TY - JOUR
T1 - 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
AU - Tsunoda, Keiichiro
AU - Yamashita, Toru
AU - Shimada, Hitoshi
AU - Nomura, Emi
AU - Takahashi, Yoshiaki
AU - Shang, Jingwei
AU - Sato, Kota
AU - Takemoto, Mami
AU - Hishikawa, Nozomi
AU - Ohta, Yasuyuki
AU - Higuchi, Makoto
AU - Suhara, Tetsuya
AU - Kokubo, Yasumasa
AU - Kuzuhara, Shigeki
AU - Abe, Koji
N1 - Funding Information:
We appreciate the cooperation of the patient. This work was partly supported by a Grant-in-Aid for Scientific Research (B) 25293202 (KA), (C) 15K09316 and Challenging Research 15K15527 (KA) and Young Research 15K21181 (TY) and (A) 26713031 (HS), by Grants-in-Aid from the Research Committees (Mizusawa H and Aoki M) from the Ministry of Health, Labour and Welfare of Japan , Grants-in-Aid for the Brain Mapping by Integrated Neurotechnologies for Disease Studies (Brain/MINDS; 15653129) (MH and TS) and Research and Development Grants for Dementia (16768966) (MH) from the Japan Agency for Medical Research and Development, and by a Grant-in-Aid for the Research Consortium of Kii ALS/PDC (KY) from Japan Agency for Medical Research and Development (AMED).
Publisher Copyright:
© 2017 Elsevier Ltd
PY - 2017/12
Y1 - 2017/12
N2 - 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-ω-fluoropropyl-2β-carbomethoxy3β-(4-iodophenyl) 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.
AB - 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-ω-fluoropropyl-2β-carbomethoxy3β-(4-iodophenyl) 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.
KW - Amyotrophic lateral sclerosis
KW - Positron emission tomography
KW - Tau
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U2 - 10.1016/j.jocn.2017.08.057
DO - 10.1016/j.jocn.2017.08.057
M3 - Article
C2 - 28890043
AN - SCOPUS:85033662605
SN - 0967-5868
VL - 46
SP - 64
EP - 67
JO - Journal of Clinical Neuroscience
JF - Journal of Clinical Neuroscience
ER -