Unified Staging System for Lewy Body Disorders: Clinicopathologic Correlations and Comparison to Braak Staging

• Published in: Journal of neuropathology and experimental neurology Vol. 78; no. 10; pp. 891 - 899
• Main Authors: Adler, Charles H, Beach, Thomas G, Zhang, Nan, Shill, Holly A, Driver-Dunckley, Erika, Caviness, John N, Mehta, Shyamal H, Sabbagh, Marwan N, Serrano, Geidy E, Sue, Lucia I, Belden, Christine M, Powell, Jessica, Jacobson, Sandra A, Zamrini, Edward, Shprecher, David, Davis, Kathryn J, Dugger, Brittany N, Hentz, Joseph G
• Format: Journal Article
• Language: English
• Published: England Oxford University Press 01.10.2019; by American Association of Neuropathologists, Inc
• Subjects: Aged; Aged, 80 and over; alpha-Synuclein - metabolism; Brain - metabolism; Brain - pathology; Brain diseases; Cognitive ability; Cognitive Dysfunction - diagnosis; Cognitive Dysfunction - metabolism; Cognitive Dysfunction - pathology; Diagnosis; Female; Humans; Lewy Bodies - metabolism; Lewy Bodies - pathology; Lewy body disease; Lewy Body Disease - diagnosis; Lewy Body Disease - metabolism; Lewy Body Disease - pathology; Male; Methods; Neurologic examination; Olfaction disorders; Original; Parkinson's disease; Severity of Illness Index; United States; a-Synuclein; Braak staging system; Incidental Lewy body disease; Parkinson disease; Unified Staging System for Lewy Body Disorders; Dementia with Lewy bodies; Lewy body
• ISSN: 0022-3069; 1554-6578; 1554-6578
• DOI: 10.1093/jnen/nlz080

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.