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26 Cards in this Set
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Which is not a common symptom of Lewy body dementia? 1. Visual hallucinations 2. Muscle rigidity 3. Visuospatial difficulties 4. Bradykinesia
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.Well done! You answered successfullyTeaching Points Visual hallucinations and extrapyramidal symptoms including bradykinesia and rigidity are typical of Lewy body dementia. Visuospatial difficulties are more common in patients with Alzheimer disease. |
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Select the cognitive function least affected with Lewy body dementia. 1. Attention 2. Memory 3. Executive function 4. Visual function
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.Well done! You answered successfullyTeaching PointsLewy body dementia is characterized by progressive cognitive decline and associated Parkinsonism.There are often cognitive function fluctuations and intermittent impairment of alertness.Visuospatial function and executive function are preferentially affected.Memory is better than in other forms of dementia. |
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What is cognitive function |
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Which of the following is least likely in Lewy body dementia? 1. Nonvisual hallucinations 2. Variations in alertness 3. Minimal response to neuroleptics 4. Multiple falls
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Well done! You answered successfullyTeaching PointsPatients with Lewy body dementia show hypersensitivity to neuroleptics.The most important criteria for diagnosis of this condition in progressive cognitive decline interfering with occupational and social function.The diagnosis is often confirmed by sudden onset of Parkinsonism.Other characteristics include delusions, nonvisual hallucinations, variations in alertness and attention, syncope, and repeated falls |
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A patient has parkinsonism for less than one year and develops dementia. What is the most likely diagnosis? 1. Alzheimer disease 2. Amyotrophic lateral sclerosis (ALS) 3. Diffuse Lewy body disease 4. Progressive supranuclear palsy
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.Well done! You answered successfullyTeaching PointsDiffuse Lewy body disease is characterized by the clinical combination of parkinsonism and dementia.If the parkinsonism has been present for less than one year before the dementia it is called Lewy body dementia. If it has been present for more than one year it is called Parkinson disease with dementia.ALS is characterized by fasciculations, spasticity and dysarthria. Alzheimer disease is the most common form of dementia.Parkinson disease has 4 cardinal signs-resting tremor, rigidity, bradykinesia and postural instability. |
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In a 65-year-old male with dementia, what is the most likely additional diagnosis if visual hallucinations are also a symptom? 1. Parkinson 2. Lewy body 3. Schizophrenia 4. Vascular
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.Well done! You answered successfullyTeaching PointsVisual hallucinations with dementia is highly suggestive of Lewy body dementia.Other signs include delusions and misidentification.10 to 20 percent of dementia is thought to be caused by Lewy bodies, according to autopsy studies.Dementia with Lewy bodies is a type of dementia that worsens over time. Symptoms may include fluctuations in alertness, visual hallucinations, slowness of movement, trouble walking, and rigidity. Excessive movement during sleep, mood changes, and depression are common. The underlying mechanism involves the buildup of Lewy bodies which are clumps of alpha-synuclein protein in neurons. The diagnosis is suspected based on symptoms, with blood tests and medical imaging is done to rule out other causes. The differential diagnosis includes Parkinson's and Alzheimer's. There is no cure. Treatment is supportive. Acetylcholinesterase inhibitors, such as donepezil, may provide benefit. Motor problems may improve with levodopa. Antipsychotics should be avoided due to side effects. |
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What is dementia of Lewy body |
It is a progressive degenerative brain disorder characterized by dementia, psychosis, and features of parkinsonism. It is the third most common type of dementia after Alzheimer disease and Vascular dementia. It characterizes by the deposition of Lewy bodies in the brain that are intraneuronal cytoplasmic inclusion bodies having aggregates of alpha-synuclein and ubiquitin. |
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What are the two types of dementia of Lewy body |
Lewy Body Dementia (LBD) encompasses two clinical entities, namely dementia with Lewy bodies and Parkinson disease dementia.
