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

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

Research output: Contribution to journalArticle

1 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)891-899
Number of pages9
JournalJournal of neuropathology and experimental neurology
Volume78
Issue number10
DOIs
StatePublished - Oct 1 2019

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

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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    Adler, C. H., Beach, T. G., Zhang, N., Shill, H. A., Driver-Dunckley, E., Caviness, J. N., Mehta, S. H., Sabbagh, M. N., Serrano, G. E., Sue, L. I., Belden, C. M., Powell, J., Jacobson, S. A., Zamrini, E., Shprecher, D., Davis, K. J., Dugger, B., & Hentz, J. G. (2019). Unified Staging System for Lewy Body Disorders: Clinicopathologic Correlations and Comparison to Braak Staging. Journal of neuropathology and experimental neurology, 78(10), 891-899. https://doi.org/10.1093/jnen/nlz080