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[目的]探讨平山病患者颈椎前路手术治疗初步效果及平山病的手术缓解机制.[方法]回顾性分析2012年11月～2016年5月接受颈椎前路手术治疗且资料完整的平山病患者13例,所有患者经神经内科确诊,并除外运动神经元疾病,且保守治疗无效,病情持续进展严重影响患者生活质量.其中10例采用颈椎前路单纯钛板螺钉内固定术,3例因存在明显颈椎间盘退变突出,采用颈椎前路ACDF手术治疗.采用改良JOA评分评价术前和末次随访时神经功能,此外,测量患者颈椎后突(C2-7Cobb角),颈椎活动度(ROM)、颈椎最大屈曲角度(MFA).[结果]所有患者随访7 ～49个月,平均32.7±15个月.末次随访时,术后伸指震颤症状减轻,但症状未完全消失;肌肉萎缩症状改善不明显.改良JOA评分由术前的(12.85±1.70)增加至末次随访时的(14.38±1.46),差异有统计学意义(P=0.021).术后屈曲位颈椎MRI可以观察到脊髓前方压迫缓解,并有局部脊髓膨胀表现.术前与末次随访时相比较,C2～7 Cobb角[(10.69±11.12)°vs (8.08±8.14)°,P=0.50]差异无统计学意义;但颈椎活动度[(71.92±15.65)° vs (37.69±11.18)°,P＜0.001]和颈椎最大屈曲角度[(37.08±6.80)° vs (20.15±8.65),P＜0.001]显著减少,差异有统计学意义. [结论]颈椎前路内固定或融合手术能够限制平山病患者颈椎过度前屈,从而减少颈椎活动时脊髓前侧的反复撞击,减缓甚至停止上肢远端肌萎缩的进展.“,”[Objective] To evaluate the primary clinical outcome of Hirayama disease treated by anterior cervical surgery,and explore mechanism of the treatment.[Methods] A retrospective study was conducted on 13 patients who suffered from Hirayama disease with complete clinical data,received surgical treatment between December 2012 and May 2016.All the patients were definitively diagnosed by the neurological specialists in our hospital after the motor neuron diseases excluded.Conservative treatment was invalid,and the disorder continued to progress with significant deterioration of the quality of life in all the 13 patients.Among them,10 patients underwent anterior cervical fixation (ACF),while the other 3 patients accompanied with cervical disc degeneration received anterior cervical discectomy fusion (ACDF).The neurologic function was assessed using the modified Japanese Orthopedic Association score.In addition,cervical motion was evaluated with cervical curvature in Cobb angle of C2-7,range of motion (ROM) and the maximum flexion angle (MFA) which were measured with the dynamic lateral radiographs before surgery and at latest follow-up.[Results] All the patients were followed up for 7 to 49 months with an average of 32.7± 15.1 months.At the latest follow up,the finger tremors alleviated in all the patients instead of disappeared completely.However,no considerable improvement in muscle atrophy was noted in any of them.The modified JOA scores statistically increased from (12.85±1.70) before operation to (14.38±1.46) at the latest follow up (P=0.021).The MRI revealed remarkable relief of anterior compression on the spinal cord associated with the cord rebounding.Compared measurements before operation with those at the latest follow up,the C2～7 Cobb angle [(10.69±11.12)° versus (8.08±8.14)°,P=0.500] varied without a statistical significance,however,the ROM [(71.92 ±15.65)° versus (37.69±11.18)°,P＜0.001] and MFA [(37.08±6.80)° versus (20.15±8.65) °,P＜0.001] statistically decreased.[Conclusions] ACF or ACDF does limit excessive flexion of the cervical spine in Hirayama disease.It maybe play role in retarding,even terminating progression of muscular atrophy of distal upper limb in Hirayama disease due to reducing the repeated impacts on the anterior spinal cord while cervical motion.