Leblhuber F, Windhager E, Reisecker F, Rittmannsberger H
Department of Gerontology, Wagner-Jauregg-Krankenhaus, Linz, Austria.
Eur Arch Psychiatry Clin Neurosci. 1991;241(2):113-4. doi: 10.1007/BF02191153.
In healthy subjects, 2 EMG responses of the thenar muscles can be distinguished, elicited by electrical stimulation of the median nerve during an isometric contraction: an early spinal response (M1) and a long latency response (LLR) (M2); earlier studies have shown that in patients with Huntington's chorea (HC) this late EMG response is missing. LLR were studied in the nine subjects at risk out of three families with definite HC. In 6 of them, LLR was clearly asymmetrical or absent on one or both sides, while normal LLRs were seen in the rest of the subjects studied; LLR abnormalities found in clinically free members of HC families may assist in early diagnosis of individuals who may later develop symptoms and may help in genetic counselling.
在健康受试者中,在等长收缩期间通过正中神经电刺激可引出2种鱼际肌肌电图反应:早期脊髓反应(M1)和长潜伏期反应(LLR)(M2);早期研究表明,患有亨廷顿舞蹈症(HC)的患者不存在这种晚期肌电图反应。对3个确诊HC家族中的9名有患病风险的受试者进行了LLR研究。其中6名受试者一侧或双侧的LLR明显不对称或缺失,而其余受试者的LLR正常;在HC家族无症状成员中发现的LLR异常可能有助于对后来可能出现症状的个体进行早期诊断,并有助于遗传咨询。