[An autopsy case of dentatorubropallidoluysian atrophy (DRPLA) clinically diagnosed as Huntington's chorea].
[An autopsy case of dentatorubropallidoluysian atrophy (DRPLA) clinically diagnosed as Huntington's chorea].
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临床诊断为亨廷顿舞蹈病的齿状核红苍白球体萎缩症（DRPLA）尸检一例[J].
DOI：
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发表时间：
1985
期刊：
影响因子：
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通讯作者：
Y. Yamamoto
中科院分区：
文献类型：
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作者：
T. Nakano;K. Iwabuchi;S. Yagishita;N. Amano;M. Akagi;Y. Yamamoto
报告了一名65岁女性齿状体白斑萎缩（DRPLA）的尸检病例。她的母亲因步态障碍去世，享年63岁。她母亲的哥哥出现了精神病症状。她姐姐的一个女儿由于产后精神状态住进精神病院时，观察到她有不自主的运动。她的弟弟从56岁左右开始出现步态障碍。她的大儿子患有精神分裂症已经很多年了。患者58岁起出现小脑性共济失调步态，3年后四肢及面部出现舞蹈性不自主运动，并逐渐突出。63岁起，偶有视幻觉引起的异常行为。63岁时，她因对丈夫持续的迫害性妄想而住进精神病院，并因其异常的舞蹈动作和伴有痴呆的精神状态被临床诊断为亨廷顿舞蹈症。高血压也被注意到了。65岁时，她死于急性肺炎。她的病持续了大约6年。中枢神经系统的组织病理学检查结果：大脑重1014克。脑干和脊髓的体积相对较小。大脑皮层保存完好。中央半谷弥漫性脑白质脱髓鞘，小血管动脉硬化改变明显。显著的病理改变包括以下两个系统的明显对称萎缩，即齿状白球系统。（摘要删节250字）
An autopsy case of a 65-year-old female with dentatorubropallidoluysian atrophy (DRPLA) is reported. Her mother had gait disturbance and died at the age of 63. Her mother's brother developed psychotic symptoms. A daughter of her older sister was observed to have involuntary movement when she admitted to a mental hospital due to post-delivery psychotic state. Her younger brother has developed gait disturbance from about 56-year-old. Her older son has suffered from schizophrenia for long years. Since 58-year-old, she developed cerebellar ataxic gait and three years later, choreic involuntary movement developed in her extremities and face and progressively became prominent. Since 63-year-old, abnormal behavior brought about by the visual hallucination was occasionally observed. At the age of 63, she admitted to a mental hospital because of persistent persecutive delusion for her husband and was clinically diagnosed as Huntington's chorea for her remarkable choreic movement and psychotic state with dementia. Hypertension was also noticed. At the age of 65, she died of acute pneumonia. The duration of her illness was about 6 years. Histopathological findings of the CNS: the brain weighed 1,014 g. Brainstem and spinal cord were noticed to be relatively small in size. The cerebral cortex was well preserved. The cerebral white matter was diffusely demyelinated in the central semiovale where arteriosclerotic change of the small vessels was remarkable. Significant pathological changes consisted of marked symmetrical atrophy of the following two systems, i. e., dentatofugal pallidoluysian systems.(ABSTRACT TRUNCATED AT 250 WORDS)