屈颈MRI对青年性上肢远端肌萎缩症的诊断价值

The diagnosis of a fully flexed neck position MRI diagnosis in juvenile muscular atrophy of the distal upper extremity

目的评价屈颈MRI对青年性上肢远端肌萎缩症的诊断价值。方法男性患者5例,平均年龄21岁,临床表现为一侧或两侧上肢远端肌萎缩。对照组为健康志愿者,21岁男性8例。2组均行常规及屈颈颈椎MR平扫,矢状、轴面SE T1 WI、T2WI、液体衰减反转恢复(FIAIR)序列扫描。结果常规颈椎扫描:5例患者下段颈髓变细;屈颈位MR扫描:下颈段颈6以下脊髓前屈、变扁平,矢状径4～6 mm,硬膜囊后壁前移,硬膜后间隙明显增宽,可见多发条状、迂曲流空信号影及软组织信号。对照组:常规扫描,下颈段脊髓(颈6-胸2)可见颈膨大,屈颈位脊髓略变细(6～7 mm),硬膜囊后壁无前移,硬膜后间隙未见扩张血管影。结论屈颈MRI有助于显示下颈段脊髓及硬膜囊改变,结合临床资料可准确诊断青年性上肢远端肌萎缩症。

Objective To investigate the value of the diagnosis of MRI during neck flexion in juvenile muscular atrophy of the distal upper extremity. Methods Five young male patients （ mean age 21 years old） with clinical and electrophysiological alterations were pedormed MR examination with routine neck position and a fully flexed neck position. Eight age-match young men were examined as control subjects. SE T1WI, T2WI, Fluid-attenuated inversion recovery （FLAIR） sequences were scanned. Results A distinctive finding in the disorder was forward displacement of the cervical dural sac, compressive flattening of the lower cervical cord during neck flexion and flow void in the posterior epidural space. The forward displacement was significantly greater in patients than in age-matched control subjects. Conclution Flexed neck position MRI is helpful to find radiological abnormalities of the lower cervical dural sac and spinal cord, which were combined with clinical disorder to diagnose juvenile muscular atrophy of the distal upper extremity.