Diffuse Lewy Body Disease

• A form of dementia involving a characteristic protein aggregate which make up Lewy bodies in the brainstem (as in idiopathic Parkinson's disease), but also distributed throughout the cerebral cortex, diencephalon and limbic structures.
• Lewy Bodies are made from an abnormaql accumulation of alpha-synuclein
• Only rare reports of familial DLB; no genetic loci have been identified

Clinical Features

• Include a combination of parkinsonism and dementia. There is some overlap with Alzheimer's Disease (AD) pathology since some patients have a mix of diffuse cortical Lewy bodies and more typical Alzheimer's type changes. The clinical features however differ from AD.
• slowly progressive cognitive decline
• fluctuating cognitive function/alertness
• Recurrent visual hallucinations
• Parkinsonism
• Neuroleptic sensitivity
• Repeated Falls, Syncope and Loss of Consciousness
• In a SPEC scan, the occipital lobe is seen to be hypoperfused, whereas in Parkinson's disease, it is not