Muscle Hypertonia

肌张力增高
  • 文章类型: Journal Article
    分析TECAR治疗(TT)减轻下肢高张力和改善慢性卒中后功能的直接效果。
    这是一项单盲随机对照临床试验。将36例慢性卒中幸存者分为两组。实验组接受30分钟的TT和下肢功能按摩(FM)。对照组接受一次30分钟的TT加FM假治疗。主要结局指标是高张力(改良的Ashworth量表,MAS)。次要结果是步态速度(4米步行测试),站立膝关节屈曲(Fugl-Meyer评估量表IV-项目),负重踝关节背屈的变化(踝关节运动测试,ALT),和功能性下肢力量(5次坐立试验)。所有测量均在基线进行,治疗后立即和30分钟。
    在MAS-膝盖中存在小组时间相互作用(p=0.044),实验组在T1和T0(p<.0001)以及T2和T0(p<.0001)之间的MAS-踝关节(p=0.018)和ALT(p=0.016)。对照组在T1和T0之间ALT显著增加(p=0.003)。
    与FM同时进行的一次TT可立即减少足底屈肌和膝伸肌张力增高,并增加慢性中风幸存者负重踝关节背屈的变化。
    电容和电阻电转移(TECAR)治疗可以改善中风幸存者的踝关节活动能力。TECAR治疗可以改善中风后的膝关节活动度。TECAR治疗可能会降低中风幸存者的下肢肌张力。TECAR疗法可与物理疗法结合使用,作为一种安全的技术,可立即减少中风幸存者的高张力。
    UNASSIGNED: To analyze immediate effects of TECAR therapy (TT) to reduce lower limb hypertonia and improve functionality in chronic post-stroke.
    UNASSIGNED: It is a single-blind randomized controlled clinical trial. A total of 36 chronic stroke survivors were divided into two groups. The experimental group received a single 30-minute session of TT with functional massage (FM) on lower limb. The control group received a single 30-minute session sham treatment of TT plus FM. The primary outcome measure was hypertonia (Modified Ashworth Scale, MAS). Secondary outcomes were gait speed (4-Meter Walk-Test), standing knee-flexion (Fugl-Meyer Assessment Scale IV-item), change in weight bearing ankle dorsiflexion (Ankle Lunge Test, ALT), and functional lower limb strength (5-Times Sit-to-Stand Test). All measurements were performed at baseline, immediately and 30-minutes after treatment.
    UNASSIGNED: There was a group-time interaction in MAS-knee (p = 0.044), MAS-ankle (p = 0.018) and ALT (p = 0.016) between T1 and T0 (p<.0001) and T2 and T0 (p<.0001) for the experimental group. There was a significant increase in ALT between T1 and T0 (p = 0.003) in the control group.
    UNASSIGNED: A single session of TT performed at the same time as FM immediately reduces plantar-flexors and knee-extensor muscle hypertonia and increases change in weight bearing ankle dorsiflexion in chronic stroke survivors.
    Capacitive and resistive electric transfer (TECAR) therapy may improve ankle mobility in stroke survivors.TECAR therapy may improve knee mobility in post-stroke.TECAR therapy may reduce lower limb muscle tone in stroke survivors.TECAR therapy could be used in combination with physiotherapy as a safe technique for the immediate reduction of hypertonia in stroke survivors.
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  • 文章类型: Journal Article
    目的:鞘内注射巴氯芬(ITB)是治疗儿童高张力的有效方法,涉及泵和导管系统的植入。ITB的最高浓度在导管尖端。导管尖端位置最常见的是在腰椎或胸椎内。由于担心通气不足和肺炎,传统上避免了宫颈尖端位置;但是,与胸部或腰椎放置相比,颈椎的这些并发症尚未得到可靠证实。一些研究表明,宫颈ITB位置更好地治疗上肢高渗症。描述宫颈ITB对高渗症的安全性和有效性的数据有限。作者提出了一个单机构回顾性病例系列,强调了使用宫颈ITB位置治疗高张力的安全性和有效性。
    方法:对2022年4月至2023年10月期间连续给药宫颈ITB的儿童进行回顾性数据分析。不可改变的危险因素,临床变量,手术特征,并收集不良结局.
