Diffuse Lewy body disease – a clinical syndrome or a disease entity?
- Reiner Benecke MD
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Most clinicians and researchers still accept diffuse Lewy body disease (DLBD) as a clinicopathological entity. Dementia with fluctuating cognitive deficits, a parkinsonian syndrome, and visual hallucinations are the core symptoms of this proposed disease entity. From a neuropathological point of view, many examples of patients with progressive dementia showing evidence of extensive Lewy body formation in the cerebral cortex together with the occurrence of Lewy bodies in substantia nigra and locus coeruleus have been identified. Confusingly, a large majority of cases showing typical features of DLBD also present with an Alzheimer pathology in the hippocampus and cerebral cortex. It is far from clear that DLBD represents a specific disease entity rather an intermediate variant between Alzheimer disease and idiopathic parkinsonian syndromes. Nevertheless, from a clinical point of view it may be of importance to characterize patients with a symptomatology of DLBD because important management issues such as avoidance of severe neuroleptic sensitivity reactions, dopaminergic antiparkinsonian treatment and a beneficial response to cholinesterase inhibitors can be applied.
- Diffuse Lewy body disease – a clinical syndrome or a disease entity?
Journal of Neurology
Volume 250, Issue 1 Supplement, pp i39-i42
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- Key words diffuse Lewy body disease
- Alzheimer's disease
- idiopathic parkinsonian syndromes
- neuroleptic therapy
- cholinesterase inhibitors
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- Reiner Benecke MD (A1)
- Author Affiliations
- A1. Department of Neurology, University of Rostock, Gehlsheimer Str. 20, 18147, Rostock, Germany