Due to the incomplete specificity in the clinical diagnosis and the pathological definition of the disease, a postmortem biopsy or autopsy is the only method to secure a definite diagnosis.Treatment / ManagementPharmacological ManagementCholinesterase inhibitors: Used to treat the cognitive symptoms of LBD and are the mainstay of treatment. Initially developed for Alzheimer disease treatment, they are probably more effective in patients with LBD. These include rivastigmine, galantamine, and donepezil.Carbidopa-Levodopa: Used to treat movement symptoms.; however, it has serious side effects and can lead to delusions, hallucinations, and confusion and practitioners should use them with caution in these patients, and they should start with low doses if required.Atypical antipsychotics: Used to treat hallucinations that cause significant distress in patients not responding to standard cholinesterase inhibitors. Commonly used drugs include clozapine, quetiapine, and aripiprazole. Use with caution due to neuroleptic sensitivity in these patients.Clonazepam: Used for REM sleep behavior disorder.SSRIs: Depression is common in patients with LBD and often requires antidepressant therapy.Support and TherapiesPatient and caregiver education regarding symptoms of a disease and their management is necessary. Understanding the disease helps the caregivers cope with the everyday challenges. They require home modifications occasionally and individual patient needs should specifically guide them.Patients can take part in different therapies to improve their quality of life, including:PhysiotherapyOccupational therapySpeech therapySupport groupsIndividual and family psychotherapies.Differential DiagnosisIt is of utmost importance to differentiate LBD from similar conditions since it is more responsive to certain medications if used early in the disease course.Similar conditions include:Parkinson diseaseAlzheimer diseaseFrontotemporal dementiaPrion-related diseasesPrognosisThe prognosis of LBD is fair to poor. Patients die from multiple complications like falls, immobility, cardiac complications, medication side effects, pneumonia, swallowing problems, and depression leading to suicide. The average life expectancy is only five to eight years after the initial diagnosis. This also can be due to a lack of knowledge regarding LBD among physicians and the population and difficulty in differentiating it from other similar conditions which leads to a delay in diagnosis which delays the onset of specific therapy. Health professionals need to improve awareness regarding LBD and there should develop investigative methods to ensure its early diagnosis.
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What is the aetiology of dementia of Lewy body |
The etiology of LBD is still unknown. Genetics, environmental factors, and changes linked to aging, however, may have a role and still require further research. |
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What is the epidemiology of dementia of Lewy body |
LBD is an under-diagnosed condition as it is poorly understood and its clinical features overlap with other more common disorders, like Parkinson's disease and Alzheimer's disease. Studies have shown, however, that it accounts for up to 20% to 30% of all dementia cases. It is more common in men, and incidence increases with advancing age. |
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What is the pathophysiology of dementia of Lewy body disease |
Like Alzheimer disease, LBD presents with acetylcholine deficiency, but it is more pronounced in LBD. Decreased levels of acetylcholine in temporal and parietal cortex result in visual hallucinations (a prominent feature of LBD), while up-regulation of muscarinic M1 receptors in the temporal lobe results in delusions. Dopamine levels also diminish. |
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What are the characteristic histological features of dementia of Lewy body |
The pathology of LBD overlaps that of Parkinson's disease and Alzheimer's disease. Neuronal cytoplasmic inclusion bodies, called Lewy bodies (comprising aggregates of ubiquitin and alpha-synuclein) and found within brain parenchyma (mainly in the brainstem, limbic system, and cerebral cortex), are characteristic of Lewy body dementia. |
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What is the pathological process |
Genetic mutations, environmental toxins, and the aging process can lead to misfolding of alpha-synuclein and its accumulation in the form of Lewy bodies via oxidative stress and mitochondrial dysfunction. Other features include Lewy neuritis, senile plaques, neurofibrillary tangles, and neuronal loss in the substantia nigra, locus coeruleus, and Meynert nucleus. |
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How does the location of Lewy bodies decide the disease |
The location of Lewy bodies determines the clinical presentation. If Lewy bodies develop initially in the brainstem and cerebral cortex, then dementia sets on early, and we call it dementia with Lewy bodies; however, if Lewy bodies develop initially in the brain stem only and extend to cerebral cortex later, then dementia occurs late in the disease process and we call it Parkinson disease dementia. |
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How can the symptoms help us come to a diagnosis |
Symptoms fluctuate with time and vary among different individuals. Diagnosis of LBD requires thorough clinical examination as many of its features overlap with other dementia disorders. History and PhysicalThere is a large variation in symptoms manifested among patients and their time of onset. The main features include progressive dementia (mainly affects attention and executive function, memory loss is not common but can occur later in the disease process), fluctuation cognitive function (with variation in attention and episodes of drowsiness), visual hallucinations (detailed and recurrent), and features of parkinsonism like muscular rigidity, tremors, and bradykinesia. Other less common features include REM sleep behavior disorder, autonomic dysfunction, unexplained falls, depression, and sensitivity to antipsychotic medication. |
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What are the diagnostic criteria for Lewy body dementia |
Diagnostic CriteriaProbable LBD: progressive dementia + 2 main features .Possible LBD: progressive dementia + 1 main feature. TypesDementia 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. |
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How will you evaluate dementia of Lewy body |
No precise test can accurately diagnose LBD. Usually, a thorough workup, including the following, is useful to reach an alternative working diagnosis or rules out similar conditions: Detailed history and examinationAssessment of mental function Blood tests (e.g., vitamin B12 levels, chemistry panel, thyroid profile, syphilis, HIV) to rule out other causes of dementia Imaging studies (e.g., CT scan, MRI scan, SPECT scan, PET scan)Sleep evaluation for REM sleep behavior disorder |
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What is the place of CHF studies |
Cerebrospinal fluid examinations have no significant role when conducted in these patients |
