Borit A, Rubinstein L J, Urich H
Brain. 1975 Mar;98(1):101-12. doi: 10.1093/brain/98.1.101.
Three new cases of striato-nigral degeneration (SND) are presented with particular reference to the identification of putaminal pigments. These were studied by histochemical methods, electron microscopy and elemental analysis. Three interrelated parenchymal perikaryal pigments were identified in the putaminal lesions of all 3 patients: a "haematin" pigment, neuromelanin and lipofuscin. The presence of neuromelanin in the putamen may be due to accumulation of dopamine in the synaptic terminals of the nigro-striatal pathway and its polymerization into pigment. This suggests that the putaminal atrophy is the primary lesion in SND which is a true supranigral form of parkinsonism. Clinically this manifests itself in predominance of rigidity over other parkinsonian symptoms and in a poor, or absent, response to treatment with L-dopa and anticholinergic drugs.
本文报告了3例新的纹状体黑质变性（SND）病例，特别提及了壳核色素的鉴定。通过组织化学方法、电子显微镜和元素分析对这些病例进行了研究。在所有3例患者的壳核病变中鉴定出三种相互关联的实质核周色素：一种“血红素”色素、神经黑素和脂褐素。壳核中神经黑素的存在可能是由于多巴胺在黑质纹状体通路的突触终末积聚并聚合成色素所致。这表明壳核萎缩是SND的原发性病变，SND是一种真正的黑质上方型帕金森病。临床上，其表现为强直比其他帕金森症状更为突出，对左旋多巴和抗胆碱能药物治疗反应不佳或无反应。
