目的:通過(guò)分析平山病的磁共振成像(MRI)表現(xiàn)特點(diǎn)和結(jié)合文獻(xiàn)復(fù)習(xí)，提高其診斷水平。方法:報(bào)道1例經(jīng)臨床證實(shí)的平山病患者的MRI表現(xiàn)，并復(fù)習(xí)文獻(xiàn)，探討MRI表現(xiàn)的相關(guān)發(fā)病機(jī)制及病理解剖基礎(chǔ)。結(jié)果:采用西門(mén)子Sonata 1.5T磁共振機(jī)，先取生理狀態(tài)下頸椎MRI檢查，顯示脊髓前角非對(duì)稱性萎縮和脊髓背側(cè)硬膜外靜脈叢擴(kuò)張；再行屈頸狀態(tài)下MRI檢查，可見(jiàn)頸髓的硬脊膜后壁向前推移，下段頸髓局限受壓變細(xì)征。結(jié)論:平山病有特征性MRI表現(xiàn)，結(jié)合臨床、常規(guī)位和功能位屈頸狀態(tài)的MRI檢查，可以提示平山病。
目的：對(duì)平山病的臨床特點(diǎn)、電生理、影像學(xué)、預(yù)后以及發(fā)病機(jī)制進(jìn)行分析。方法：以“平山病”或“上肢肌萎縮”為主題詞，以中國(guó)數(shù)字期刊總庫(kù)和中華醫(yī)學(xué)會(huì)數(shù)字化期刊為數(shù)據(jù)庫(kù)，共檢索167篇文獻(xiàn)，剔除重復(fù)報(bào)道以及病例資料不全的文獻(xiàn)，對(duì)22篇國(guó)內(nèi)雜志發(fā)表及我院診治的病例共192例患者的臨床資料進(jìn)行分析。結(jié)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(xiàn)多以一側(cè)上肢遠(yuǎn)端肌肉無(wú)力伴肌萎縮，右側(cè)多見(jiàn)，77.6%患者有伸指顫動(dòng)，81.3%有寒冷麻痹。全部患者患肢肌電圖運(yùn)動(dòng)單位電位時(shí)限延長(zhǎng)，95.8%對(duì)側(cè)上肢遠(yuǎn)端肌電圖出現(xiàn)類似的改變。頸部自然位MRI 44.3%發(fā)現(xiàn)低位頸髓萎縮，屈曲位均發(fā)現(xiàn)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(wěn)定狀態(tài)，部分患者經(jīng)頸托治療病情好轉(zhuǎn)。結(jié)論：平山病是一種少見(jiàn)的良性自限性疾病，好發(fā)于青少年，主要表現(xiàn)上肢遠(yuǎn)端不對(duì)稱肌萎縮，早期佩戴頸托可以阻止病情進(jìn)展。
摘要:目的:探討平山病的MRI影像特點(diǎn)及其臨床應(yīng)用價(jià)值。方法:5例臨床確診平山病病例組和10例正常對(duì)照組進(jìn)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(wǎng)膜下腔及硬膜外腔變化情況。結(jié)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(wǎng)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(quán)像及T1WI增強(qiáng)呈高信號(hào),其中1例內(nèi)見(jiàn)血管流空信號(hào)影。對(duì)照組為10例志愿者,自然位: 4例C3~7頸髓形態(tài)、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(wǎng)膜下腔大小與自然位比較無(wú)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(hào)影。結(jié)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(xiàn)。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.
目的探討平山病的臨床特點(diǎn)。 方法分析2010年11月收治的1例平山病患者的臨床表現(xiàn)及輔助檢查，并復(fù)習(xí)該病相關(guān)文獻(xiàn)。 結(jié)果該例患者為青年男性，主要癥狀為雙手握力差，伴肌萎縮，手指伸展時(shí)可見(jiàn)肌束顫動(dòng)。肌電圖示雙上肢大魚(yú)際肌及第一骨間肌神經(jīng)源性損害。腦MRI平掃未見(jiàn)異常。頸椎過(guò)屈位MRI顯示頸5～頸7平面頸髓變細(xì)，椎管變窄，頸4～胸1平面脊髓后方硬膜外脂肪間隙增寬，增強(qiáng)見(jiàn)索樣改變，斑片狀強(qiáng)化及血管流空信號(hào)，符合平山病的診斷。 結(jié)論當(dāng)青少年出現(xiàn)上肢遠(yuǎn)端不對(duì)稱肌無(wú)力伴肌萎縮時(shí)，診斷需考慮平山病，結(jié)合神經(jīng)電生理檢查和屈頸位MRI檢查可明確診斷，佩戴頸托可能有助于延緩病情進(jìn)展。