• 四川大學(xué)華西醫(yī)院神經(jīng)內(nèi)科,四川成都 610041;

目的:對平山病的臨床特點(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,以中國數(shù)字期刊總庫和中華醫(yī)學(xué)會數(shù)字化期刊為數(shù)據(jù)庫,共檢索167篇文獻(xiàn),剔除重復(fù)報道以及病例資料不全的文獻(xiàn),對22篇國內(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)端肌肉無力伴肌萎縮,右側(cè)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(yùn)動單位電位時限延長,95.8%對側(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)端不對稱肌萎縮,早期佩戴頸托可以阻止病情進(jìn)展。

引用本文: 周波,周東. 平山病國內(nèi)文獻(xiàn)報道192例分析. 華西醫(yī)學(xué), 2009, 24(2): 409-412. doi: 復(fù)制

1. Hirayama K,Toyokura Y,Tsubaki T.Juvenile muscular atrophy of unilateral upper extremity:a new clinical entity[J].Psychiatry Neurol Jpn,1959,61:2190 -2198..
2. Hirayama K.Juvenile muscular atrophy of distal upper extremity(Hirayama disease)[J].Internal Medicine,2000,39,(4):283-290..
3. 張穎冬,鄧榮昆.青少年單側(cè)上肢遠(yuǎn)端肌萎縮癥(附10例報告)[J].臨床神經(jīng)病學(xué)雜志,1990,3(2):9495..
4. 王醒華.青年上肢遠(yuǎn)端肌萎縮癥1例[J].中國冶金工業(yè)醫(yī)學(xué)雜志,1996,13(4):249..
5. 謝美蘊(yùn).青少年上肢遠(yuǎn)端肌萎縮癥2例[J].中國實(shí)用內(nèi)科雜志,1997,17(11):682..
6. 李作漢,張平,陳詒,等.青年上肢遠(yuǎn)端肌萎縮癥的臨床、肌電圖及肌肉病理的研究[J].中華神經(jīng)精神科雜志,1994,27(3):131-134..
7. 楊曉蘇,肖波,張麗芳,等.青年型單上肢遠(yuǎn)端肌萎縮癥[J].中國神經(jīng)精神疾病雜志,2002,28(3):227-228..
8. 許蕾,黃勃源,李彩英.平山病4例臨床和影像學(xué)分析[J].中國神經(jīng)精神疾病雜志,2006,32(1):23-26..
9. 曹建,方琪,董萬利.青年型上肢遠(yuǎn)端肌萎縮癥2例[J].實(shí)用醫(yī)學(xué)雜志,2006,22(18):2111..
10. 王向波.右手肌肉萎縮1年余 平山病(家族性)[J].中國神經(jīng)精神疾病雜志,2006,32(3):附1-3..
11. 劉懷軍,李彩英,賀丹,等.屈頸MRI對青年性上肢遠(yuǎn)端肌萎縮癥的診斷價值[J].中華放射學(xué)雜志,2006,40(7):745-747..
12. 高鴻興,鐘長揚(yáng),王璐,等.平山病誤診為運(yùn)動神經(jīng)元病1例報告[J].臨床誤診誤治,2007,20(3):49-50..
13. 傅 瑜,樊東升,張 俊,等.平山病的臨床及頸磁共振成像影像學(xué)特點(diǎn)[J].北京大學(xué)學(xué)報(醫(yī)學(xué)版),2007,39(2):189-192..
14. 朱丹,張繼華,陳嘉峰,等.平山病1例報告(附發(fā)病30年后肌電改變)[J].卒中與神經(jīng)疾病雜志,2007,24(5):624-625..
15. 孫小添,馬英.平山病1例[J].現(xiàn)代中西醫(yī)結(jié)合雜志,2007,16(21):3052-3053..
16. 陳興泳,唐榮華,唐洲平,等.平山病1例及文獻(xiàn)復(fù)習(xí)[J].臨床薈萃,2007,22(17):1256-1257..
