Journal of Neurology

, Volume 249, Supplement 3, pp iii1–iii5

Staging of the intracerebral inclusion body pathology associated with idiopathic Parkinson's disease (preclinical and clinical stages)

  • Heiko Braak
  • Kelly Del Tredici
  • Hansjürgen Bratzke
  • John Hamm-Clement
  • Daniele Sandmann-Keil
  • Udo Rüb

DOI: 10.1007/s00415-002-1301-4

Cite this article as:
Braak, H., Del Tredici, K., Bratzke, H. et al. J Neurol (2002) 249: iii1. doi:10.1007/s00415-002-1301-4

Abstract.

The synucleinopathy known as idiopathic Parkinson's disease (IPD) is a multi-system disorder in the course of which only a few predisposed nerve cell types in specific regions of the human brain become progressively involved. The underlying neuropathological process (formation of proteinaceous intraneuronal inclusion bodies) intracerebrally begins in clearly defined induction sites and advances in a topographically predictable sequence. Components of the autonomic, limbic, and motor systems sustain especially heavy damage. During the presymptomatic stages 1 and 2, the IPD-related inclusion body pathology remains confined to the medulla oblongata and olfactory bulb. In stages 3 and 4, the substantia nigra and other nuclear grays of the midbrain and basal forebrain are the focus of initially subtle and, then, severe changes. The illness reaches its symptomatic phase. In end-stages 5 and 6, the pathological process encroaches upon the telencephalic cortex. IPD manifests itself in all of its dimensions, which under the influence of the supervening cortical pathology are subject to increasing complexity.

Key words α-synucleinIdiopathic Parkinson's diseaseLewy neurites/bodiesPathoarchitectonicsStaging

Copyright information

© Steinkopff Verlag 2002

Authors and Affiliations

  • Heiko Braak
    • 1
  • Kelly Del Tredici
    • 1
  • Hansjürgen Bratzke
    • 2
  • John Hamm-Clement
    • 1
  • Daniele Sandmann-Keil
    • 1
  • Udo Rüb
    • 1
  1. 1.Institute for Clinical Neuroanatomy, J. W. Goethe University, Theodor Stern Kai 7, 60590 Frankfurt/Main, Germany. Braak@em.uni-frankfurt.deDE
  2. 2.Institute for Forensic Medicine, J. W. Goethe University, Theodor Stern Kai 7, 60590 Frankfurt/Main, GermanyDE