量研学術機関リポジトリ「QST-Repository」は、国立研究開発法人 量子科学技術研究開発機構に所属する職員等が生み出した学術成果（学会誌発表論文、学会発表、研究開発報告書、特許等）を集積しインターネット上で広く公開するサービスです。 Welcome to QST-Repository where we accumulates and discloses the academic research results(Journal Publications, Conference presentation, Research and Development Report, Patent, etc.) of the members of National Institutes for Quantum Science and Technology.
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A migration case of Kii amyotrophic lateral sclerosis/parkinsonism dementia complex with the shortest stay in the endemic area and the longest incubation to develop the disease
利用統計を見る
Amyotrophic lateral sclerosis/parkinsonism dementia complex (ALS/PDC) is an endemic disease observed
in the Kii peninsula, Guam, and Papua. We report a case of a 76-year old man with ALS/PDC of the Kii
peninsula of Japan (Kii ALS/PDC). The patient was born and grew up in the Kii peninsula. He moved
out at age three, and developed symptoms 73 years later. He showed pyramidal sign, parkinsonian symptoms,
and mildly impaired cognitive function. 131I-metaiodobenzylguanidine myocardial scintigraphy
showed decreased cardiac sympathetic nerve function, and dopamine transporter single photon emission
computed tomography imaging showed decreased 123I-N-x-fluoropropyl-2b-carbomethoxy3b-(4-iodo
phenyl) nortropane accumulation. Cerebral blood flow showed hypoperfusion. Positron emission tomography
showed widespread tau deposition in his brain. This is a migration case of Kii ALS/PDC with the
shortest stay in the endemic area and the longest delay to develop the disease, indicating a genetic factor
for the disease development in a considerable degree.
A migration case of Kii amyotrophic lateral sclerosis/parkinsonism dementia complex with the shortest stay in the endemic area and the longest incubation to develop the disease
