目的:通过分析平山病的磁共振成像(MRI)表现特点和结合文献复习，提高其诊断水平。方法:报道1例经临床证实的平山病患者的MRI表现，并复习文献，探讨MRI表现的相关发病机制及病理解剖基础。结果:采用西门子Sonata 1.5T磁共振机，先取生理状态下颈椎MRI检查，显示脊髓前角非对称性萎缩和脊髓背侧硬膜外静脉丛扩张；再行屈颈状态下MRI检查，可见颈髓的硬脊膜后壁向前推移，下段颈髓局限受压变细征。结论:平山病有特征性MRI表现，结合临床、常规位和功能位屈颈状态的MRI检查，可以提示平山病。
目的：对平山病的临床特点、电生理、影像学、预后以及发病机制进行分析。方法：以“平山病”或“上肢肌萎缩”为主题词，以中国数字期刊总库和中华医学会数字化期刊为数据库，共检索167篇文献，剔除重复报道以及病例资料不全的文献，对22篇国内杂志发表及我院诊治的病例共192例患者的临床资料进行分析。结果：192例患者，男∶女=6.7∶1，起病年龄平均18.6岁。隐匿起病，表现多以一侧上肢远端肌肉无力伴肌萎缩，右侧多见，77.6%患者有伸指颤动，81.3%有寒冷麻痹。全部患者患肢肌电图运动单位电位时限延长，95.8%对侧上肢远端肌电图出现类似的改变。颈部自然位MRI 44.3%发现低位颈髓萎缩，屈曲位均发现颈髓前移，硬脊膜后壁前移，硬脊膜外间歇增宽。病情在3~4年后处于稳定状态，部分患者经颈托治疗病情好转。结论：平山病是一种少见的良性自限性疾病，好发于青少年，主要表现上肢远端不对称肌萎缩，早期佩戴颈托可以阻止病情进展。
摘要:目的:探讨平山病的MRI影像特点及其临床应用价值。方法:5例临床确诊平山病病例组和10例正常对照组进行颈椎自然位及屈颈位MRI检查,矢状位T1WI、T2WI及轴位T2WI,观察颈髓、蛛网膜下腔及硬膜外腔变化情况。结果:病例组的5例平山病均系16~20岁男性。自然位:5例下位颈髓均萎缩变扁,硬膜外间隙未显示增宽。屈颈位:5例C5~7颈髓前移变扁中,将变扁颈髓又分为上中下三段,以中段最窄,上下段渐移行至正常;C5~7蛛网膜下腔亦变窄,硬脊膜伴随前移;而C4~7硬脊膜后间隙则增宽,呈新月形影,增宽程度分为轻、中、重三度,最重者位于C6椎体平面,T2加权像及T1WI增强呈高信号,其中1例内见血管流空信号影。对照组为10例志愿者,自然位: 4例C3~7颈髓形态、大小基本一致,6例颈髓自颈3逐渐移行与胸1脊髓其大小一致;屈颈位:颈髓和蛛网膜下腔大小与自然位比较无明显变化,硬膜后间隙自C3平面向下延至T1平面,T2WI上呈均匀线样高信号影。结论:下位颈髓萎缩变扁,屈颈位颈髓及硬脊膜前移、硬脊膜后间隙增宽呈新月形影等,是临床诊断平山病较特征性的MRI表现。Abstract: Objective: To evaluate clinical value and MRI features of Hirayama disease. Methods: Five cases of hirayama disease, which had been clinically confirmed using siemens sonata 1.5T MRI scan, physiological condition and flexional condition, Sagittal view T1WI, T2WI and Axial View T2WI, and GdDTPA enhanced examination, for MRI changes of spinal cord, subarachnoid cavity, duramater of spinal membrae and extra dural space, etc were studied. Results: In case group of 5 cases of hirayama disease, age was mainly in 16–20 years old, All of 5 cases were men. Which were pressed and become thinner of spinal cord, strictic changes of subarachnoid cavity, new moony shape and enlargement changes and pushed forward of extra duramater space, and higher intensity signal of GdDTPA enhancement, and vascular flow effect (one case ) in C5–C7. but also, for contrast group 10 cases of normal volunteer, physiological condition:4 cases in cervical spinal cord with shape and structure were uniformity, and duramater, subarachnoid cavity, extra duramater space etc in C3–C7 were abnormal. Six cases in cervical spinal cord with shape and structure gradully changed from C3 to T1; flexional condition: 10 cases of MRI changes of spinal cord, subarachnoid cavity were as same as it in physiological condition,all of T2 WI, higher intensity signal were homogeneous of extraduramater space in C3–T1. Conclusion: The feature findings of cervical spinal cord became thinner, and cervical cord, durameter pussed forward, new moony shape and enlargment of extradurameter space, vascular flow effusion, etc in MRI were useful value for clinical diagnosis.
ObjectiveTo investigate the clinical features of Hirayama disease (HD). MethodsWe analyzed the clinical manifestations and assistant examination results of one patient with HD diagnosed in November, 2010. In addition, we reviewed the related literatures. ResultsThe patient was a young man, and the main symptoms were bilateral hand weakness with muscular atrophy. The muscle bundles trembled when he stretched his fingers. The electromyography showed neurogenic damage of both the thenar muscles and the first interosseous muscles. Brain magnetic resonance imaging (MRI) showed normal; cervical spine flexion MRI showed thinning cervical spinal cord with spinal canal narrowing at C5-C7 plane. In addition, epidural fat gap widened at C4-T1 plane, and enhanced scan showed cord-like changes, patchy strengthening and blood flow void shadow. ConclusionWhen asymmetric distal upper limb muscle weakness with muscle atrophy appears in adolescents, HD should be considered. The combination of neuroelectrophysiological examination and cervical spine flexion MRI scan is helpful for diagnosis. Wearing cervical collar may slow disease progression.