title: Multiple organ involvement by alpha-synuclein pathology in Lewy body disorders.
abstract: Lewy body (LB) diseases are characterized by alpha-synuclein (AS) aggregates in the central nervous system (CNS). Involvement of the peripheral autonomic nervous system (pANS) is increasingly recognized, although less studied. The aim of this study was to systematically analyze the distribution and severity of AS pathology in the CNS and pANS. Detailed postmortem histopathological study of brain and peripheral tissues from 28 brain bank donors (10 with Parkinson's disease [PD], 5 with dementia with LB [DLB], and 13 with non-LB diseases including atypical parkinsonism and non-LB dementia). AS aggregates were found in the pANS of all 15 LB disease cases (PD, DLB) in stellate and sympathetic ganglia (100%), vagus nerve (86.7%), gastrointestinal tract (86.7%), adrenal gland and/or surrounding fat (53.3%), heart (100%), and genitourinary tract (13.3%), as well as in 1 case of incidental Lewy body disease (iLBD). A craniocaudal gradient of AS burden in sympathetic chain and gastrointestinal tract was observed. DLB cases showed higher amounts of CNS AS aggregates than PD cases, but this was not the case in the pANS. No pANS AS aggregates were detected in Alzheimer's disease (AD) cases with or without CNS AS aggregates. All pathologically confirmed LB disease cases including 1 case of iLBD had AS aggregates in the pANS with a craniocaudal gradient of pathology burden in sympathetic chain and gastrointestinal tract. AS was not detected in the pANS of any AD case. These findings may help in the search of peripheral AS aggregates in vivo for the early diagnosis of PD.
authors.affiliation: Neurological Tissue Bank of the Biobanc-Hospital Clinic-Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
authors.forename: Ellen
authors.initials: E
authors.lastname: Gelpi
authors.forename: Judith
authors.initials: J
authors.lastname: Navarro-Otano
authors.forename: Eduardo
authors.initials: E
authors.lastname: Tolosa
authors.forename: Carles
authors.initials: C
authors.lastname: Gaig
authors.forename: Yaroslau
authors.initials: Y
authors.lastname: Compta
authors.forename: María Jesús
authors.initials: MJ
authors.lastname: Rey
authors.forename: Maria José
authors.initials: MJ
authors.lastname: Martí
authors.forename: Isabel
authors.initials: I
authors.lastname: Hernández
authors.forename: Francesc
authors.initials: F
authors.lastname: Valldeoriola
authors.forename: Ramon
authors.initials: R
authors.lastname: Reñé
authors.forename: Teresa
authors.initials: T
authors.lastname: Ribalta
mesh_terms: D000311:Adrenal Glands / Q000378:metabolism / Q000473:pathology; D000368:Aged; D000369:Aged, 80 and over; D001341:Autonomic Nervous System / Q000378:metabolism* / Q000473:pathology; D002490:Central Nervous System / Q000378:metabolism / Q000473:pathology; D005260:Female; D041981:Gastrointestinal Tract / Q000378:metabolism / Q000473:pathology; D006801:Humans; D020961:Lewy Body Disease / Q000378:metabolism / Q000473:pathology*; D008297:Male; D009206:Myocardium / Q000378:metabolism / Q000473:pathology; D012189:Retrospective Studies; D014566:Urogenital System / Q000378:metabolism / Q000473:pathology; D051844:alpha-Synuclein / Q000378:metabolism*
chemical_list: D051844:alpha-Synuclein
keywords: Parkinson's disease; Parkinson's disease with dementia; alpha-synuclein; autonomic diseases; dementia with Lewy bodies