Okuyama H, Mizutani T, Bando M, Mitani K, Nagura H, Yamanouchi H, Murata M, Goto J
Department of Neurology, Tokyo Metropolitan Geriatric Hospital, Japan.
No To Shinkei. 1996 Aug;48(8):741-6.
Senile chorea has been an ill-defined clinical entity because of the difficulty of differentiating it from Huntington's disease (HD) of late-onset type. The gene specific for HD has recently been found to contain an abnormal (CAG)n trinucleotide repeat which allows it to be differentiated from the other conditions. Our case of late-onset chorea was differentiated from HD by PCR. An 80-year-old man had experienced gradually increasing chorea of his tongue, arms and legs for 4 years, but had not exhibited character changes, mental symptoms or dementia. He died of pneumonia at 84 years. The pathological findings in this case were different from those of HD. The brain weighted 1220 g, and did not show striatal atrophy or neuronal loss. The most remarkable findings were numerous foamy spheroids scattered in the caudate and putamen and proliferation of fibrous astrocytes within the thin myelinated fiber bundles and in the neuropil. Similar findings were reported by Freidman et al. (1990). Although it is debatable whether this finding was responsible for the development of the chorea, this case should contribute to our understanding of senile chorea as a clinicopathological entity.
由于难以将老年舞蹈症与迟发型亨廷顿舞蹈症（HD）区分开来，它一直是一个定义不明确的临床实体。最近发现，HD的特异性基因含有异常的（CAG）n三核苷酸重复序列，这使得它能够与其他病症区分开来。我们通过聚合酶链反应（PCR）将我们的迟发型舞蹈症病例与HD区分开来。一名80岁男性4年来舌、手臂和腿部的舞蹈症逐渐加重，但未出现性格改变、精神症状或痴呆。他84岁时死于肺炎。该病例的病理结果与HD不同。大脑重量为1220克，未显示纹状体萎缩或神经元丢失。最显著的发现是尾状核和壳核中散在许多泡沫样球体，以及薄髓鞘纤维束内和神经毡中的纤维星形胶质细胞增生。弗里德曼等人（1990年）报告了类似的发现。尽管这一发现是否是舞蹈症发展的原因尚有争议，但该病例应有助于我们将老年舞蹈症理解为一种临床病理实体。
