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. 2019 Oct 1;78(10):891-899.
doi: 10.1093/jnen/nlz080.

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

Charles H Adler  1 , Thomas G Beach  2 , Nan Zhang  3 , Holly A Shill  4 , Erika Driver-Dunckley  1 , John N Caviness  1 , Shyamal H Mehta  1 , Marwan N Sabbagh  5 , Geidy E Serrano  2 , Lucia I Sue  2 , Christine M Belden  6 , Jessica Powell  6 , Sandra A Jacobson  7 , Edward Zamrini  6 , David Shprecher  6 , Kathryn J Davis  6 , Brittany N Dugger  8 , Joseph G Hentz  3
Affiliations

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

Charles H Adler et al. J Neuropathol Exp Neurol. .

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: Braak staging system; Dementia with Lewy bodies; Incidental Lewy body disease; Lewy body; Parkinson disease; Unified Staging System for Lewy Body Disorders; a-Synuclein.

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Figures

FIGURE 1.
FIGURE 1.
Graphic depiction of the Unified Staging System for Lewy Body Disorders. Red is olfactory bulb and tract; orange is brainstem nuclei-substantia nigra, dorsal motor nucleus of the vagus, and locus coeruleus; blue is limbic regions-amygdala and cingulate cortex; and green is neocortex.
FIGURE 2.
FIGURE 2.
Correlation of USSLB stages with UPDRS parts II and III total scores, timed tap scores, Hoehn and Yahr staging scale, Epworth Sleepiness Scale scores, MMSE, SCOPA-Aut, and the UPSIT for the 145 cases without concomitant AD. Data displayed as mean ± standard error of the mean (SEM).
FIGURE 3.
FIGURE 3.
The percentage of 145 cases, without concomitant AD, at each USSLB stage with a UPDRS part II (handwriting, speech, cutting food, and walking) or part III (finger taps, rigidity, gait, hand movements, leg agility) score of ≥2.
See this image and copyright information in PMC

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