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中文摘要
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该项目的目标是了解大脑如何使用脊髓回路来协调运动,以及神经系统疾病如何改变运动回路的功能。在过去的几年里,我们已经研究了选择性影响特定神经元类别的疾病中皮质和脊髓运动回路的变化。在2006财年,我们研究了脊髓灰质炎长期幸存者(运动神经元损失较低)和原发性侧索硬化症(一种影响上运动神经元的神经退行性疾病)患者皮质运动回路的功能变化。在脊髓灰质炎幸存者中,我们发现皮质脊髓系统的激活发生了变化,使脊髓灰质炎影响的肌肉的运动输出最大化。这些适应与脊髓灰质炎后综合症的症状无关。在PLS患者中,我们报道了来自运动前和运动皮层的与自主运动相关的慢皮层脑电图电位减弱,但与运动相关的β频率振荡信号的变化被保留。
英文摘要
The goals of this project are to understand how the brain uses spinal cord circuits to coordinate movement and how neurological disorders alter the functioning of motor circuits. In previous years, we have examined changes in cortical and spinal motor circuits in disorders that selectively affect specific neuron classes. In FY2006 we studied functional changes in cortical motor circuits in long-term survivors of polio who have lower motor neuron loss and in patients with primary lateral sclerosis (PLS), a neurodegenerative disorder affecting upper motor neurons. In polio survivors, we found changes in the activation of the corticospinal system that maximize the motor output to muscles that had been affected by polio. These adaptations were not correlated with symptoms of post-polio syndrome. In patients with PLS we reported that the slow cortical EEG potentials associated with voluntary movement from pre-motor and motor areas of the cortex were diminished, but that changes in the beta-frequency oscillatory signals associated with movement were preserved. In FY2007, we expanded on these observations in PLS patients in three areas. The first was a collaboration with Dr. Ou Bai to use single-trial changes in oscillatory signals for an EEG-based brain-computer interface. The preliminary findings are promising that these signals may be useful for development of a neural-driven prosthesis in patients with motor neuron disorders. Further description of this project can be found in the Human Motor Control section report. The second line of study was aimed at assessing the extent of (non-motor) frontal cortical dysfunction in PLS patients, and to compare them to patients with amyotrophic lateral sclerosis (ALS). It has been proposed that ALS and fronto-temporal dementia have a common pathology, and that many ALS patients have frontal cognitive dysfunction. In this study, we have worked extensively with the Cognitive Neuroscience section to use the same testing battery that they have used in their studies of patients with fronto-temporal dementia. This protocol, which is planned to run for 3 years, includes neuropsychological tests, psychiatric assessment, and anatomical MRI with diffusion tensor imaging. The imaging studies will be examined in an exploratory fashion to determine whether any quantitative measures correlate to motor function or cognitive deficits. Lastly, recognizing that PLS is likely to be complex disorder, we are collecting samples from PLS patients for the NINDS DNA repository for motor neuron diseases at Coriell, so that this rare population will be accessible to the general scientific community for further studies.
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Spasticity and Upper Motor Neuron Disorders
Spasticity and spinal mechanisms of human motor control
Spasticity and Upper Motor Neuron Disorders
Natural history and biomarker discovery in C9orf72 Amyotrophic lateral sclerosis and frontotemporal dementia
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