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

286 Citations (Scopus)

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 - 2009

Fingerprint

Lewy Bodies
Brain Stem
Lewy Body Disease
Olfactory Bulb
Parkinson Disease
Synucleins
Corpus Striatum
Tyrosine 3-Monooxygenase
Substantia Nigra
Alzheimer Disease
Neurons
Cognitive Dysfunction
Brain

Keywords

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

ASJC Scopus subject areas

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

Cite this

Beach, T. G., Adler, C. H., Lue, L. F., Sue, L. I., Bachalakuri, J., Henry-Watson, J., ... 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

Unified staging system for Lewy body disorders : Correlation with nigrostriatal degeneration, cognitive impairment and motor dysfunction. / Beach, Thomas G.; Adler, Charles H.; Lue, LihFen F.; Sue, Lucia I.; Bachalakuri, Jyothi; Henry-Watson, Jonette; Sasse, Jeanne; Boyer, Sarah; Shirohi, Scophil; Brooks, Reed; Eschbacher, Jennifer; White, Charles L.; Akiyama, Haru; Caviness, John; Shill, Holly A.; Connor, Donald J.; Sabbagh, Marwan N.; Walker, Douglas G.

In: Acta Neuropathologica, Vol. 117, No. 6, 2009, p. 613-634.

Research output: Contribution to journalArticle

Beach, TG, Adler, CH, Lue, LF, Sue, LI, Bachalakuri, J, Henry-Watson, J, Sasse, J, Boyer, S, Shirohi, S, Brooks, R, Eschbacher, J, White, CL, Akiyama, H, Caviness, J, Shill, HA, Connor, DJ, Sabbagh, MN & Walker, DG 2009, 'Unified staging system for Lewy body disorders: Correlation with nigrostriatal degeneration, cognitive impairment and motor dysfunction', Acta Neuropathologica, vol. 117, no. 6, pp. 613-634. https://doi.org/10.1007/s00401-009-0538-8
Beach, Thomas G. ; Adler, Charles H. ; Lue, LihFen F. ; Sue, Lucia I. ; Bachalakuri, Jyothi ; Henry-Watson, Jonette ; Sasse, Jeanne ; Boyer, Sarah ; Shirohi, Scophil ; Brooks, Reed ; Eschbacher, Jennifer ; White, Charles L. ; Akiyama, Haru ; Caviness, John ; Shill, Holly A. ; Connor, Donald J. ; Sabbagh, Marwan N. ; Walker, Douglas G. / Unified staging system for Lewy body disorders : Correlation with nigrostriatal degeneration, cognitive impairment and motor dysfunction. In: Acta Neuropathologica. 2009 ; Vol. 117, No. 6. pp. 613-634.
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