Lewy Body Disease

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Clinically Relevant Anatomy

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Mechanism of Injury / Pathological Process

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Clinical Presentation

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Diagnostic Procedures

Lewy Body Disease (LBD) can have two forms of clinical diagnoses which are dementia with Lewy bodies and Parkinson’s disease dementia . 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 .

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 . But overall, when individuals progress and develop more symptoms of LBD then it becomes easier to diagnosis.
To diagnosis the type of LBD, physical and neurological evaluations are used and a thorough patient interview is done. For example,

• 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 . 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 . There can also be volume loss in the hippocampus, amygdala, and the basal ganglia specifically the substantia nigra . In particular with Parkinson disease dementia, there can be atrophy of the nirgrostriatal system and also compromised dopamine transporters .

Clinical Diagnosis of DLB:
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 If two are present, a higher case can be made for a patient with DLB.

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 . The changes can occur from day to day and can vary in amplitude for a patient experiencing these cognitive changes.

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 . 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 .

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 .

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 .
• Unexplained loss of consciousness
• Falls and syncope
• Severe autonomic dysfunction
• Systematized delusions

Clinical Diagnosis of PDD:
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 . 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 . The tests to evaluate the cognitive domains are described in Table 2 in .

Outcome Measures

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Management / Interventions

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Differential Diagnosis

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 . 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 .

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 . With frontotemporal dementia, the common symptoms are changes in personality and behaviour, language and speech impairments and movement changes .

Key Evidence

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Resources

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