摘要
目的分析平山病的临床及磁共振(magnetic resonance imaging,MRI)特点,探讨MRI对平山病的诊断价值。方法回顾性分析13例男性青年平山病患者的临床、肌电图检查和影像学资料;MRI检查包括自然位及屈颈位平扫、T1WI增强及扩散张量成像(diffusion tensor imaging,DTI)。结果 13例患者主要表现单侧或双侧上肢远端进行性肌萎缩、肌无力。肌电图示受累肌肉均发生神经源性损害。自然位MRI表现:①13例颈椎序列曲度失常;颈椎间盘变性、膨出或突出。②脊髓受累平面主要位于下段颈髓;横轴位见下段颈髓萎缩变扁;7例受累脊髓节段内见缺血坏死及胶质细胞增生的T2WI高信号。屈颈位MRI表现:①硬脊膜囊后壁前移并压迫相应节段颈髓;硬脊膜囊后间隙增宽;9例于增宽间隙内见弧形或小圆点状血管流空影。②3例增强扫描示背侧硬脊膜外间隙明显均匀强化。③3例DTI示病变颈髓区ADC值升高,FA值降低;2例病灶区神经纤维束变细稀疏,1例平扫示软化灶区的神经纤维束中断不连续。结论平山病的临床及MRI表现均有一定特点,动态体位MRI更具特征性,DTI技术可以定量评价病灶内神经纤维束的结构和功能,有助于诊断及判断预后。
Objective To analyze the clinical and magnetic resonance imaging（MRI） manifestations of Hirayama disease for improving diagnostic accuracy.Methods Clinical,electromyography and MRI data of 13 male patients were reviewed retrospectively.Cervical spine MRI scanning included plain scanning in both neutral and flexed positions,contrast enhancement scanning and diffusion tensor imaging（DTI）（3 cases）.Results Progressive muscular weakness and atrophy of unilateral or bilateral distal upper extremities were the main manifestations of the 13 patients.The results of electromyography presented neurogenic damage in the involved muscles.MRI manifestations on neutral position showed： ① Cervical array and curvature were abnormal,and cervical intervertebral discs degeneration,bulging and protrusion were also observed in all patients.② Atrophic and flattening spinal cord was mainly located in lower cervical segments on the axial position,and the affected segments in 7 patients presented abnormal intramedullary high signal that was the same as ischemia,necrosis and mild proliferation of glial cells.MRI findings on flexed position showed： ① Lower cervical dural sac shifted forward and compressed the corresponding segments of spinal cord in all patients,and an obvious dilation was observed in posterior epidural space.Small round and curvilinear flow void signal in the dilated epidural space was observed in 9 patients.② Strong homogeneous enhancement of posterior epidural space on the post contrast flexion position imaging was observed in 3 patients.③ DTI in 3 patients showed that the ADC value of the lesions increased and the FA value decreased.The number of nerve fiber tracts in 2 patients became fewer in the atrophic segments,and the disruption of the nerve fiber tracts was observed in the malacia lesion on T 2 WI in 1 patient.Conclusion There are some characteristics of Hirayama disease in both MRI and clinical manifestations,especially in dynamic position MRI.DTI may provide quantitative evaluation and be helpful for the diagnosis and prognosis of Hirayama disease.
出处
《第三军医大学学报》
CAS
CSCD
北大核心
2013年第15期1620-1623,共4页
Journal of Third Military Medical University
基金
重庆市自然科学基金(CSTC2011jjA10082)~~
关键词
平山病
核磁共振成像
弥散张量成像
Hirayama disease
magnetic resonance imaging
diffusion tensor imaging
