Biomaterial Database

[Amyotrophic lateral sclerosis-parkinsonism-dementia complex of the Kii Peninsula of Japan]. 2002

Shigeki Kuzuhara

Department of Neurology, Mie University School of Medicine, Edobashi, Tsu.

The Hohara village of the Kii peninsula is one of the high incidence ALS foci, and the high incidence was reported to have ended in early 1980s. However, we have found the ALS incidence rate has been still high, more than 100 times of the other areas of Japan. In addition, we have found many cases of parkinsonism-dementia complex (PDC). ALS and PDC often occur simultaneously in a single patient or in a single family. Family history was positive in more than 70% of patients. ALS and PDC showed common neuropathological findings consisting of ALS pathology and many neurofibrillary tangles (NFT) without senile plagues, and isoform pattern of NFT tau protein was similar to that of Alzheimer disease (AD), but different from that of progressive supranuclear palsy (PSP) or Pick's disease (PiD). Kii ALS/PDC may be a novel tauopathy that differ from AD, PSP or PiD.

UI	MeSH Term	Description	Entries
D007564	Japan	A country in eastern Asia, island chain between the North Pacific Ocean and the Sea of Japan, east of the Korean Peninsula. The capital is Tokyo.	Bonin Islands
D008297	Male		Males
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000690	Amyotrophic Lateral Sclerosis	A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94)	ALS - Amyotrophic Lateral Sclerosis,Lou Gehrig Disease,Motor Neuron Disease, Amyotrophic Lateral Sclerosis,Amyotrophic Lateral Sclerosis With Dementia,Amyotrophic Lateral Sclerosis, Guam Form,Amyotrophic Lateral Sclerosis, Parkinsonism-Dementia Complex of Guam,Amyotrophic Lateral Sclerosis-Parkinsonism-Dementia Complex 1,Charcot Disease,Dementia With Amyotrophic Lateral Sclerosis,Gehrig's Disease,Guam Disease,Guam Form of Amyotrophic Lateral Sclerosis,Lou Gehrig's Disease,Lou-Gehrigs Disease,ALS Amyotrophic Lateral Sclerosis,Amyotrophic Lateral Sclerosis Parkinsonism Dementia Complex 1,Amyotrophic Lateral Sclerosis, Parkinsonism Dementia Complex of Guam,Disease, Guam,Disease, Lou-Gehrigs,Gehrig Disease,Gehrigs Disease,Sclerosis, Amyotrophic Lateral