IEEE Trans:肌电控制的电动液压手矫形器治疗一例杜氏肌营养不良患者

2021-05-20 MedSci原创 MedSci原创

杜氏肌肉营养不良症( Duchenne Muscular Dystrophy,DMD),乃遗传性肌肉萎缩病。它的基因( Dystrophin gene)存在于X 性染色体中( Xp21 ),

杜氏肌肉营养不良症( Duchenne Muscular Dystrophy,DMD),乃遗传性肌肉萎缩病。它的基因( Dystrophin gene)存在于X 性染色体中( Xp21 ),因此它是透过性连锁式隐性遗传型态传播的。男性只有一个X性染色体,因此病患者大多为男性;若女性的一对X性染色体中其一个携有异变的 Dystrophin 基因,她便成为一个 DMD 的携带者,她的儿子有二分一的机会成为病患者,她的女儿则有二分一机会成为 DMD基因携带者。杜氏肌肉营养不良症会导致肌纤维无力、萎缩。主要是进行性的肌肉无力和萎缩。多数是在4岁以前发病,比如开始发现男孩走路摇摇摆摆,之后就发现他上台阶特别费劲,他起身也很困难,如果要是想起来,需要蹭着身起来。主要是肌肉无力,少部分还会连累心脏,还有的病人会影响智力,包括落后和低下。

手在日常生活活动(ADL)中起着核心作用。ADL需要足够的抓地力和额外的调节能力,不需要额外的努力或疲劳。在DMD中,手握力在12岁后显著下降,同时伴有早期疲劳,导致越来越多的人无法执行ADL。在DMD患者中,没有训练握力调节或手部疲劳减轻的证据。现有的研究强调了手的功能在DMD中的重要性,以及需要更多关于抓取力的研究,以显示早期干预可能减缓恶化过程。越来越多的证据表明,需要对患有DMD的人进行全面和多学科的康复。动态手矫形器是一种很好的技术可用于治疗患者。动态手矫形器需要一种直观的方式来解码用户的意图,并控制最终的治疗方式。 对于患有肌营养不良症的参与者来说,手部运动的运动意图解码显示出了有希望的结果。液压传动具有高能量密度和透明力传递,以及柔性结构,以尽量减少皮肤上的压力分量,适用于DMD治疗。本系统定义为SymbiHand。本研究的目的是评估SymbiHands系统在临床个案研究中协助DMD患者的抓取功能的潜力。

Fig. 1. - The participant with DMD grasping the sensorized object while wearing SymbiHand orthosis. 1) SymbiHand, consisting of four finger modules. 2) The thermoplastic hand splint, used to stabilize the wrist and thumb while providing an anchoring surface for the four finger modules. 3) Wireless sEMG sensor, placed on the extensor digitorum communis muscle. 4) The cylindrical sensorized object, used for measuring grasping force.

患有DMD的受试者在佩戴手部矫形器 1) 四个手指模块。2) 热塑性手夹板3) 无线表面肌电传感器4) 圆柱形传感物体

一名23岁被诊断为DMD的男性参与者,参与了这项研究。他不能把手举到嘴边,但可以用手拿笔或从桌上捡硬币。上肢功能(PUL)评分为8分,最高为74分)。他抓握力大幅下降。SymbiHand由信号、能量和机械领域的组件组成。它通过与用户交换信号和物理交互来帮助用户执行任务,而用户又反过来与环境交互。参与者的意图通过直接的表面肌电控制实时解码,结合一阶导纳模型,实现手矫形器的自动打开/关闭。一个圆柱形物体传感器被用来测量抓取力,作为实时力跟踪任务的输入。表面肌电控制被用来解码一个单自由度(DOF)手部运动(开/关)。液压活塞泵被用来将电能转换成液压形式的机械功。参与者参加了两个环节,第一个环节包括在职业治疗师的帮助下制作热塑性手夹板和测量定制手的手指。在第二环节中,为了评估symbhand是否有可能在日常生活活动中提供帮助,要求参与者使用抓取力作为输入执行力跟踪任务。为此,在无手矫形器和有手矫形器的情况下,对感测的圆柱形物体进行开拳圆柱形抓握。

SymbiHand系统能够将患者对圆柱形物体的最大抓取力从2.4 N增加到8 N,患者屈肌肌肉活动略有增加。屈肌活动的轻微增加可能是戴上矫形器的结果。受试者经历了轻微的挛缩通过限制了手的活动范围,以适应受试者的舒适极限。以食指为例,没有/有手矫形器时的最大屈曲角度约为46°°/51°。参与者表示手指模块感觉不舒服。尽管经过抛光处理,3D打印材料仍感觉粗糙,边缘有些粗糙。由于受试者的手指和皮肤比健康人敏感得多,手指关节掌侧的皮肤折痕处会有割伤感。 

这个案例研究表明,一个患有DMD的个体在戴着一只手的同时,握力增强。结果表明,随着握力的增加,在力追踪任务中肌肉活动减少。然而,在日常环境中使用时,需要进行调整,以便于更舒适地佩戴设备,并减少由于其总质量和质量分布而产生的不利影响。

R. A. Bos, K. Nizamis, B. F. J. M. Koopman, J. L. Herder, M. Sartori and D. H. Plettenburg, "A Case Study With Symbihand: An sEMG-Controlled Electrohydraulic Hand Orthosis for Individuals With Duchenne Muscular Dystrophy," in IEEE Transactions on Neural Systems and Rehabilitation Engineering, vol. 28, no. 1, pp. 258-266, Jan. 2020, doi: 10.1109/TNSRE.2019.2952470.

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    2021-10-10 bsmagic9140
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    2021-05-21 yangchou

    肌营养不良的**。

    0

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    2021-05-20 1209e435m98(暂无昵称)

    学习了,谢谢分享

    0

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肌脂蛋白(SLN)是肌肉/内质网(SR)Ca 2+ ATP酶(SERCA)的抑制剂,在杜氏肌营养不良(DMD)患者和动物模型中肌肉里表达异常升高。本研究中,研究人员发现减少SLN的水平可改善重症肌营养不良蛋白/肌萎缩蛋白双重突变体(mdx:utr - / -)DMD模型小鼠的营养不良。SLN基因的一个等位基因的种系失活可使SLN的表达正常化,恢复SERCA的功能,减轻骨骼肌和心脏病理,改善肌肉再生

Lancet:长期糖皮质激素疗法对杜氏肌营养不良患者疾病里程碑和死亡率的影响

研究认为长期的糖皮质激素治疗方案可降低杜氏肌营养患者的疾病进展风险,延长患者生存期

PNAS:如何无创监测Duchenne肌营养不良症疾病进展

在最近的一项研究中,来自斯坦福大学医学院的研究人员描述了一种在DMD的mdx模型中无创评估疾病进展的新方法。

Nat Commun:DMD患者的新希望:BETs抑制可减轻氧化应激,保护肌肉完整性

杜氏肌肉萎缩症(DMD)是最常见的肌肉萎缩症形式。这种X-linked隐性疾病是由Dystrophin基因突变引起的,它影响着全世界大约1/3500的男性新生儿。

Neurology:Eteplirsen治疗杜氏肌营养不良长期疗效研究

研究证实,Eteplirsen长期治疗可诱导肌细胞穿透、外显子跳跃并促进新的肌萎缩蛋白表达