基于多模态影像学和神经电生理技术的平山病发病机制综合研究

平山病好发于青少年男性，以手无力伴肌萎缩为主要特征，近年在中国等亚洲地区临床病例不断增多。目前存在脊髓动力因素和生长发育因素等多种发病机制假说，但国内外学者尚未达成共识。本课题组前期通过常规MRI及F波等研究发现平山病患者屈颈后脊髓形态和外周神经功能存在规律变化，但缺少脊髓功能层面的深入研究和动物模型构建。为此，本课题拟采用弥散张量成像、磁共振神经成像等多模态影像学技术和神经传导检测、F波及运动单位数目估计等神经电生理技术，评估平山病患者“中立位-屈颈位”下脊髓功能、颈神经根形态等指标以及正中神经、尺神经CMAP波幅、F波频率和肌肉MUNE值等指标的特点与变化规律，并与健康者对比；在此基础上，采用山羊通过颈椎手术尝试构建平山病动物模型并进行验证，探讨动力性因素与牵张性因素在发病机制中的作用。以期明确平山病具体发病机制，建立合适的平山病动物模型，为提高其临床诊治水平提供科学依据与理论基础。

Hirayama disease, which usually affects young males and is characterized by hand weakness accompanied by muscular atrophy, has increased gradually in Asian countries such as China recently. At present, there are many hypotheses of pathogenesis such as spinal cord dynamic factors and growth and development factors, but researchers have not reached a consensus. Our previous studies using conventional MRI and F wave have found out the changes of spinal cord morphology and peripheral nerve function in patients with Hirayama disease after cervical flexion. However, the functional changes of the spinal cord and the animal model of Hirayama disease still need to be investigated. To this end, this study intends to use multi-modal imaging techniques such as diffusion tensor imaging, magnetic resonance neurography and neuro-electrophysiological techniques such as nerve conduction study, F wave and motor unit number estimation to evaluate the spinal cord function, cervical nerve roots morphology and other indicators in patients with Hirayama disease in cervical neutral and flexion positions, as well as the CMAP amplitude and F wave frequency of the median nerve and ulnar nerve, together with the MUNE of the distal muscle, and then compares them with the healthy volunteers’. On this basis, we use goats to attempt to establish an animal model of Hirayama disease by cervical spine surgery, and then validate this animal model to explore the role of dynamic and stretching factors in pathogenesis. This study aims to clarify the specific pathogenesis of Hirayama disease, try to establish a suitable animal model, and provide scientific and theoretical basis for the improvement of the diagnosis and treatment.