Kuzuhara Shigeki
Department of Neurology, Mie University School of Medicine, Edobashi, Tsu.
Rinsho Shinkeigaku. 2002 Nov;42(11):1073-6.
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.
纪伊半岛的小原村是肌萎缩侧索硬化症（ALS）的高发地区之一，据报道，该地区的高发病率在20世纪80年代初已结束。然而，我们发现ALS的发病率仍然很高，是日本其他地区的100多倍。此外，我们还发现了许多帕金森痴呆综合征（PDC）病例。ALS和PDC经常在单个患者或单个家庭中同时出现。超过70%的患者有家族病史。ALS和PDC表现出共同的神经病理学特征，包括ALS病理和许多没有老年斑的神经原纤维缠结（NFT），NFT tau蛋白的异构体模式与阿尔茨海默病（AD）相似，但与进行性核上性麻痹（PSP）或皮克病（PiD）不同。纪伊ALS/PDC可能是一种不同于AD、PSP或PiD的新型tau蛋白病。
