Vidailhet Marie, Lehéricy Stéphane
Bull Acad Natl Med. 2013 Apr-May;197(4-5):847-58; discussion 858-9.
Recent developments in magnetic resonance imaging have provided further insights into the pathophysiology of movement disorders including dystonias and Tourette syndrome. Both structural and functional abnormalities have been described in dystonic patients, with a number of genotype-phenotype correlations. Interactions between the cerebello-thalamo-cortical and basal ganglia-cortex networks play a role in the penetrance and expression of dystonia. In Tourette syndrome, motor symptoms and behavioral disorders correlate with structural changes in limbic, motor and associative fronto-striato-parietal circuits of the brain. Both disorders may be related to subtle developmental abnormalities. Compensatory mechanisms may have either a positive or a negative effect (adaptive reactions or faulty activity).
磁共振成像的最新进展为包括肌张力障碍和妥瑞氏综合征在内的运动障碍的病理生理学提供了进一步的见解。肌张力障碍患者存在结构和功能异常,且有多种基因型与表型的相关性。小脑-丘脑-皮质和基底神经节-皮质网络之间的相互作用在肌张力障碍的外显率和表现中起作用。在妥瑞氏综合征中,运动症状和行为障碍与大脑边缘、运动和联合性额-纹状体-顶叶回路的结构变化相关。这两种疾病都可能与细微的发育异常有关。代偿机制可能产生积极或消极的影响(适应性反应或错误活动)。