@article{05607a79b52641f6bf32eeeb5cc28a7c,
title = "Unified staging system for Lewy body disorders: Clinicopathologic correlations and comparison to Braak staging",
abstract = "This study was designed to correlate clinical findings with the extent of pathologic a-synuclein (aSyn) in the brain using the Unified Staging System for Lewy Body disorders (USSLB). Data from 280 cases from the Arizona Study of Aging and Neurodegenerative Disorders are presented. Each case had a complete USSLB staging and at least 1 full research clinical assessment, including subspecialty neurologist-administered movement and cognitive evaluation. Of the 280, 25.7% were cognitively normal, 8.6% had mild cognitive impairment, and 65.7% had dementia. All cases could be categorized into 1 of 5 USSLB stages (8.6% stage I—olfactory bulb only; 15.4% IIa—brainstem predominant; 13.6% IIb—limbic predominant; 31.8% III—brainstem and limbic; and 30.7% IV—neocortical) yet using the Braak staging system 70 cases (25.3%) could not be classified. Those with USSLB stages III and IV died at a younger age. Multiple measures of motor parkinsonism, cognitive impairment, hyposmia, and probable RBD were significantly correlated with increasing USSLB stage. We conclude that the USSLB is the most comprehensive staging system for all Lewy body disorders and allows for categorization and ranking of all brains with significant correlations to many motor and nonmotor clinical signs and symptoms.",
keywords = "A-Synuclein, Braak staging system, Dementia with Lewy bodies, Incidental Lewy body disease, Lewy body, Parkinson disease, Unified Staging System for Lewy Body Disorders",
author = "Adler, {Charles H.} and Beach, {Thomas G.} and Nan Zhang and Shill, {Holly A.} and Erika Driver-Dunckley and Caviness, {John N.} and Mehta, {Shyamal H.} and Sabbagh, {Marwan N.} and Serrano, {Geidy E.} and Sue, {Lucia I.} and Belden, {Christine M.} and Jessica Powell and Jacobson, {Sandra A.} and Edward Zamrini and David Shprecher and Davis, {Kathryn J.} and Dugger, {Brittany N.} and Hentz, {Joseph G.}",
note = "Funding Information: From the Parkinson{\textquoteright}s Disease and Movement Disorders Center, Department of Neurology, Mayo Clinic, Scottsdale, Arizona (CHA, ED-D, JNC, SHM); Civin Laboratory for Neuropathology, Banner Sun Health Re-search Institute, Sun City, Arizona (TGB, GES, LIS); Department of Bio-statistics, Mayo Clinic, Scottsdale, Arizona (NZ, JGH); Barrow Neurological Institute, Phoenix, Arizona (HAS); Cleveland Clinic Lou Ruvo Center for Brain Health, Las Vegas, Nevada (MNS); Cleo Roberts Center, Banner Sun Health Research Institute, Sun City, Arizona (CMB, JP, EZ, DS, KJD); University of Arizona College of Medicine, Phoenix, Arizona (SAJ); and Department of Pathology and Laboratory Medicine, University of California Davis, Sacramento, California (BND) Send correspondence to: Charles H. Adler, MD, PhD, Department of Neurol-ogy, Mayo Clinic Arizona, 13400 E. Shea Blvd, Scottsdale, AZ 85259; E-mail: cadler@mayo.edu This study was funded by the National Institute of Neurological Disorders and Stroke (U24 NS072026 National Brain and Tissue Resource for Par-kinson{\textquoteright}s Disease and Related Disorders), the National Institute on Aging (P30 AG19610 Arizona Alzheimer{\textquoteright}s Disease Core Center), the Arizona Department of Health Services (contract 211002, Arizona Alzheimer{\textquoteright}s Research Center), the Arizona Biomedical Research Commission (con-tracts 4001, 0011, 05-901, and 1001 to the Arizona Parkinson{\textquoteright}s Disease Consortium), the Michael J. Fox Foundation for Parkinson{\textquoteright}s Research, and Mayo Clinic Foundation. Publisher Copyright: {\textcopyright} 2019 American Association of Neuropathologists, Inc. All rights reserved.",
year = "2019",
month = oct,
day = "1",
doi = "10.1093/jnen/nlz080",
language = "English (US)",
volume = "78",
pages = "891--899",
journal = "Journal of neuropathology and experimental neurology",
issn = "0022-3069",
number = "10",
}