Geographical distribution of amyotrophic lateral sclerosis with neurofibrillary tangles in the Kii Peninsula of Japan.
Between 1984 and 1996 we histopathologically examined 26 autopsy cases of amyotrophic lateral sclerosis (ALS) from the Mie Prefecture in eastern and southern Kii Peninsula, which includes the Hohara ALS focus. Four of the individuals had a moderate number of neurofibrillary tangles in the locus coeruleus, substantia nigra, raphe nucleus, periaqueductal grey and hippocampus in addition to the histological changes of ALS. All four came from the vicinity of Hohara; symptoms of ALS developed in 1979, 1987, 1991 and 1993. Two had family history of ALS, and one, of parkinsonism-dementia. These findings confirm that Kii type ALS occurs continuously in and near the Hohara focus.
Kokubo
Y
Y
Department of Neurology, Mie University School of Medicine, 2-174 Edobashi, Tsu, Mie-ken 514-8507, Japan.
Kuzuhara
S
S
Narita
Y
Y
D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology*; D005260:Female; D006801:Humans; D007564:Japan; D008297:Male; D008875:Middle Aged; D016874:Neurofibrillary Tangles / Q000473:pathology*