title: Unified staging system for Lewy body disorders: correlation with nigrostriatal degeneration, cognitive impairment and motor dysfunction.
abstract: The two current major staging systems in use for Lewy body disorders fail to classify up to 50% of subjects. Both systems do not allow for large numbers of subjects who have Lewy-type alpha-synucleinopathy (LTS) confined to the olfactory bulb or who pass through a limbic-predominant pathway that at least initially bypasses the brainstem. The results of the current study, based on examination of a standard set of ten brain regions from 417 subjects stained immunohistochemically for alpha-synuclein, suggest a new staging system that, in this study, allows for the classification of all subjects with Lewy body disorders. The autopsied subjects included elderly subjects with Parkinson's disease, dementia with Lewy bodies, incidental Lewy body disease and Alzheimer's disease with Lewy bodies, as well as comparison groups without Lewy bodies. All subjects were classifiable into one of the following stages: I. Olfactory Bulb Only; IIa Brainstem Predominant; IIb Limbic Predominant; III Brainstem and Limbic; IV Neocortical. Progression of subjects through these stages was accompanied by a generally stepwise worsening in terms of striatal tyrosine hydroxylase concentration, substantia nigra pigmented neuron loss score, Mini Mental State Examination score and score on the Unified Parkinson's Disease Rating Scale Part 3. Additionally, there were significant correlations between these measures and LTS density scores. It is suggested that the proposed staging system would improve on its predecessors by allowing classification of a much greater proportion of cases.
authors.affiliation: Sun Health Research Institute, Sun City, AZ 85351, USA. thomas.beach@bannerhealth.com
authors.forename: Thomas G
authors.initials: TG
authors.lastname: Beach
authors.forename: Charles H
authors.initials: CH
authors.lastname: Adler
authors.forename: LihFen
authors.initials: L
authors.lastname: Lue
authors.forename: Lucia I
authors.initials: LI
authors.lastname: Sue
authors.forename: Jyothi
authors.initials: J
authors.lastname: Bachalakuri
authors.forename: Jonette
authors.initials: J
authors.lastname: Henry-Watson
authors.forename: Jeanne
authors.initials: J
authors.lastname: Sasse
authors.forename: Sarah
authors.initials: S
authors.lastname: Boyer
authors.forename: Scophil
authors.initials: S
authors.lastname: Shirohi
authors.forename: Reed
authors.initials: R
authors.lastname: Brooks
authors.forename: Jennifer
authors.initials: J
authors.lastname: Eschbacher
authors.forename: Charles L
authors.initials: CL
authors.lastname: White
authors.forename: Haru
authors.initials: H
authors.lastname: Akiyama
authors.forename: John
authors.initials: J
authors.lastname: Caviness
authors.forename: Holly A
authors.initials: HA
authors.lastname: Shill
authors.forename: Donald J
authors.initials: DJ
authors.lastname: Connor
authors.forename: Marwan N
authors.initials: MN
authors.lastname: Sabbagh
authors.forename: Douglas G
authors.initials: DG
authors.lastname: Walker
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000375:Aging; D000544:Alzheimer Disease / Q000503:physiopathology; D001057:Apolipoproteins E / Q000235:genetics; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D003072:Cognition Disorders / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D020961:Lewy Body Disease / Q000150:complications / Q000473:pathology* / Q000503:physiopathology*; D008297:Male; D009069:Movement Disorders / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D009410:Nerve Degeneration / Q000473:pathology; D019636:Neurodegenerative Diseases / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D010300:Parkinson Disease / Q000503:physiopathology; D012720:Severity of Illness Index; D051844:alpha-Synuclein / Q000378:metabolism
chemical_list: D001057:Apolipoproteins E; D051844:alpha-Synuclein