21. Parkinson's Disease

  1. Dennis W. Dickson MD2 and
  2. Roy O. Weller MD, PhD, FRCPath3
  1. Kurt A. Jellinger

Published Online: 21 SEP 2011

DOI: 10.1002/9781444341256.ch21

Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders, Second Edition

Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders, Second Edition

How to Cite

Jellinger, K. A. (2011) Parkinson's Disease, in Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders, Second Edition (eds D. W. Dickson and R. O. Weller), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444341256.ch21

Editor Information

  1. 2

    Departments of Pathology (Neuropathology) and Neuroscience Mayo Clinic, Jacksonville, FL, USA

  2. 3

    Clinical Neurosciences, University of Southampton School of Medicine, Southampton General Hospital, Southampton, UK

Author Information

  1. Institute of Clinical Neurobiology, Medical University Vienna, Vienna, Austria

Publication History

  1. Published Online: 21 SEP 2011
  2. Published Print: 2 SEP 2011

ISBN Information

Print ISBN: 9781405196932

Online ISBN: 9781444341256

SEARCH

Keywords:

  • α-synuclein;
  • clinical features;
  • differential diagnosis;
  • etiopathogenesis;
  • Lewy bodies;
  • neuropathology;
  • parkinsonism;
  • Parkinson's disease;
  • pathophysiology

Summary

Parkinson's disease is the most frequent neurodegenerative movement disorder, characterized by degeneration of the dopaminergic nigrostriatal and other neuronal networks caused by cell loss in the substantia nigra and many other neuronal systems, associated with widespread occurrence of intracytoplasmic Lewy bodies and dystrophic Lewy neurites mainly containing phosphorylated α-synuclein. The resulting striatal dopamine deficiency and other biochemical deficits cause the clinical picture of this multisystem disorder. For the diagnosis of definite Parkinson's disease, histopathological confirmation is required. The etiology of Parkinson's disease remains still elusive, but a complex combination of environmental factors, intrinsic cellular metabolic properties and susceptible genetic alleles has been assumed, and important genetic and pathological clues have been found. Two forms of Parkinson's disease are recognized: a “familial” or early-onset Parkinson's disease (<10% of all patients) in which gene mutations have been identified, and a sporadic late-onset Parkinson's disease (>85%) that does not appear to exhibit heredity, but familial components suggest genetic factors. Although genetic and experimental models have contributed to explore the pathomechanisms and treatment options of Parkinson's disease, there is still no optimal animal model of the disease, and the etiology of Parkinson's disease is still far from being elucidated.