The Japanese journal of neuropsychology

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ArticleTitle	Amyotrophic lateral sclerosis-parkinsonism-dementia complex of the Kii peninsula, Guam and New Guinea
Language	J
AuthorList	Shigeki Kuzuhara
Affiliation	Department of Neurology, National Center Hospital of Neurology and Psychiatry, National Center of Neurology and Psychiatry Japan
Publication	Japanese Journal of Neuropsychology: 25 (3), 182-191, 2009
Received
Accepted
Abstract	Strikingly high concentration of amyotrophic lateral sclerosis (ALS), nearly 100 fold of that of the other areas of the world existed in the Kii peninsula, Guam and West New Guinea along longitude 140° East, which was reported to have disappeared until early 1980s. We investigated the previously high incidence village and found continuing high incidence of ALS together with autopsy-verified parkinsonism-dementia complex (PDC) similar to PDC on Guam. The neuropathological findings of Kii ALS and PDC were very similar to each other, showing many neurofibrillary tangles in the brainstem and cerebrum, TDP-43 proteinopathy, and ALS pathology. More than 70% of patients had family history of ALS/PDC, suggesting a heredodegenerative disease, and analyses of all the known genes related to ALS and motor neuron diseases, parkinsonism, and dementia failed to disclose any abnormalities so far. No specific environmental risk factors including food and drinking water were found. Clinical and neuropathological features of ALS/PDC of Guam and West New Guinea were presented with, and recent advances of the research on the cause and pathogenesis of ALS/PDC were reviewed.
Keywords	amyotrophic lateral sclerosis, parkinsonism-dementia complex, Kii peninsula, Guam, West New Guinea