Tranchant C, Maquet J, Eber A M, Dietemann J L, Franck P, Warter J M
Clinique Neurologique, Hôpital Central, Strasbourg.
Rev Neurol (Paris). 1991;147(8-9):599-602.
On four occasions since 1978, this 53 year-old woman presented with a right hemicorporal hypotonia, symptomatic of a hemispheric cerebellar syndrome. In 1981, she experienced the progressive development of a cervical dystonia. CT scan and RM scan showed a cavernous angioma in the right cerebellar hemisphere. The 18F-2-fluoro-2-deoxy-glucose PET scan revealed a right cerebellar and a contralateral cortical and striatal hypometabolism. This crossed cerebello-cortical diaschisis can be interpreted as a functional interruption of the cerebello-cerebral pathways. This case raises the question of the role played by a cerebellar lesion in the development of a focal dystonia.
自1978年以来,这位53岁的女性曾4次出现右侧半身肌张力减退,这是半球性小脑综合征的症状。1981年,她出现了进行性颈部肌张力障碍。CT扫描和磁共振扫描显示右侧小脑半球有一个海绵状血管瘤。18F - 2 - 氟 - 2 - 脱氧葡萄糖PET扫描显示右侧小脑以及对侧皮质和纹状体代谢减低。这种交叉性小脑 - 皮质失联络可被解释为小脑 - 大脑通路的功能性中断。该病例提出了小脑病变在局灶性肌张力障碍发展中所起作用的问题。