17. 吳永勝.平山病誤診4例分析[J].中國誤診學(xué)雜志,2007,7(15):3548-3549..
18. 張亦工,王彥偉,劉志松,等.平山病誤診為脊髓型頸椎病1例分析[J].中國誤診學(xué)雜志,2007,7(9):2049-2050..
19. 徐家立,耿磊鈺,劉玉璽,等.以雙上肢肌萎縮為表現(xiàn)的平山病一例[J].中華神經(jīng)科雜志,2007,40(12):841..
20. 黃忠華.平山病1例[J].中國誤診學(xué)雜志,2007,7(11):2676..
21. 董曼麗,劉江,翟明.平山病6例分析[J].中國誤診學(xué)雜志,2008,8(4):973-974..
22. 王文祥,甘明珠,姜勇,等.青年上肢遠(yuǎn)端不對稱性肌萎縮1例臨床分析[J].中國實(shí)用神經(jīng)疾病雜志,2008,11(2):88-90..
23. 李志軍,胡曉晴,唐娜.平山病的臨床神經(jīng)電生理學(xué)特點(diǎn)[J].神經(jīng)損傷與功能重建,2008,3(4):422-425..
24. 劉麗,黃旭升,陳朝暉,等.平山病的肌電圖及神經(jīng)傳導(dǎo)特點(diǎn)[J].解放軍醫(yī)學(xué)雜志,2008,33(1):88-90..
25. HirayamaK,T okumaruY.Cervical dural sac and spinal cord injuvenile Muscular atrophy of distal upper extremity[J].Neurology,2000,54(10):19 22-1926..
26. Misra UK,Kalita J,Mishra VN,et al.A clinical,magnetic resonance imaging,and survival motor neuron gene deletion study of Hirayama disease[J].Arch Neurol,2005,62:120-123..
27. Peiris JB,Seneviratne KN,Wickremasinghe HR,et al.Non familial juvenile distal spinal muscular atrophy of upper extremity[J].J Neurol Neurosurg Psychiatry,1989,52:314 -319..
28. Tan CT.Juvenile muscular atrophy of distal upper extremities[J].J Neurol Neurosurg Psychiatry,1985,48:285-286..
29. Huang YC,Ro LS,Chang HS,et al.A clinical study of Hirayama disease in Taiwan[J].Muscle Nerve,2008,37(5):576-582..
30. Kao KP,Wu ZA,Chern CM.Juvenile lower cervical spinal muscular atrophy in Taiwan:report of 27 Chinese cases[J].Neuroepidemiology,1993,12:331-335..
31. Misra UK,Kalita J.Central motor conduction in Hirayama disease[J].Electroencephalogr Clin Neurophysiol,1995,97(2):73-76..
32. Rowin J,Meriggioli MN,Cochran EJ.Monomelic amyotrophy with late progression[J].Neuromuscul Disord,2001,11(3):305..
33. Kuwabara S,Nakajima M,Hattori T,Hirayama K.Electrophysiology of juvenile muscular atrophy of unilateral upper limb(Hirayama’s disease)[J].Rinsho Shinkeigaku(Clin Neurol),1999,39:508-512..
34. Hirayama K,Tomonaga M,Kitano K,et al.The first autopsy case of “juvenile muscular atrophy of unilateral upper extremity”[J].Shinkei Naika(Neurol Med),1985,22:85-88..
35. Schroder R,Keller E,F(xiàn)lacke S,et al.MRI findings in Hirayama’s disease:flexioninduced cervical myelopathy or intrinsic motor neuron disease[J]? J Neurol,1999,246:1069-1074..
36. Hirayama K,Toyokura Y.Cervical dural sac and spinal cord in Juvenile muscular atrophy of distal upper extremity[J].Neurology,2000,54(10):1922-1926..
37. Baba Y,Nakajima M,Utsunomiya H,et al.Magnetic resonanec imaging of thoracic epidural venous dilation in Hirayama disease[J].Neurology,2004,62(8):1426-1428..
