Unified staging system for Lewy body disorders: Correlation with nigrostriatal degeneration, cognitive impairment and motor dysfunction

Thomas G. Beach, Charles H. Adler, LihFen F. Lue, Lucia I. Sue, Jyothi Bachalakuri, Jonette Henry-Watson, Jeanne Sasse, Sarah Boyer, Scophil Shirohi, Reed Brooks, Jennifer Eschbacher, Charles L. White, Haru Akiyama, John Caviness, Holly A. Shill, Donald J. Connor, Marwan N. Sabbagh, Douglas G. Walker

Research output: Contribution to journalArticle

316 Scopus citations

Abstract

The two current major staging systems in use for Lewy body disorders fail to classify up to 50% of subjects. Both systems do not allow for large numbers of subjects who have Lewy-type α-synucleinopathy (LTS) confined to the olfactory bulb or who pass through a limbic-predominant pathway that at least initially bypasses the brainstem. The results of the current study, based on examination of a standard set of ten brain regions from 417 subjects stained immunohistochemically for α-synuclein, suggest a new staging system that, in this study, allows for the classification of all subjects with Lewy body disorders. The autopsied subjects included elderly subjects with Parkinson's disease, dementia with Lewy bodies, incidental Lewy body disease and Alzheimer's disease with Lewy bodies, as well as comparison groups without Lewy bodies. All subjects were classifiable into one of the following stages: I. Olfactory Bulb Only; IIa Brainstem Predominant; IIb Limbic Predominant; III Brainstem and Limbic; IV Neocortical. Progression of subjects through these stages was accompanied by a generally stepwise worsening in terms of striatal tyrosine hydroxylase concentration, substantia nigra pigmented neuron loss score, Mini Mental State Examination score and score on the Unified Parkinson's Disease Rating Scale Part 3. Additionally, there were significant correlations between these measures and LTS density scores. It is suggested that the proposed staging system would improve on its predecessors by allowing classification of a much greater proportion of cases.

Original languageEnglish (US)
Pages (from-to)613-634
Number of pages22
JournalActa Neuropathologica
Volume117
Issue number6
DOIs
StatePublished - Apr 28 2009

Keywords

  • Alzheimer's disease
  • Amgydala
  • Brain stem
  • Dementia with Lewy bodies
  • Incidental Lewy bodies
  • Limbic
  • Neocortex
  • Olfactory bulb
  • Parkinson's disease
  • Parkinsonism
  • α-synuclein

ASJC Scopus subject areas

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

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    Beach, T. G., Adler, C. H., Lue, L. F., Sue, L. I., Bachalakuri, J., Henry-Watson, J., Sasse, J., Boyer, S., Shirohi, S., Brooks, R., Eschbacher, J., White, C. L., Akiyama, H., Caviness, J., Shill, H. A., Connor, D. J., Sabbagh, M. N., & Walker, D. G. (2009). Unified staging system for Lewy body disorders: Correlation with nigrostriatal degeneration, cognitive impairment and motor dysfunction. Acta Neuropathologica, 117(6), 613-634. https://doi.org/10.1007/s00401-009-0538-8