雑誌文献を検索します。書籍を検索する際には「書籍検索」を選択してください。

検索

書誌情報 詳細検索 by 医中誌

Japanese

Hyperbranching Axons Template α-Synuclein Deposits and Non-Localizing Clinical Manifestations of Parkinson Disease Toshiki Uchihara 1 1Laboratory of Structural Neuropathology, Tokyo Metropolitan Institute of Medical Science Keyword: structural template , distal axons , hyperbranching , focal Lewy body disease pp.1459-1475
Published Date 2013/12/1
DOI https://doi.org/10.11477/mf.1416101666
  • Abstract
  • Look Inside
  • Reference

Abstract

 The "proteinopathy" hypothesis, which states that pathological inclusions result in neuronal death, is classically invoked to explain neurodegeneration. In this review on α-synuclein (αS), attention is shifted to the distal axons, where αS deposits earlier than in the cytoplasmic formation of Lewy bodies (LBs). Because LBs are preferentially formed in axons with abundant branching, hyperbranching may accentuate axonal degeneration and αS deposition in their distal ends. This hyperbranching may also explain why motor/non-motor symptoms of Parkinson disease (PD) are so generalized and diffuse with barely any localizing value. Such underlying structure templates both the distal-dominant degeneration with αS deposition, and the non-localizing nature of clinical manifestations of PD, and as such can be considered a "structural template" thereof. The evolution of PD symptoms can be highly variable, ranging from restricted LB lesions and corresponding, long-lived, symptoms (pure autonomic failure, cardiac denervation, essential tremor, and REM-related behavioral disorders, which may be collectively called "focal LB disease") to the more haphazard appearance of dementia or anosmia without developing parkinsonism or brainstem lesions. This variability is better explained by the parallel involvement of multiple systems with hyperbranching axons rather than the stereotyped upward spread of LB in the brainstem. Awareness of presynaptic dysfunctions of these hyperbranching systems may enhance the sensitivity and specificity of clinical diagnoses of PD for earlier therapeutic intervention.


Copyright © 2013, Igaku-Shoin Ltd. All rights reserved.

基本情報

電子版ISSN 1344-8129 印刷版ISSN 1881-6096 医学書院

関連文献

もっと見る

文献を共有