DLB and PDD boundary issues
Diagnosis, treatment, molecular pathology, and biomarkers
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Abstract
For more than a decade, researchers have refined criteria for the diagnosis of dementia with Lewy bodies (DLB) and at the same time have recognized that cognitive impairment and dementia occur commonly in patients with Parkinson disease (PD). This article addresses the relationship between DLB, PD, and PD with dementia (PDD). The authors agreed to endorse “Lewy body disorders” as the umbrella term for PD, PDD, and DLB, to promote the continued practical use of these three clinical terms, and to encourage efforts at drug discovery that target the mechanisms of neurodegeneration shared by these disorders of α-synuclein metabolism. We concluded that the differing temporal sequence of symptoms and clinical features of PDD and DLB justify distinguishing these disorders. However, a single Lewy body disorder model was deemed more useful for studying disease pathogenesis because abnormal neuronal α-synuclein inclusions are the defining pathologic process common to both PDD and DLB. There was consensus that improved understanding of the pathobiology of α-synuclein should be a major focus of efforts to develop new disease-modifying therapies for these disorders. The group agreed on four important priorities: 1) continued communication between experts who specialize in PDD or DLB; 2) initiation of prospective validation studies with autopsy confirmation of DLB and PDD; 3) development of practical biomarkers for α-synuclein pathologies; 4) accelerated efforts to find more effective treatments for these diseases.
Footnotes
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↵*Author affiliations are listed in the appendix.
Supported by Forest Laboratories, Inc.; Lehigh Valley PD Support Group; The Movement Disorder Society; National Parkinson Foundation, Inc.; Novartis Pharmaceuticals Corporations; PD Foundation, Inc.; Schwarz Pharma, Inc.; NINDS/NIH (conference grant NS054546-01 and grants AG09215 and AG10124); the intramural program of the National Institute on Aging, NIH, DHHS.
Disclosure: Research support/grants more than $10,000/year: Duda, National Parkinson's Foundation; Emre, Novartis; Fahn, NINDS, Parkinson's Disease Foundation; Lippa, NINDS; Tanner, NINDS and National Parkinson's Foundation; Wszolek, NINDS. Honoraria less than $10,000/year: Galvin, Forest; Leverenz, Forest; Lippa, Novartis and Pfizer. Honoraria more than $10,000/year: Leverenz, Novartis; Olanow, Novartis. Given expert testimony related to the subject of this study, less than $10,000: Hurtig.
Received June 23, 2006. Accepted in final form November 7, 2006.
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