Kokubo Yasumasa, Kuzuhara Shigeki
Department of Neurology, Mie University School of Medicine, Tsu, Mie-ken, Japan.
Arch Neurol. 2003 Sep;60(9):1257-61. doi: 10.1001/archneur.60.9.1257.
The Kii peninsula of Japan, together with Guam and West New Guinea, has one of the highest incidences of amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia complex (PDC) in the world.
To perform neuroimaging studies on patients with ALS and PDC on the Kii peninsula.
Results of computed tomography, magnetic resonance imaging, and single-photon emission computed tomography were studied in 4 patients with ALS and in 10 patients with PDC from the Hohara village on the Kii peninsula of Japan.
In patients with PDC, there was mild to severe atrophy of the frontal and temporal lobes on computed tomography and magnetic resonance imaging and a marked decrease in cerebral blood flow on single-photon emission computed tomography. In contrast, in patients with ALS, there was a decrease in cerebral blood flow of the frontal and temporal lobes, although the patients did not show signs of clinical dementia or obvious brain atrophy on computed tomography or magnetic resonance imaging.
The finding of an obvious decrease in cerebral blood flow of the frontal and temporal lobes in patients with PDC and ALS with or without cerebral atrophy supports the concept that the 2 conditions are different manifestations of a single frontotemporal tauopathy.
日本纪伊半岛与关岛及新几内亚西部一样，是世界上肌萎缩侧索硬化症（ALS）和帕金森病痴呆综合征（PDC）发病率最高的地区之一。
对纪伊半岛上的ALS和PDC患者进行神经影像学研究。
对来自日本纪伊半岛小原村的4例ALS患者和10例PDC患者的计算机断层扫描、磁共振成像及单光子发射计算机断层扫描结果进行了研究。
在PDC患者中，计算机断层扫描和磁共振成像显示额叶和颞叶有轻度至重度萎缩，单光子发射计算机断层扫描显示脑血流量显著减少。相比之下，在ALS患者中，额叶和颞叶的脑血流量减少，尽管这些患者在计算机断层扫描或磁共振成像上未表现出临床痴呆迹象或明显的脑萎缩。
PDC和ALS患者无论有无脑萎缩，额叶和颞叶脑血流量均明显减少，这一发现支持了这两种疾病是单一额颞叶tau蛋白病不同表现形式的观点。
