Lewy Body Disease: Difference between revisions

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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]]


'''Lead Editors''' &nbsp;  
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== Definition ==
== 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>


Lewy Bodies were first discovered by a German neurologist in 1912, Dr. Friederich Lewy. Lewy body disease/dementia (LBD) has since been 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, other causes include: Alzheimer’s, stroke, brain tumours and Parkinson’s.  
# 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.


There are 2 types of LBD generally described in the literature based on an arbitrary “1-year rule”:<ref name="Mckeith" /><br>1) Dementia with Lewy bodies (DLB) – Cognitive deficits appear within a year of onset of parkinsonism related deficits. <br>2) Parkinson’s disease with dementia (PDD) – Cognitive deficits appear beyond one year of parkinsonism.<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>  
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  ==


People with either type generally develop similar symptoms as the disease progresses. To further complicate things, individuals with DLB may present as having Alzheimer’s disease. However, distinct symptoms such as: slowness of movement, rigidity, REM sleep behavior disorder and visual hallucinations can help distinguish DLB from Alzheimer’s disease.<ref name="National" /><br>  
* 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" />.


<br>  
== Etiology ==
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>


{{#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.


== Clinically Relevant Anatomy<br> ==
== Clinical Presentation ==
 
[[File:Frontal lobe.jpeg|right|frameless]]
Although not fully understood, alpha synuclein has been noted to be important to many functions in the brain, particularly at synapses between neurons in the brain. 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 death of those neurons in the brain.<ref name="National" />
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]].  
 
Postmortem studies have shown development of Lewy Bodies in the substantia nigra, locus corulues, 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>


Regions of the brain affected by LBD include: <br>• Cerebral cortex<br>• Limbic cortex<br>• Hippocampus<br>• Midbrain<br>• Brain stem
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>
 
[[Image:Lewybody.png]]<br><br>
 
== 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. (Mckeith) Age seems to be the greatest risk factor, typically developing in individuals who are over the age of 50. Although it varies greatly (2 to 20 years), the disease on average lasts 5 to 7 years from onset to death with men being affected more than women.<ref name="National" />
 
Other potential risk factors include:<ref name="National" /><br>• Parkinson’s disease <br>• REM sleep disorder<br>• Genetics (although only a small genetic link has been found)<br>• No association between lifestyle and LBD, but a healthy and active lifestyle may decrease ones chances of developing age related dementias<br>
 
== Clinical Presentation  ==


The clinical features of Lewy body disease are mainly a consequence in the blockage of information transfer from the striatum to the cortex, more notably the frontal lobe.  
# Fluctuating cognitive impairment especially in executive function, attention and alertness
# Visuospatial impairment, including visual hallucinations (detailed and vivid)
# 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
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>


Signs and symptoms of LBD:<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's diseases. Journal of Neurology, Neurosurgery &amp;amp;amp;amp;amp; Psychiatry. 1997 Mar 1;62(3):243-52. http://jnnp.bmj.com/content/jnnp/62/3/243.full.pdf</ref>
{{#ev:youtube|RSRbR1R4mz0|300}}


*Recurrent visual hallucinations
== Pathophysiology  ==
*Visuospatial impairment (Stroop test, Clock face test)
*Executive function deficits (Nelson Card sort test)
*Parkinsonian motor features (mild gait impairments, resting tremor)
*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><br>


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


== Pathophysiology  ==


The distinguishing feature of dementia with Lewy bodies is the aggregation and findings of these Lewy bodies inside neurons of the cerebral cortex. Despite the numerous cases of patients with dementia, the pathology regarding Lewy bodies is still unclear as 3-10% of elderly individuals possess Lewy bodies but display no signs or symptoms.<ref name="Cum">Cummings JL. Lewy Body Diseases with Dementia-Pathophysiology and Treatment. Brain and cognition. 1995 Aug 31;28(3):266-80. [http://www.sciencedirect.com/science/article/pii/S0278262685712572 doi.org/10.1006/brcg.1995.1257]</ref> However, with patients whom are indeed symptomatic, relationships have been drawn demonstrating a link between lewy bodies and presenting symptoms. Recent research by Aarsland et al, has 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" /> In addition, increased Lewy body densities in the limbic and frontal lobes also correlate strongly with the severity 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" />
Source (http://labiotech.eu/major-cns-disease-milestones-in-biotech-2015/)


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


Lewy Body Disease (LBD) can have two forms of clinical diagnoses which are dementia with Lewy bodies and Parkinson’s disease dementia <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>. There are many different diagnostic tools that can be used to diagnose an individual with Lewy body Disease. For every patient that gets diagnosed with LBD, it varies with different types of symptoms that they experience. Symptoms may change from day to day or hour to hour <ref name="Lewy" />.<br>
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):  
 
