A Review of our Neuropathological Studies of Amyotrophic Lateral Sclerosis

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  • Koichi Okamoto
    Geriatrics Research Institute Department of Neurology, Gunma University Graduate School of Medicine

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  • 筋萎縮性側索硬化症の神経病理
  • 筋萎縮性側索硬化症の神経病理 －我々の研究を中心に－
  • キン イシュクセイ ソクサク コウカショウ ノ シンケイ ビョウリ : ワレワレ ノ ケンキュウ オ チュウシン ニ
  • - 我々の研究を中心に -

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Abstract

Our neuropathological studies on amyotrophic lateral sclerosis (ALS), especially about Bunina bodies, fragmentation of Golgi apparatus and cortical ubiquitin-positive and tau-negative neuronal inclusions, are reviewed in this paper. Bunina bodies, which are small eosinophilic intraneuronal inclusions in the remaining lower motor neurons, are generally considered to be a specific pathologic hallmark of ALS. At present, only three proteins (cystatin C, transferrin and peripherin) have been shown to be present in Bunina bodies. Fragmentation of Golgi apparatus was frequently observed in neurons containing Bunina bodies and TDP-43 positive inclusions. In 1991, new ubiquitin-positive and tau-negative inclusions were first reported in extra-motor neurons of hippocampal granular cell layers and frontal and temporal cortices. These inclusions were more frequently observed in ALS patients with dementia. In 2006, TDP-43 has been identified as a major component of the inclusions.

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