    结果:本研究包括25名患者(8名女性)。植入时的平均年龄为12.4岁,平均手术时间为90分钟。平均Barry-Albright肌张力障碍量表评分下降9.5分(p=0.01)。上肢改良Ashworth量表的平均总评分下降了2.14分(p=0.04),下肢下降4.98分(p<0.01)。每个患者(4%)有感染和巴氯芬毒性。两名患者(8%)有呼吸抑制,需要持续气道正压通气。没有肺炎或伤口开裂的发生率。
    结论:ITB的宫颈导管尖端位置是安全的,有效控制音调,应考虑用于治疗高张力症。需要进行更大的研究和更长时间的随访,以进一步确定这些患者的上限给药安全性以及长期功能益处。
    Intrathecal baclofen (ITB) is an effective treatment for hypertonia in children involving the implantation of a pump and catheter system. The highest concentration of ITB is at the catheter tip. The catheter tip location is most commonly within the lumbar or thoracic spine. The cervical tip location has traditionally been avoided because of concerns of hypoventilation and pneumonia; however, these complications in cervical compared with thoracic or lumbar placement have not been reliably proven. Some studies have suggested that cervical ITB location better treats upper-extremity hypertonia. There are limited data describing the safety and efficacy of cervical ITB on hypertonia. The authors present a single-institution retrospective case series highlighting the safety and efficacy of using cervical ITB location for the treatment of hypertonia.
    Retrospective data analysis was performed for children who underwent continuous dosing cervical ITB between April 2022 and October 2023. Nonmodifiable risk factors, clinical variables, operative characteristics, and adverse outcomes were collected.
    This study included 25 patients (8 female). The mean age at implantation was 12.4 years, and the mean operative duration was 90 minutes. The mean Barry-Albright Dystonia Scale score decreased by 9.5 points (p = 0.01). The mean aggregated modified Ashworth scale score in the upper extremities decreased by 2.14 points (p = 0.04), and that in the lower extremities decreased by 4.98 points (p < 0.01). One patient each (4%) had infection and baclofen toxicity. Two patients (8%) had respiratory depression requiring continuous positive airway pressure. There was no incidence of pneumonia or wound dehiscence.
    The cervical catheter tip location for ITB is safe, is effective to control tone, and should be considered for the treatment of hypertonia. Larger studies with longer follow-up are necessary to further determine upper-limit dosing safety along with long-term functional benefits in these patients.
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  • 文章类型: Journal Article
    目的:脑瘫(CP)患儿常出现药物难治性高张力,有手术治疗,包括神经调节和神经根切断术。医学难治性混合性高张力或肌张力障碍的传统手术治疗包括鞘内巴氯芬泵和选择性背根切断术。非选择性腰骶部腹侧-背根切断术(VDR;腹侧和背根病变80%-90%)有可能解决传统手术选择的局限性。作者强调了非选择性腰骶部VDR用于重度CP非卧床患者姑息性音调管理的机构安全性和有效性。
    方法:作者对2022年至2023年接受腰骶部VDR的患者进行了回顾性分析。人口因素,临床变量,并收集手术特征。感兴趣的主要结果包括语气控制和生活质量改善。次要结果指标包括,作为一种安全措施,围手术期事件,如感觉异常。还注意到术后并发症。
    结果:14名患者(7名女性)被纳入研究。所有患者均接受了T12-L2骨成形术和双侧L1-S1VDR。9名患者患有四肢瘫痪混合性高张力,4人四肢瘫痪痉挛,1例广泛性继发性肌张力障碍。在VDR之后,下肢改良Ashworth量表(MAS)评分(均差[MD]-2.77±1.0,p<0.001)和上肢MAS评分(MD-0.71±0.76,p=0.02)均显着降低,平均随访3个月。在患有广泛性肌张力障碍的患者中,下肢Barry-Albright肌张力障碍量表评分从8分降至0分,总分从32分降至13分.所有父母都注意到护理的便利性增加了,特别是在定位方面,转账,和改变。平均每日肠内巴氯芬剂量从术前47mg降至术后24.5mg(p<0.001)。三个病人出现伤口裂开,其中2人并发感染。
    结论:腰骶部VDR是安全的,对音调控制有效,并且可以改善医学难治性下肢混合性高渗症患者的生活质量。腰骶段VDR可用于重度CP的非卧床患者的姑息性音调控制。需要进行更大的研究和更长时间的随访,以进一步确定这些患者的安全性和长期益处。
    Children with cerebral palsy (CP) often experience medically refractory hypertonia, for which there are surgical therapies including neuromodulation and rhizotomy. Traditional surgical treatment for medically refractory mixed hypertonia or dystonia includes intrathecal baclofen pumps and selective dorsal rhizotomy. A nonselective lumbosacral ventral-dorsal rhizotomy (VDR; ventral and dorsal roots lesioned by 80%-90%) has the potential to address the limitations of traditional surgical options. The authors highlighted the institutional safety and efficacy of nonselective lumbosacral VDR for palliative tone management in nonambulatory patients with more severe CP.
