Bergmann M, Schmidtke K, Danek A, Gullotta F, Mehraein P
Institut für Neuropathologie, Universität Münster.
Schweiz Arch Neurol Psychiatr (1985). 1990;141(5):389-405.
Two cases of striato-nigral degeneration are reported. In case 1 the female patient showed a Parkinson syndrome, cardiac arrhythmias and vasomotor disturbances. Morphologically mainly the putamen and substantia nigra revealed severe atrophic changes according to the restricted form of striato-nigral degeneration (SND). In case 2 there was a disorder of the upper and lower motor neuron in a female patient. Morphologically this case was an example of a multisystem atrophy with changes in the striato-nigral, olivo-ponto-cerebellar systems and spinal motor and autonomic neurons. These cases demonstrate the variability of the striato-nigral degenerations which are met with in two forms: a "pure" form as in case one and as a part of multisystem atrophies. This is underlined in a review of 69 literature cases, which also shows that SND, Shy-Drager syndrome and olivo-ponto-cerebellar atrophy represent very probably different varieties of one and the same degenerative process.
报告了两例纹状体黑质变性病例。病例1中,女性患者表现出帕金森综合征、心律失常和血管舒缩功能障碍。形态学上,根据纹状体黑质变性(SND)的局限形式,主要是壳核和黑质显示出严重的萎缩性改变。病例2中,一名女性患者存在上下运动神经元障碍。形态学上,该病例是多系统萎缩的一个例子,伴有纹状体黑质、橄榄脑桥小脑系统以及脊髓运动和自主神经元的改变。这些病例证明了纹状体黑质变性的变异性,其有两种形式:如病例1中的“纯”形式以及作为多系统萎缩的一部分。对69例文献病例的回顾强调了这一点,该回顾还表明,SND、夏伊-德雷格综合征和橄榄脑桥小脑萎缩很可能代表同一变性过程的不同变体。