It is difficult to diagnose Lewy Body Disease with dementia because it shows similar signs and symptoms to those of Alzheimer and Parkinson’s disease. Therefore LBD can sometimes be missed when diagnosing an individual <ref name="Lewy" />. But overall, when individuals progress and develop more symptoms of LBD then it becomes easier to diagnosis.<br>To diagnosis the type of LBD, physical and neurological evaluations are used and a thorough patient interview is done. For example,<br>
 
*Medical history (past and current diseases and disorders)
*Medications
*Movement evaluation
*Current symptoms
*Social history
*Functional abilities
*Cognitive abilities (attention, language, memory, visual)
*Brain imaging (CT or MRI)
*Blood tests
 
Ultimately, all these evaluations can’t provide a conclusive diagnosis of LBD. The only way to confirm if an individual has LBD is to have a post mortem autopsy of their brain <ref name="Lewy" />. With clinical imaging, cortical and subcortical atrophy can be noticeable with LBD. Atrophy can also be seen in the grey matter of the temporal, frontal, and parietal lobes and the insular cortex in an individual 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" />. In particular with Parkinson disease dementia, there can be atrophy of the nirgrostriatal system and also compromised dopamine transporters <ref name="Morra" />. <br>
 
'''Clinical Diagnosis of DLB:'''<br>To make a diagnosis of dementia with lewy bodies, it has to have one of the core features which are fluctuation in cognition, visual hallucinations, and motor parkinsonism <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> If two are present, a higher case can be made for a patient with DLB.<br> <br>With changes in cognition, a patient can experience variations in attention and alertness. They can also experience confusion from time to time and can have changes in fluctuation in mental abilities and behaviour <ref name="Mckeith" />. The changes can occur from day to day and can vary in amplitude for a patient experiencing these cognitive changes.<br> <br>With motor parkinsonism, it is usually mild with patients with DLB. Some of the features are rigidity, bradykinesia, masked face, stooped posture, and shuffling gait <ref name="Mckeith" />. A patient can have a mix of these features associated with motor parkinsonism. If a patient experiences motor parkinsonism more than a year after a diagnosis of DLB, it is more appropriately to diagnose the patient with Parkinson's with dementia instead <ref name="Mckeith" />.<br> <br>Furthermore, there are suggestive secondary features that can exist with a patient with DLB. If the one of the core features exist with an existing secondary feature, the diagnosis of DLB is very likely <ref name="Mckeith" />.<br> <br>'''Suggestive Features:'''
 
*REM sleep behaviour disorder
*Severe neuroleptic sensitivity
*Low dopamine transporter uptake in basal ganglia
 
Moreover, there are other features that DLB can show but are not diagnostic specificity <ref name="Mckeith" />.
 
*Unexplained loss of consciousness
*Falls and syncope
*Severe autonomic dysfunction
*Systematized delusions
 
'''Clinical Diagnosis of PDD:'''<br>One of the core features of developing Parkinson’s disease dementia is having idiopathic PD before getting dementia and to have a confirmed diagnosis of Parkinson disease <ref name="Poewe">Poewe W, Gauthier S, Aarsland D, Leverenz JB, Barone P, Weintraub D, Tolosa E, Dubois B. Diagnosis and management of Parkinson’s disease dementia. International journal of clinical practice. 2008 Oct 1;62(10):1581-7. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2658001/ PMID:2658001]</ref>. Also individuals with PDD will have a decreased global cognitive impairment that disrupts daily life. They will present with a impairment of one of the cognitive domains which are attention, executive function, visual abilities, and memory <ref name="Poewe" />. The tests to evaluate the cognitive domains are described in Table 2 in <ref name="Poewe" />. <br>
 
== 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. This study, conducted by Ballard, McKeith, Burn, Harrison, O’Brien, Lowery, Campbell, Perry &amp; Ince assessed 73 patients using the UPDRS and staged using the Hoehn &amp; Yahr system.<ref name="Ballard">Ballard C, Burn D, Campbell M, Harrison R, Ince P, Lowery K, McKeith I, O’Brien J, Perry R. The UPDRS scale as a means of identifying extrapyramidal signs in patients suffering from dementia with Lewy bodies. Acta Neurologica Scandinavica, 1997 Dec; 96(6):366-71. [https://www.ncbi.nlm.nih.gov/pubmed/9449473 PMID:9449473]</ref>&nbsp;They suggest that a 5-item subscale of the UPDRS be used as it “provides a reliable and generally applicable instrument for the assessment of parkinsonism in [patients with Lewy Body Disease]”.<br>
 
