Michel D, Tommasi M, Laurent B, Trillet M, Schott B
Rev Neurol (Paris). 1976 Jan;132(1):3-22.
Two sporadic cases of striato-nigral degeneration were characterized by the onset at the age of 61 of an akinetic-hypertonic syndrome and of a minimal or absent rest tremor, with a pyramidal syndrome, sphincter disorders, dysphonia, difficulty in swallowing and an unfavorable development in the space of two or three years not affected by L-Dopa. In one case, total insomnia was demonstrated by means of 3 polygraphic recordings during the night, two of them consecutive, and aprobenecide test showed a definite fall in H.V.A. and 5 H.I.A.A. in the lumbar cerebrospinal fluid. Anatomical verification showed, in one case, isolated putamino-nigral degeneration, and in the other associated with lesions of the olivo-cerebellopontine system. Much of the excess pigmentation found only in the putamen was melanotic in character and resulted perhaps from the striato-nigral degeneration, making it possible to classify this more accurately among the other multisystem degenerations, especially olivo-ponto-cerebellar atrophy and Shy-Drager syndrome
61岁起病，表现为运动不能-张力亢进综合征，静止性震颤轻微或无，伴有锥体束综合征、括约肌功能障碍、发音困难、吞咽困难，且在两三年时间里病情进展不佳，左旋多巴治疗无效。其中一例，通过夜间3次多导睡眠图记录证实存在完全失眠，其中两次记录连续进行，丙磺舒试验显示腰椎脑脊液中高香草酸（H.V.A.）和5-羟吲哚乙酸（5 H.I.A.A.）明显降低。解剖学证实，一例为孤立的壳核黑质变性，另一例伴有橄榄小脑脑桥系统病变。仅在壳核发现的大量色素沉着具有黑色素性质，可能是纹状体黑质变性所致，从而有可能将其更准确地归类于其他多系统变性疾病，尤其是橄榄脑桥小脑萎缩和夏伊-德雷格综合征。