    The authors performed a retrospective analysis of patients who had undergone lumbosacral VDR between 2022 and 2023. Demographic factors, clinical variables, and operative characteristics were collected. The primary outcomes of interest included tone control and quality of life improvement. Secondary outcome measures included, as a measure of safety, perioperative events such as paresthesias. Postoperative complications were also noted.
    Fourteen patients (7 female) were included in the study. All patients had undergone a T12-L2 osteoplastic laminoplasty and bilateral L1-S1 VDR. Nine patients had quadriplegic mixed hypertonia, 4 had quadriplegic spasticity, and 1 had generalized secondary dystonia. Following VDR, there was a significant decrease in both lower-extremity modified Ashworth Scale (mAS) scores (mean difference [MD] -2.77 ± 1.0, p < 0.001) and upper-extremity mAS scores (MD -0.71 ± 0.76, p = 0.02), with an average follow-up of 3 months. In the patient with generalized dystonia, the lower-extremity Barry-Albright Dystonia Scale score decreased from 8 to 0, and the overall score decreased from 32 to 13. All parents noted increased ease in caregiving, particularly in terms of positioning, transfers, and changing. The mean daily enteral baclofen dose decreased from 47 mg preoperatively to 24.5 mg postoperatively (p < 0.001). Three patients developed wound dehiscence, 2 of whom had concurrent infections.
    Lumbosacral VDR is safe, is effective for tone control, and can provide quality of life improvements in patients with medically refractory lower-limb mixed hypertonia. Lumbosacral VDR can be considered for palliative tone control in nonambulatory patients with more severe CP. Larger studies with longer follow-ups are necessary to further determine safety and long-term benefits in these patients.
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  • 文章类型: Journal Article
    目的:由颈部区域的重复性工作或劳损引起的肌肉功能障碍可干扰肌肉反应。肌肉功能障碍可能是引起颈椎病的重要因素。然而,当枕下肌群出现功能障碍时,上颈椎的生物力学特性如何改变还没有研究。本研究的目的是利用有限元(FE)方法研究颈椎病的生物力学证据,从而为临床医生进行穴位治疗提供指导。
    方法:通过改变枕下肌的弹性模量,在正常肌肉功能和肌肉功能障碍的条件下重建C0-C3运动段的四个FE模型。对于两个正常条件下的有限元模型,在一个肌肉功能障碍FE模型中,C0-C3运动节段两侧的枕下肌肉的弹性模量相等且在正常范围内,两侧的弹性模量相等且大于37kPa,代表肌肉高张力;另一方面,左右枕下肌的弹性模量不同,表明肌肉不平衡。外侧寰枢关节(LAAJ)的生物力学行为,寰齿突关节(ADJ),通过模拟分析椎间盘(IVD),在六次屈曲载荷下进行,扩展,左右横向弯曲,左右轴向旋转。
    结果:在屈曲下,肌肉失衡的LAAJ最大应力高于正常肌肉和高张力,而高渗模型中IVD的最大应力高于正常模型和不平衡模型。在所有模型的所有载荷中,ADJ中的最大应力在延伸下最大。肌肉失衡和高张力不会引起ADJ的过度应激和应激分布异常。
    结论:肌肉功能障碍增加了LAAJ和IVD的压力,但不影响ADJ.
