IRCCS Neuromed Institute, 'Sapienza' University of Rome, Pozzilli (Isernia), Italy.
Department of Human Neuroscience, 'Sapienza' University of Rome, Rome, Italy.
Eur J Neurol. 2019 May;26(5):701-e52. doi: 10.1111/ene.13928. Epub 2019 Mar 15.
Corticobasal degeneration (CBD) is a neurodegenerative condition characterized by 4R tau protein deposition in several brain regions that clinically manifests itself as a heterogeneous atypical parkinsonism typically expressed in adulthood. The prototypical clinical phenotype of CBD is corticobasal syndrome (CBS). Important insights into the pathophysiological mechanisms underlying motor and higher cortical symptoms in CBS have been gained by using advanced neuroimaging and neurophysiological techniques. Structural and functional neuroimaging studies often show asymmetric cortical and subcortical abnormalities, mainly involving perirolandic and parietal regions and basal ganglia structures. Neurophysiological investigations including electroencephalography and somatosensory evoked potentials provide useful information on the origin of myoclonus and on cortical sensory loss. Transcranial magnetic stimulation demonstrates heterogeneous and asymmetric changes in the excitability and plasticity of primary motor cortex and abnormal hemispheric connectivity. Neuroimaging and neurophysiological abnormalities in multiple brain areas reflect asymmetric neurodegeneration, leading to asymmetric motor and higher cortical symptoms in CBS.
皮质基底节变性（CBD）是一种神经退行性疾病，其特征是在几个脑区存在 4R tau 蛋白沉积，临床上表现为异质性非典型帕金森病，通常在成年期表现出来。CBD 的典型临床表型是皮质基底节综合征（CBS）。通过使用先进的神经影像学和神经生理学技术，我们对 CBS 中运动和高级皮质症状的病理生理机制有了重要的了解。结构性和功能性神经影像学研究经常显示不对称的皮质和皮质下异常，主要涉及额-顶叶和基底节结构。包括脑电图和体感诱发电位在内的神经生理学研究为肌阵挛的起源和皮质感觉缺失提供了有用的信息。经颅磁刺激显示初级运动皮层兴奋性和可塑性的异质性和不对称变化以及异常的半球间连接。多个脑区的神经影像学和神经生理学异常反映了不对称性的神经退行性变，导致 CBS 中不对称性的运动和高级皮质症状。
