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ALS in the Kii Peninsula of Japan, with Special Reference to Neurofibrillary Tangles and Aluminum
Author(s) -
Kihira Tameko,
Yoshida Sohei,
Mitani Kazuo,
Yasui Masayuki,
Yase Yoshiro
Publication year - 1993
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/j.1440-1789.1993.tb00222.x
Subject(s) - pathology , neurofibrillary tangle , amyotrophic lateral sclerosis , cortex (anatomy) , pathogenesis , french horn , senile plaques , anatomy , biology , medicine , alzheimer's disease , neuroscience , disease , psychology , pedagogy
Epidemiological and environmental findings of the foci of amyotrophi lateral sclerosis (ALS) in the Western Pacific indicate a possible contributing role of metals, especially aluminum, in the pathogenesis. Al is also a candidate as a causative agent in the formation of neurofibrillary tangles (NFTs), which are characteristic of Guam ALS. To elucidate the pathogenesis of ALS, we conducted neuropathological examination and metal analysis, with special reference to NFT and Al in cases of ALS in the Kii Peninsula of Japan. We examined six of these ALS cases, ten ALS cases from non‐focused areas (non‐focused ALS) and eight disease controls. The neuropathologic characteristics of Kii ALS were the appearance of NFT in Ammon's horn, temporal cortex, mid‐brain and pons, in ccombination with typical ALS pathology. No senile plaque was found in the Kii cases. Non‐foused ALS patients rarely showed NFTs in Ammon's horn. Al analysis of Ammon's horns by particle‐induced X‐ray emission spectrometry (PIXE) revealed a high level of Al signals in the Kii cases, with positive correlation between Al content and NFT frequency. Kii ALS cases showed high level of Al signals in the cervical and lumbar spinal cords ccompared with non‐foused ALS and controls. The Morin fluorescent technique revealed more conspicuous green fluorescence in the cytoplasm and nuclei of both the NFT‐laden neurons and the degenerative neurons without NFT in Ammon's horn in the Kii ALS cases than in non‐focused ALS cases. Green fluorescence was seen in a small number (2%) of the NFTs themselves. We suggest that Al induces abnormality in cytoskeletal protein such as neurofilaments and/or tau, and may cause the formation of NFT in Ammon's horn and also degeneration of motor neurons in Kii ALS.