Chapter

Parkinson’s Disease and Related Disorders

Volume 70 of the series Journal of Neural Transmission. Supplementa pp 99-103

Critical appraisal of brain pathology staging related to presymptomatic and symptomatic cases of sporadic Parkinson’s disease

  • G. M. HallidayAffiliated withPrince of Wales Medical Research Institute, University of New South Wales
  • , K. Del TrediciAffiliated withInstitute for Clinical Neuroanatomy, JW Goethe University Clinic
  • , H. BraakAffiliated withInstitute for Clinical Neuroanatomy, JW Goethe University Clinic

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Summary

Clinical Parkinson’s disease (PD) is a well-characterised syndrome that benefits significantly from dopamine replacement therapies. A staging procedure for sporadic PD pathology was developed by Braak et al. assuming that the abnormal deposition of α-synuclein indicates the intracellular process responsible for clinical PD. This paradigm has merit in corralling patients with similar cellular mechanisms together and determining the potential sequence of events that may herald the clinical syndrome. Progressive pathological stages were identified — 1) preclinical (stages 1–2), 2) early (stages 3–4, 35% with clinical PD) and 3) late (stages 5–6, 86% with clinical PD). However, preclinical versus early versus late-stage cases should on average be progressively older at the time of sampling, a feature not observed in the cohort analysed. In this cohort preclinical cases would have developed extremely late-onset PD compared with the other types of cases analysed. While the staging scheme is a valuable concept, further development is required.