== Management / Interventions<br>  ==
 
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 carer, 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>
 
'''Medical Management:'''<br>Medications have been shown to provide some benefit, however, it is very individualized. A medication prescription may work better for an individual than for another one.&nbsp;<ref name="Lewy" /><br> <br>Treatment can be categorized based on symptoms: autonomic, cognitive, movement, neuropsychiatric and sleep.<br> <br>'''1. Autonomic Symptoms<ref name="Boot" />'''
 
*Postural Hypotension
*Reduction or cessation of antihypertensive medication
*Salt supplementation
*Fludrocortisone
*Domperidone
*Cholinesterase inhibitors
*Pyrodostigmine
 
'''2. Cognitive Symptoms'''


*Cholinesterase Inhibitors:
* 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


Considered the “standard” treatment for Lewy Body Disease. <ref name="Lewy" />&nbsp;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" /><br>  
* [[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" />


*Memantine
== Management    ==


'''3. Movement Symptoms'''&nbsp;<br>Levodopa/Carbidopa is most useful in patients with Lewy Body Disease that experience parkinsonism without neuropsychiatric symptoms. <ref name="Boot" />&nbsp;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><br> <br>'''4. Neuropsychiatric Symptoms<ref name="Boot" />'''<br>
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" />.


*Cholinesterase Inhibitors
The other pharmacological agents are used to treat behavioural symptoms.
*Antipsychotic medications (quetiapine, clozapine)


'''5. Sleep Symptoms'''&nbsp;<br>Excessive daytime sleepiness, rapid eye movement sleep behaviour disorder (RBD), restless leg syndrome and periodic limb movement disorder are very common with synuclein disorders. These symptoms can be quite responsive to treatment.<ref name="Boot" /><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" />  


&nbsp; &nbsp; Excessive Daytime Sleepiness:  
== Prognosis ==
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" />


*Caffeine (for patients without periodic limb movement disorder)
=== Physiotherapy Management ===
*Methylphenidate
[[File:Elderly-people-doing-balance-exercise.jpg|thumb|Balance training]]
*Dextroamphetamine
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" />  
*Modafinil
*Armodafinil<br>
 
&nbsp; &nbsp; RBD:
 
*Melatonin
*Rivastigmine
*Clonazepam
 
&nbsp; &nbsp; 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.
 
<br> '''Non-Medical Management:'''&nbsp;<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 treatments and interventions, which include strengthening and flexibility exercises and gait training. Aerobic exercise can be implemented to increase cardiovascular fitness as well.<ref name="Lewy" /> Physiotherapy is especially helpful in improving balance and postural stability to minimize 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 to help maintain health.<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 149: Line 78:
*Make exercise fun and enjoyable
*Make exercise fun and enjoyable


Individuals may also benefit from occupational therapy, speech language therapy, and psychotherapy.<br><br>
== References ==
 
== Differential Diagnosis<br>  ==
 
There are many similar diseases and disorders similar to Lewy body dementia. LBD is a form of dementia but there many different forms of dementia. For example, Alzheimer’s disease is another form of dementia. Those symptoms include progressive loss of memory, regression of cognitive dysfunction (thinking and judgement), depression, personality changes and disorientation of time and place <ref name="Lewy" />. An individual can develop Alzheimer’s disease and then progress to express core features of dementia with Lewy bodies. The difference between Lewy body Disease with dementia and Alzheimer’s is unpredictable cognitive abilities and attention, changes in movement patterns, visual hallunications, and REM sleep behaviour disorder <ref name="Lewy" />.
 
With Parkinson with dementia, it often starts with a movement disorder that leads to the diagnose of Parkinson disease then the core the features of Parkinson with dementia start to appear.
 
Other forms of dementia that are similar to LBD are vascular dementia and frontotemporal dementia. Vascular dementia is when a stroke occurs within the brain which causes decrease in oxygen in the brain and symptoms of disorientation, walking difficulties, shuffling gait, and inappropriate behaviour <ref name="Lewy" />. With frontotemporal dementia, the common symptoms are changes in personality and behaviour, language and speech impairments and movement changes <ref name="Lewy" />. <br><br>
 
== Resources <br>  ==
 
https://www.lbda.org/
 
http://www.parkinson.ca/site/c.kgLNIWODKpF/b.8647145/k.6D4A/Diffuse_Lewy_Body_Disease.htm<br>
 
http://www.lewybodyjournal.org/
 
http://www.lewybodyjournal.org/links.html<br>
 
<br>
 
== References ==
 
<references />
 
References will automatically be added here, see [[Adding References|adding references tutorial]].


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

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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