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

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Abstract

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.

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Acknowledgments

This work has been supported by Grants-in-Aid 20300123 (K.W.), 20591361 (F.M.) and 20590335 (K.T.) for Scientific Research from the Ministry of Education, Culture, Sports, Science, and Technology, Japan, a Grant for Hirosaki University Institutional Research (K.W.), and a Grant-in-Aid for Studies on the Development of Diagnostic Technique and Therapies for Lewy Body Disease, the Ministry of Health, Labour and Welfare, Japan (K.W.).

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  1. Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki, 036-8562, Japan

    Koichi Wakabayashi, Fumiaki Mori & Kunikazu Tanji

  2. Department of Neurology, Kanto Central Hospital, Tokyo, Japan

    Satoshi Orimo

  3. Department of Pathology, Brain Research Institute, University of Niigata, Niigata, Japan

    Hitoshi Takahashi

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  1. Koichi Wakabayashi
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Correspondence to Koichi Wakabayashi.

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Wakabayashi, K., Mori, F., Tanji, K. et al. Involvement of the peripheral nervous system in synucleinopathies, tauopathies and other neurodegenerative proteinopathies of the brain. Acta Neuropathol 120, 1–12 (2010). https://doi.org/10.1007/s00401-010-0706-x

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