From the Guidelines
Lewy bodies are primarily associated with Parkinson's disease, Dementia with Lewy Bodies (DLB), and can also be found in multiple system atrophy (MSA) and sometimes in Alzheimer's disease as a co-pathology. These diseases are characterized by the abnormal accumulation of alpha-synuclein, a protein that misfolds and aggregates, leading to the formation of Lewy bodies. The presence of Lewy bodies in the brain stem typically causes movement disorders characteristic of Parkinson's disease, while their spread to the cerebral cortex leads to cognitive impairment and hallucinations seen in DLB 1.
Some key points to consider:
- Parkinson's disease is a neurodegenerative disease and movement disorder characterized by progressive degeneration of the dopaminergic neurons in the substantia nigra/striatum, with Lewy bodies composed of alpha-synuclein and ubiquitin 1.
- Dementia with Lewy Bodies (DLB) is a neurodegenerative disorder characterized by the presence of Lewy bodies in the cerebral cortex, leading to cognitive impairment and hallucinations 1.
- Multiple system atrophy (MSA) is a synucleinopathy characterized by the accumulation of alpha-synuclein in oligodendroglia, leading to a range of symptoms including Parkinsonian features, ataxia, and dysautonomia 1.
- Alzheimer's disease can also have Lewy bodies as a co-pathology, which can affect the clinical trajectory and phenotypes of the disease 1.
It's essential to recognize that these diseases exist on a spectrum of related neurodegenerative conditions, sometimes called "synucleinopathies," and that the presence of Lewy bodies can be a common feature across these conditions 1. Understanding the relationship between Lewy bodies and these diseases is crucial for accurate diagnosis and treatment.
From the Research
Diseases with Lewy Bodies
The following diseases have been associated with Lewy bodies:
- Dementia with Lewy bodies (DLB) 2, 3, 4, 5, 6
- Parkinson's disease dementia (PDD) 2, 3, 4, 5
- Incidental Lewy body disease 5
- Parkinson's disease (PD) 4, 5
- Alzheimer's disease with Lewy bodies (DLB-AD) 5
Characteristics of Lewy Body Diseases
Lewy body diseases are characterized by:
- Abnormal eosinophilic A-synuclein neural inclusions (Lewy bodies) in the brain 6
- Cognitive impairment 2, 3, 4, 5
- Parkinsonism 2, 3, 4, 5
- Visual hallucinations 2, 3, 4, 5
- Fluctuating attention 2, 3, 4, 5
- Autonomic dysfunction 3
- Sleep disorders 3
- Behavioral symptoms such as hallucinations and delusions 3
Clinical Differentiation
Clinical differentiation between DLB and PDD is based on the time of onset of motor and cognitive symptoms: