选择性脊神经后根切断术治疗遗传性痉挛性截瘫4例报告并文献复习

Selective posterior rhizotomy for hereditary spastic paraplegia:4 cases report and literature review

目的探讨选择性脊神经后根切断(SPR)术对遗传性痉挛性截瘫(HSP)患者的治疗效果。方法分析4例HSP患者的临床资料;采用SPR手术及术后康复的综合治疗方案。手术方式为选择性腰骶段脊神经后根切断并椎板复位。对比治疗前与治疗后12个月时,患者的改良Asworth痉挛分级评分、关节活动度和Hoffer步行能力分级。结果4例HSP患者均表现明显的痉挛状态,步态困难;其中3例为单纯型HSP,1例为复杂型HSP。4例患者SPR术后12个月的痉挛分级评分显示,MAS评分均值显著低于术前(均P<0.01);股角活动度平均改善11.3°,腘窝角活动度改善20.5°,足背屈角活动度改善4.0°。2例单纯型HSP患儿Hoffer步行活动能力得到改善,由Ⅱ级提高到Ⅲ级;1例单纯型HSP成人患者则没有改善,另外1例复杂型HSP患儿的步态和平衡变差。3例单纯型HSP患者的肌力无明显减退,1例复杂型HSP患者的肌力有减退。结论SPR结合术后康复,可以有效缓解单纯型HSP患者的痉挛状态,改善患者的步态和移动能力。通过SPR缓解痉挛,有可能降低HSP患者肢体废用性肌萎缩的发生率。

Objective To explore the value of selective posterior rhizotomy(SPR)in the treatment of hereditary spastic paraplegia(HSP).Methods The data of 4 patients with HSP were selected,and the comprehensive treatment plan of SPR and postoperative rehabilitation was adopted.Patients before treatment and 12 months after treatment were compared in the changes of modified as worth spasticity grade,joint activity grade and Hoffer walking ability grade.The operative method was selective posterior rhizotomy and replacement laminoplasty at lumbosacral segment.Results 4 cases of HSP were with prominent spasticity and difficult gait,of which 3 simple HSP and 1 complex HSP.After 12 months of SPR,the spasticity in 4 patients showed that the mean value of MAS was significantly lower than that before SPR(all P<0.01).The mean improvement of femoral angle activity was 11.3°,popliteal angle activity was 20.5°,and dorsiflexion angle activity was 4.0°.The Hoffer walking ability in 2 simple HSP children was improved from levelⅡto levelⅢwhile that in 1 simple HSP adult case wasn't.In the other complex HSP case,the gait and balance became worse.3 simple HSP cases had no significant decrease in muscle strength,while 1 complex HSP case's muscle strength decreased continuously.Conclusions SPR combined with postoperative rehabilitation can effectively relieve the spasticity of simple HSP patients,improve their gait and mobility.It is possible to reduce the incidence of disuse atrophy in HSP patients by SPR.