The spectrum of Lewy body disease

Lewy bodies probably do not occur in normal ageing. Their presence indicates neurological disease, the clinical presentation varying according to the site of Lewy body formation and associated neuronal loss.(5) Three main clinicopathological syndromes have been described.

• Parkinson's disease, an extrapyramidal movement disorder—associated with degeneration of subcortical neurones, particularly in substantia nigra.

• Dementia with Lewy bodies, a dementing disorder with prominent neuropsychiatric features—associated with degeneration of cortical neurones, particularly in frontal, anterior cingulate, insular, and temporal regions.

• Primary autonomic failure with syncope and orthostatic hypotension—associated with degeneration of sympathetic neurones in spinal cord.

In clinical practice, elderly patients often have heterogenous combinations of parkinsonism, dementia, and autonomic failure, reflecting pathological involvement at multiple locations.

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