38. Chen C J,Hsu H L,Tseng Y C,et al.Hirayama flexion myelopathy neutralposition MR imaging findingsimportance of loss of attachment[J].Radiology,2004,231(1):39-44.
  1. 1. Hirayama K,Toyokura Y,Tsubaki T.Juvenile muscular atrophy of unilateral upper extremity:a new clinical entity[J].Psychiatry Neurol Jpn,1959,61:2190 -2198..
  2. 2. Hirayama K.Juvenile muscular atrophy of distal upper extremity(Hirayama disease)[J].Internal Medicine,2000,39,(4):283-290..
  3. 3. 張穎冬,鄧榮昆.青少年單側(cè)上肢遠(yuǎn)端肌萎縮癥(附10例報告)[J].臨床神經(jīng)病學(xué)雜志,1990,3(2):9495..
  4. 4. 王醒華.青年上肢遠(yuǎn)端肌萎縮癥1例[J].中國冶金工業(yè)醫(yī)學(xué)雜志,1996,13(4):249..
  5. 5. 謝美蘊(yùn).青少年上肢遠(yuǎn)端肌萎縮癥2例[J].中國實(shí)用內(nèi)科雜志,1997,17(11):682..
  6. 6. 李作漢,張平,陳詒,等.青年上肢遠(yuǎn)端肌萎縮癥的臨床、肌電圖及肌肉病理的研究[J].中華神經(jīng)精神科雜志,1994,27(3):131-134..
  7. 7. 楊曉蘇,肖波,張麗芳,等.青年型單上肢遠(yuǎn)端肌萎縮癥[J].中國神經(jīng)精神疾病雜志,2002,28(3):227-228..
  8. 8. 許蕾,黃勃源,李彩英.平山病4例臨床和影像學(xué)分析[J].中國神經(jīng)精神疾病雜志,2006,32(1):23-26..
  9. 9. 曹建,方琪,董萬利.青年型上肢遠(yuǎn)端肌萎縮癥2例[J].實(shí)用醫(yī)學(xué)雜志,2006,22(18):2111..
  10. 10. 王向波.右手肌肉萎縮1年余 平山病(家族性)[J].中國神經(jīng)精神疾病雜志,2006,32(3):附1-3..
  11. 11. 劉懷軍,李彩英,賀丹,等.屈頸MRI對青年性上肢遠(yuǎn)端肌萎縮癥的診斷價值[J].中華放射學(xué)雜志,2006,40(7):745-747..
  12. 12. 高鴻興,鐘長揚(yáng),王璐,等.平山病誤診為運(yùn)動神經(jīng)元病1例報告[J].臨床誤診誤治,2007,20(3):49-50..
  13. 13. 傅 瑜,樊東升,張 俊,等.平山病的臨床及頸磁共振成像影像學(xué)特點(diǎn)[J].北京大學(xué)學(xué)報(醫(yī)學(xué)版),2007,39(2):189-192..
  14. 14. 朱丹,張繼華,陳嘉峰,等.平山病1例報告(附發(fā)病30年后肌電改變)[J].卒中與神經(jīng)疾病雜志,2007,24(5):624-625..
  15. 15. 孫小添,馬英.平山病1例[J].現(xiàn)代中西醫(yī)結(jié)合雜志,2007,16(21):3052-3053..
  16. 16. 陳興泳,唐榮華,唐洲平,等.平山病1例及文獻(xiàn)復(fù)習(xí)[J].臨床薈萃,2007,22(17):1256-1257..
  17. 17. 吳永勝.平山病誤診4例分析[J].中國誤診學(xué)雜志,2007,7(15):3548-3549..
  18. 18. 張亦工,王彥偉,劉志松,等.平山病誤診為脊髓型頸椎病1例分析[J].中國誤診學(xué)雜志,2007,7(9):2049-2050..
  19. 19. 徐家立,耿磊鈺,劉玉璽,等.以雙上肢肌萎縮為表現(xiàn)的平山病一例[J].中華神經(jīng)科雜志,2007,40(12):841..
