平山病患者大脑皮层活化规律及神经电生理特性的综合研究

Hirayama’s disease, also known as juvenile distal spinal muscular atrophy of upper extremities, more and more cases have recently been reported in mainland China, and other areas in Asia. It has been suggested that Hirayama’s disease might be due to flexion myelopathy. Because of lack of effective drug treatment, some scholars have achieved clinical success with surgical interventions recently, but there are much controversial in the choice of the surgical program selection and quantitative assessment. In the previous study, we found that there was a certain regular changes in the cortex activation area and dynamic F-waves and other indicators in the postoperative patients with Hirayama disease, which is expected to be objective quantitative indicators for the severity and efficacy evaluation, and provides a new research idea for the internal mechanism of neck flexion in Hirayama’s disease. Therefore, this study intends to use BOLD-fMRI (blood oxygen level dependent functional magnetic resonance imaging) and electrophysiological testing technologies to comprehensive assess the functional status of patients before and after surgical intervention for the Hirayama disease patients. By comparison study with healthy volunteers, to analyze the cortex activation characteristics and dynamic F-waves under different neck positions, and further to explore the inherent influence of neck flexion in the onset of Hirayama patients. The expected results will provide some objective quantitative indicators for the clinical assessment and postoperative functional outcome in Hirayama disease patients.

平山病又称青少年上肢远端肌萎缩症，近年在中国大陆等亚洲地区临床病例不断增多。目前认为平山病可能为一种屈性脊髓病。由于该病缺乏有效药物治疗，进行外科干预成为重要的治疗手段；但在手术干预时机选择、疗效评估等方面尚存在较大的分歧。我们的前期研究发现平山病患者术后大脑皮层激活区域及动力位F波等指标呈现规律性变化，有望成为平山病病情与疗效评估的客观性量化指标，同时为明确颈部屈曲在其发病过程中的内在机制提供研究新思路。为此，本研究拟以BOLD-fMRI（血氧水平依赖功能磁共振成像）与电生理检测技术对平山病患者手术干预前后的功能状态进行联合研究。通过与健康志愿者比对研究，对平山病患者手术干预前后颈部不同体位下大脑皮层激活特点、动力位F波等电生理指标的变化规律进行分析；同时对颈部屈曲在平山病患者发病过程的内在影响进行研究分析，以期为平山病患者的病情和疗效评估及术后功能转归等提供客观量化指标和科学依据。

平山病又称青少年上肢远端肌萎缩症，近年来在中国大陆等亚洲地区临床报道逐渐增多。在前期研究中，我们发现平山病患者手术干预前后大脑皮层激活区域及动力位F波等指标存在规律性变化因此，有望成为重要的病情和疗效评估方法，并有助于明确颈部屈曲在平山病发病过程中的内在影响及机制。该项目按照研究计划时间节点顺利完成24例平山病患者术前及术后握力定量测定、BOLD-fMRI检查、正中神经/尺神经动力位F波检测等；顺利完成颈椎前路融合治疗以及完成24例健康志愿者的时间点匹配检测。并进一步完成17例平山病患者术后3月、6月的BOLD-fMRI检查、正中神经/尺神经动力位F波等检查随访；并对患者术后功能恢复进行评估。随访对术后随访结果进行分析、总结。..我们研究发现：.①屈颈位MRI作为平山病临床诊断的常用影像学检查技术具有较好的临床价值，但对平山病手术干预节段的选择方面存在一定的不足，手术节段的选择需要结合颈椎动力位X线片等其他临床资料进行综合判断。.②平山病患者屈颈位F波较颈椎中立位时存在明显的规律性改变，尤以F波波幅的异常增大最为明显。颈部屈曲对平山病患者，尤其是症状较重侧正中神经/尺神经F波存在明显影响，从而证实颈部屈曲在平山病发病机制中存在重要影响。.③颈部屈曲对健侧肢体激活无明显影响，但能够造成患侧肢体对侧半球运动区活化的减弱。进一步研究显示颈椎前路融合术后3月及6月随访部分患侧肢体仍存在双侧半球活化现象，患侧肢体活化与健侧肢体的差异逐渐减小，健侧肢体诱发的活化不受颈部曲屈的影响，患侧肢体诱发的活化不再受颈部曲屈的影响。..在该项目研究成果的基础上，提出了意在更好指导临床实际工作的平山病临床诊断新标准，以及平山病首个临床分型系统及临床诊治规范专家共识，将显著提升平山病的认识水平和临床诊治效果。.