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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) characteristics of Pingshan disease and explore the diagnostic value of MRI for Pingshan disease. Methods The clinical, electromyographic and imaging data of 13 young patients with Pyeongshan disease were retrospectively analyzed. The MRI examination included plain and flexion neck scan, T1WI enhancement and diffusion tensor imaging (DTI). Results Thirteen patients had progressive muscular atrophy and muscle weakness in unilateral or bilateral upper limbs. Electromyographic signs of muscle involvement are neurogenic damage. Natural MRI manifestations: ① 13 cases of cervical curvature disorder; cervical degeneration, bulging or prominent. ② The level of spinal cord involvement mainly lies in the lower cervical cord; the horizontal axis shows the atrophy and flattening of the cervical cord in the lower segment; the high signal of T2WI in 7 spinal cord segments with ischemic necrosis and glial hyperplasia. The flexion-neck MRI showed ① the posterior wall of the dural sac was advanced and the corresponding segment of the cervical cord was compressed; the dural space was widened after the dural sac; 9 cases saw curved or punctate vascular emptying in the widening gap shadow. ② 3 cases showed enhanced scrotal dorsal epidural space was significantly enhanced. ③ In 3 cases of DTI, the ADC value of cervical spinal cord increased and the FA value decreased. In 2 cases, the nerve fiber bundles became thinner and thinner, and in 1 case, the nerve fiber bundles in flat area showed discontinuity. Conclusion The clinical manifestations and MRI manifestations of Pingshan have some characteristics. MRI of dynamic position is more characteristic. DTI can quantitatively evaluate the structure and function of nerve fiber bundles in lesions and help to diagnose and prognosis.