  20. 20. 黃忠華.平山病1例[J].中國誤診學(xué)雜志,2007,7(11):2676..
  21. 21. 董曼麗,劉江,翟明.平山病6例分析[J].中國誤診學(xué)雜志,2008,8(4):973-974..
  22. 22. 王文祥,甘明珠,姜勇,等.青年上肢遠(yuǎn)端不對稱性肌萎縮1例臨床分析[J].中國實(shí)用神經(jīng)疾病雜志,2008,11(2):88-90..
  23. 23. 李志軍,胡曉晴,唐娜.平山病的臨床神經(jīng)電生理學(xué)特點(diǎn)[J].神經(jīng)損傷與功能重建,2008,3(4):422-425..
  24. 24. 劉麗,黃旭升,陳朝暉,等.平山病的肌電圖及神經(jīng)傳導(dǎo)特點(diǎn)[J].解放軍醫(yī)學(xué)雜志,2008,33(1):88-90..
  25. 25. HirayamaK,T okumaruY.Cervical dural sac and spinal cord injuvenile Muscular atrophy of distal upper extremity[J].Neurology,2000,54(10):19 22-1926..
  26. 26. Misra UK,Kalita J,Mishra VN,et al.A clinical,magnetic resonance imaging,and survival motor neuron gene deletion study of Hirayama disease[J].Arch Neurol,2005,62:120-123..
  27. 27. Peiris JB,Seneviratne KN,Wickremasinghe HR,et al.Non familial juvenile distal spinal muscular atrophy of upper extremity[J].J Neurol Neurosurg Psychiatry,1989,52:314 -319..
  28. 28. Tan CT.Juvenile muscular atrophy of distal upper extremities[J].J Neurol Neurosurg Psychiatry,1985,48:285-286..
  29. 29. Huang YC,Ro LS,Chang HS,et al.A clinical study of Hirayama disease in Taiwan[J].Muscle Nerve,2008,37(5):576-582..
  30. 30. Kao KP,Wu ZA,Chern CM.Juvenile lower cervical spinal muscular atrophy in Taiwan:report of 27 Chinese cases[J].Neuroepidemiology,1993,12:331-335..
  31. 31. Misra UK,Kalita J.Central motor conduction in Hirayama disease[J].Electroencephalogr Clin Neurophysiol,1995,97(2):73-76..
  32. 32. Rowin J,Meriggioli MN,Cochran EJ.Monomelic amyotrophy with late progression[J].Neuromuscul Disord,2001,11(3):305..
  33. 33. Kuwabara S,Nakajima M,Hattori T,Hirayama K.Electrophysiology of juvenile muscular atrophy of unilateral upper limb(Hirayama’s disease)[J].Rinsho Shinkeigaku(Clin Neurol),1999,39:508-512..
  34. 34. Hirayama K,Tomonaga M,Kitano K,et al.The first autopsy case of “juvenile muscular atrophy of unilateral upper extremity”[J].Shinkei Naika(Neurol Med),1985,22:85-88..
  35. 35. Schroder R,Keller E,F(xiàn)lacke S,et al.MRI findings in Hirayama’s disease:flexioninduced cervical myelopathy or intrinsic motor neuron disease[J]? J Neurol,1999,246:1069-1074..
  36. 36. Hirayama K,Toyokura Y.Cervical dural sac and spinal cord in Juvenile muscular atrophy of distal upper extremity[J].Neurology,2000,54(10):1922-1926..
  37. 37. Baba Y,Nakajima M,Utsunomiya H,et al.Magnetic resonanec imaging of thoracic epidural venous dilation in Hirayama disease[J].Neurology,2004,62(8):1426-1428..
  38. 38. Chen C J,Hsu H L,Tseng Y C,et al.Hirayama flexion myelopathy neutralposition MR imaging findingsimportance of loss of attachment[J].Radiology,2004,231(1):39-44.