Journal Article 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

Tsunoda, Keiichiro  ,  Yamashita, Toru  ,  Shimada, Hitoshi  ,  Nomura, Emi  ,  Takahashi, Yoshiaki  ,  Shang, Jingwei  ,  Sato, Kota  ,  Takemoto, Mami  ,  Hishikawa, Nozomi  ,  Ohta, Yasuyuki  ,  Higuchi, Makoto  ,  Suhara, Tetsuya  ,  Kokubo, Yasumasa  ,  Kuzuhara, Shigeki  ,  Abe, Koji

Description
Amyotrophic lateral sclerosis/parkinsonism dementia complex (ALS/PDC) is an endemic disease observedin the Kii peninsula, Guam, and Papua. We report a case of a 76-year old man with ALS/PDC of the Kiipeninsula of Japan (Kii ALS/PDC). The patient was born and grew up in the Kii peninsula. He movedout at age three, and developed symptoms 73 years later. He showed pyramidal sign, parkinsonian symptoms,and mildly impaired cognitive function. 131I-metaiodobenzylguanidine myocardial scintigraphyshowed decreased cardiac sympathetic nerve function, and dopamine transporter single photon emissioncomputed tomography imaging showed decreased 123I-N-x-fluoropropyl-2b-carbomethoxy3b-(4-iodophenyl) nortropane accumulation. Cerebral blood flow showed hypoperfusion. Positron emission tomographyshowed widespread tau deposition in his brain. This is a migration case of Kii ALS/PDC with theshortest stay in the endemic area and the longest delay to develop the disease, indicating a genetic factorfor the disease development in a considerable degree.

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