Dementia with Lewy bodies

Dementia with Lewy bodies is a neurodegenerative disease marked by cognitive decline, recurrent visual hallucinations, fluctuating attention, and parkinsonism. Its symptoms reflect abnormal alpha-synuclein deposits in the brain.

Lewy body: An abnormal intracellular inclusion containing aggregated alpha-synuclein, found in neurons in several neurodegenerative diseases. These inclusions give the disease its name and mark its characteristic pathology.
Visual hallucination: A perception of a visual object or event without a corresponding external stimulus. Recurrent, often detailed visual hallucinations are a core clinical feature.
Parkinson's disease dementia: Dementia developing in a person with established Parkinson's disease. The one-year rule distinguishes this diagnosis from dementia with Lewy bodies.
McKeith criteria: Consensus clinical criteria for diagnosing dementia with Lewy bodies using core features and indicative biomarkers. They organize the clinical features and tests used to establish the diagnosis.
Friedrich Lewy: A German-American neurologist who described neuronal inclusions later called Lewy bodies. His pathological observations supplied the eponym for the disease's defining inclusions.
Alpha-synuclein: A presynaptic neuronal protein that can misfold and aggregate in synucleinopathies. Misfolded alpha-synuclein is the principal protein in Lewy bodies.
Cognitive fluctuation: Marked variation over time in alertness, attention, or cognitive performance. Pronounced shifts in attention and alertness are characteristic of the disease.
Alzheimer's disease: A progressive neurodegenerative disease and the most common cause of dementia. Its typical memory-led course differs from the early hallucinations and fluctuations often seen here.
Dopamine transporter scan: A nuclear imaging test that measures presynaptic dopamine transporter availability in the striatum. Reduced striatal uptake can support the diagnosis when clinical features are uncertain.
Kenji Kosaka: A Japanese neuropathologist who characterized diffuse Lewy body disease as a distinct clinicopathological entity. His work helped establish diffuse Lewy body disease as a distinct form of dementia.

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