Acta Neuropathologica

, Volume 120, Issue 1, pp 1–12 | Cite as

Involvement of the peripheral nervous system in synucleinopathies, tauopathies and other neurodegenerative proteinopathies of the brain

  • Koichi Wakabayashi
  • Fumiaki Mori
  • Kunikazu Tanji
  • Satoshi Orimo
  • Hitoshi Takahashi


Involvement of the peripheral nervous system (PNS) is relatively common in some neurodegenerative proteinopathies of the brain and may be pathogenetically and diagnostically important. In Parkinson’s disease, neuronal α-synuclein aggregates are distributed throughout the nervous system, including the central nervous system (CNS), sympathetic ganglia, enteric nervous system, cardiac and pelvic plexuses, submandibular gland, adrenal medulla and skin. The pathological process may target the PNS and CNS at the same time. In multiple system atrophy, numerous glial cytoplasmic inclusions composed of filamentous α-synuclein are widely distributed in the CNS, while α-synuclein accumulation is minimal in the sympathetic ganglia and is restricted to neurons. Neurofibrillary tangles can occur in the sympathetic and spinal ganglia in tauopathy, although they appear to develop independently of cerebral Alzheimer’s disease pathology. In amyotrophic lateral sclerosis, neuronal loss with TDP-43-positive neuronal cytoplasmic inclusions in the spinal ganglia is more frequent than previously thought. Peripheral ganglia and visceral organs are also involved in polyglutamine diseases. Further elucidation and characterization of PNS lesions will have implications for intravital biopsy diagnosis in neurodegenerative proteinopathy, particularly in Parkinson’s disease.


α-Synuclein Enteric nervous system Lewy body Parkinson’s disease Tauopathy TDP-43 


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Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Koichi Wakabayashi
    • 1
  • Fumiaki Mori
    • 1
  • Kunikazu Tanji
    • 1
  • Satoshi Orimo
    • 2
  • Hitoshi Takahashi
    • 3
  1. 1.Department of NeuropathologyInstitute of Brain Science, Hirosaki University Graduate School of MedicineHirosakiJapan
  2. 2.Department of NeurologyKanto Central HospitalTokyoJapan
  3. 3.Department of PathologyBrain Research Institute, University of NiigataNiigataJapan

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