摘要:目的:探討平山病的MRI影像特點及其臨床應用價值。方法:5例臨床確診平山病病例組和10例正常對照組進行頸椎自然位及屈頸位MRI檢查,矢狀位T1WI、T2WI及軸位T2WI,觀察頸髓、蛛網膜下腔及硬膜外腔變化情況。結果:病例組的5例平山病均系16~20歲男性。自然位:5例下位頸髓均萎縮變扁,硬膜外間隙未顯示增寬。屈頸位:5例C5~7頸髓前移變扁中,將變扁頸髓又分為上中下三段,以中段最窄,上下段漸移行至正常;C5~7蛛網膜下腔亦變窄,硬脊膜伴隨前移;而C4~7硬脊膜后間隙則增寬,呈新月形影,增寬程度分為輕、中、重三度,最重者位于C6椎體平面,T2加權像及T1WI增強呈高信號,其中1例內見血管流空信號影。對照組為10例志愿者,自然位: 4例C3~7頸髓形態、大小基本一致,6例頸髓自頸3逐漸移行與胸1脊髓其大小一致;屈頸位:頸髓和蛛網膜下腔大小與自然位比較無明顯變化,硬膜后間隙自C3平面向下延至T1平面,T2WI上呈均勻線樣高信號影。結論:下位頸髓萎縮變扁,屈頸位頸髓及硬脊膜前移、硬脊膜后間隙增寬呈新月形影等,是臨床診斷平山病較特征性的MRI表現。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.
目的:通過分析平山病的磁共振成像(MRI)表現特點和結合文獻復習，提高其診斷水平。方法:報道1例經臨床證實的平山病患者的MRI表現，并復習文獻，探討MRI表現的相關發病機制及病理解剖基礎。結果:采用西門子Sonata 1.5T磁共振機，先取生理狀態下頸椎MRI檢查，顯示脊髓前角非對稱性萎縮和脊髓背側硬膜外靜脈叢擴張；再行屈頸狀態下MRI檢查，可見頸髓的硬脊膜后壁向前推移，下段頸髓局限受壓變細征。結論:平山病有特征性MRI表現，結合臨床、常規位和功能位屈頸狀態的MRI檢查，可以提示平山病。
目的：對平山病的臨床特點、電生理、影像學、預后以及發病機制進行分析。方法：以“平山病”或“上肢肌萎縮”為主題詞，以中國數字期刊總庫和中華醫學會數字化期刊為數據庫，共檢索167篇文獻，剔除重復報道以及病例資料不全的文獻，對22篇國內雜志發表及我院診治的病例共192例患者的臨床資料進行分析。結果：192例患者，男∶女=6.7∶1，起病年齡平均18.6歲。隱匿起病，表現多以一側上肢遠端肌肉無力伴肌萎縮，右側多見，77.6%患者有伸指顫動，81.3%有寒冷麻痹。全部患者患肢肌電圖運動單位電位時限延長，95.8%對側上肢遠端肌電圖出現類似的改變。頸部自然位MRI 44.3%發現低位頸髓萎縮，屈曲位均發現頸髓前移，硬脊膜后壁前移，硬脊膜外間歇增寬。病情在3~4年后處于穩定狀態，部分患者經頸托治療病情好轉。結論：平山病是一種少見的良性自限性疾病，好發于青少年，主要表現上肢遠端不對稱肌萎縮，早期佩戴頸托可以阻止病情進展。