Lewy Body Disease

Introduction[edit|edit source]

Lewy body disease or Lewy bodydementia(LBD), is aneurodegenerative disease(specifically asynucleinopathy) related toParkinson disease.^[1]

通常有两种类型的小黑裙在描述e 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, REMsleepbehaviour 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.
• LBD is one of the most common causes of dementia (accounting for 15-20% of cases^[1]) along withAlzheimers disease (AD)^[3].

Etiology[edit|edit source]

The etiology of LBD is unknown.Genetics, environmental factors, and changes linked toaging, may have a role and research is still ongoing.^[4]

LBD has been found to be strongly linked to aproteincalledalpha-synuclein. The abnormal accumulation of thisproteinin certain regions of thebraincauses dramaticcognitiveand motor deficits affecting behaviour, mood, movement, and thinking.

Clinical Presentation[edit|edit source]

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

Core features:^[5]

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 earlyextrapyramidalfeatures (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].

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):

• Detailed history and examination
• Assessment ofcognitivefunction
• Blood tests(e.g.,vitamin B12levels, chemistry panel,thyroidprofile,syphilis,HIV) to rule out other causes of dementia

• Imagingstudies (e.g., CT scan, MRI scan, SPECT scan, PET scan)
• Cerebrospinal fluidexaminations have no significant role when conducted in these patients
• Sleep evaluationfor REM sleep behavior disorder^[4]

Management[edit|edit source]

There is no effective treatment for LBD and the condition is progressive. The available pharmacological agents are only used to treat behavioral 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 cures the cognitive changes and the drugs simply manage behavior 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.^[4]

Physiotherapy Management[edit|edit source]

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 asstrengtheningandflexibilityexercises andgaittraining.Aerobicexercise should be included to optimisecardiovascularfitness.^[6]在改善理疗是尤其有用balanceandposturalstability to minimize the risk offalls. 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^[7]suggests that a high-intensity functional exercise program has positive outcomes on balance in these patients.

Tips to help make exercise easier to maintain:^[6]

• 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]