of what he was doing. LBD took over the life of the man you're about to meet."

Pathophysiology[edit | edit source]

The characteristic feature of dementia with Lewy bodies is the accumulation of Lewy bodies throughout the brain. These intracellular inclusions result from the aggregation of misfolded α-synuclein. Neurofibrillary tangles are also present, however they lack an amyloid core, as seen in AD[1].

Regions of the brain affected by LBD include: cerebral cortex, limbic cortex, hippocampus, midbrain, brainstem.


Source (http://labiotech.eu/major-cns-disease-milestones-in-biotech-2015/)

Diagnostic Procedures[edit | edit source]

It is important to realise that there is a significant overlap between many neurodegenerative diseases, and that a clear-cut distinction between entities is not always possible. No precise test can accurately diagnose LBD. A thorough assessment is useful to reach an alternative working diagnosis (or rules out similar conditions):

Management[edit | edit source]

There is no effective treatment for LBD and the condition is progressive. Pharmacological Management includes cholinesterase inhibitors. They treat the cognitive symptoms of LBD and are central to treatment. eg rivastigmine, galantamine, and donepezil. Acetylcholine is an important neurotransmitter for memory. People with eg LBD have reduced levels present in their brains. Cholinesterase inhibitors inhibit this enzyme , improving brain network communication.[4].

The other pharmacological agents are used to treat behavioural symptoms.

  • Home care nurses play a crucial role in regularly assessing the patient and providing support services.
  • Education of the caregiver is essential, the loved ones needing to be aware of the behavior changes, hallucinations, and fluctuations in cognition. Caregivers have to monitor the patient closely as they have a love level of functioning, with most unable to perform ADLs and are prone to falls and aspiration pneumonia.
  • The pharmacist needs to educate caregivers that no medical therapy will cure the cognitive changes and the drugs simply manage behaviour and motor deficits ( and many have adverse effects).
  • A mental health nurse is often needed as depression is common. Close communication between members of the interprofessional team is vital to improve outcomes.[5]

Prognosis[edit | edit source]

LBD has a poor prognosis, with an average life expectancy from initial diagnosis to death 5-8 years. Death usually occurs from one of the many complications eg falls, immobility, cardiac complications, medication side effects, pneumonia, swallowing problems, suicide.[4]

Physiotherapy Management[edit | edit source]

Balance training

Physiotherapy for Lewy Body Disease is similar to that of Parkinson’s Disease. It can help manage parkinsonism that is prevalent in LBD by providing intervention such as strengthening and flexibility exercises and gait training. Aerobic exercise should be included to optimise cardiovascular fitness.[7] Physiotherapy is especially helpful in improving balance and postural stability to minimize the risk of falls. With the addition of exercise, non-motor symptoms such as cognition, sleep and fatigue will improve. As the disease progresses and the dementia increases, exercise can be hard to do. Therefore, it is important to incorporate exercise in the early and middle stages of Lewy Body Disease. A study[8] suggests that a high-intensity functional exercise program has positive outcomes on balance in these patients.

Tips to help make exercise easier to maintain:[7]

  • Provide visual cues by demonstrating exercises
  • Play upbeat music or music the person enjoys
  • Arrange exercise classes or include the support/care-person
  • Do exercises in sitting
  • Make exercise fun and enjoyable

References[edit | edit source]

  1. ↑ 1.0 1.1 Radiopedia Dementia with Lewy bodies Available:https://radiopaedia.org/articles/dementia-with-lewy-bodies (accessed 13.9.2022)
  2. ↑ McKeith IG, Galasko D, Kosaka K, Perry EK, Dickson DW, Hansen LA, Salmon DP, Lowe J, Mirra SS, Byrne EJ, Lennox G. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB) Report of the consortium on DLB international workshop. Neurology. 1996 Nov 1;47(5):1113-24. PMID:8909416
  3. ↑ 3.0 3.1 National Institute on Aging. Lewy Body Dementia: Information for Patients, Families, and Professionals. (Accessed 4 May 2017). https://www.nia.nih.gov/alzheimers/publication/lewy-body-dementia/basics-lewy-body-dementia
  4. ↑ 4.0 4.1 4.2 4.3 Haider A, Spurling BC, Sánchez-Manso JC. Lewy body dementia. InStatPearls [Internet] 2022 Oct 23. StatPearls Publishing.Available:https://www.ncbi.nlm.nih.gov/books/NBK482441/ (accessed 6.10.2023)
  5. ↑ 5.0 5.1 5.2 Haider A, Spurling BC, Sánchez-Manso JC. Lewy body dementia. InStatPearls [Internet] 2021 Jul 12. StatPearls Publishing.Available:https://www.ncbi.nlm.nih.gov/books/NBK482441/ (accessed 13.9.2022)
  6. ↑ Gnanalingham KK, Byrne EJ, Thornton A, Sambrook MA, Bannister P. Motor and cognitive function in Lewy body dementia: comparison with Alzheimer's and Parkinson'ss. Journal of Neurology, Neurosurgery & Psychiatry. 1997 Mar 1;62(3):243-52. http://jnnp.bmj.com/content/jnnp/62/3/243.full.pdf
  7. ↑ 7.0 7.1 Lewy Body Dementia Association. What is LBD? Available from: https://www.lbda.org/category/3437/what-is-lbd.htm [Accessed May 5, 2017]
  8. ↑ Sondell A, Littbrand H, Holmberg H, Lindelöf N, Rosendahl E. Is the Effect of a High-Intensity Functional Exercise Program on Functional Balance Influenced by Applicability and Motivation among Older People with Dementia in Nursing Homes?. The journal of nutrition, health & aging. 2019 Dec 1;23(10):1011-20.
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