    OBJECTIVE: Muscle dysfunction caused by repetitive work or strain in the neck region can interfere muscle responses. Muscle dysfunction can be an important factor in causing cervical spondylosis. However, there has been no research on how the biomechanical properties of the upper cervical spine change when the suboccipital muscle group experiences dysfunction. The objective of this study was to investigate the biomechanical evidence for cervical spondylosis by utilizing the finite element (FE) approach, thus and to provide guidance for clinicians performing acupoint therapy.
    METHODS: By varying the elastic modulus of the suboccipital muscle, the four FE models of C0-C3 motion segments were reconstructed under the conditions of normal muscle function and muscle dysfunction. For the two normal condition FE models, the elastic modulus for suboccipital muscles on both sides of the C0-C3 motion segments was equal and within the normal range In one muscle dysfunction FE model, the elastic modulus on both sides was equal and greater than 37 kPa, which represented muscle hypertonia; in the other, the elastic modulus of the left and right suboccipital muscles was different, indicating muscle imbalance. The biomechanical behavior of the lateral atlantoaxial joint (LAAJ), atlanto-odontoid joint (ADJ), and intervertebral disc (IVD) was analyzed by simulations, which were carried out under the six loadings of flexion, extension, left and right lateral bending, left and right axial rotation.
    RESULTS: Under flexion, the maximum stress in LAAJ with muscle imbalance was higher than that with normal muscle and hypertonia, while the maximum stress in IVD in the hypertonic model was higher than that in the normal and imbalance models. The maximum stress in ADJ was the largest under extension among all loadings for all models. Muscle imbalance and hypertonia did not cause overstress and stress distribution abnormalities in ADJ.
    CONCLUSIONS: Muscle dysfunction increases the stress in LAAJ and in IVD, but it does not affect ADJ.
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  • 文章类型: Published Erratum
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  • 文章类型: Journal Article
    主要哮喘症状的发生主要归因于气道迷走神经张力增高,其中的核心机制尚不清楚。这项研究检验了以下假设:内皮素1介导的脑干神经胶质激活通过增强5'-三磷酸腺苷对神经元嘌呤能P2X4受体的作用而产生哮喘性气道迷走神经张力增高。使用卵清蛋白制备哮喘大鼠模型。通过喉返放电和肺功能的体积描记测量来评估气道迷走神经张力。使用ELISA检查脑干的变化,蛋白质印迹,荧光素-荧光素酶,定量逆转录聚合酶链反应,酶活性测定和免疫荧光染色,分别。结果表明,在大鼠的髓质中,内皮素受体B型和P2X4受体主要在星形胶质细胞和神经元中表达,分别,两者,随着内皮素-1的含量,卵清蛋白致敏后显著增加。卵清蛋白敏化显著增加了喉返放电,通过急性脑池内注射P2X4受体拮抗剂5-BDBD阻断,脑干P2X4受体敲除,和慢性腹腔注射内皮素受体拮抗剂B型BQ788。卵清蛋白致敏激活小胶质细胞和星形胶质细胞,并显着降低髓质中的ecto-5'-核苷酸酶活性,所有这些,随着髓样P2X4受体表达的增加和肺功能的下降,被慢性BQ788治疗逆转。这些结果表明,在大鼠中,过敏性气道激发通过增强内皮素-1/内皮素受体B型信号传导激活髓质中的小胶质细胞和星形胶质细胞,随后通过增强的5'-三磷酸腺苷/P2X4受体信号在气道迷走神经反射的中枢神经元中引起气道迷走神经高张力。
    The occurrence of major asthma symptoms is largely attributed to airway vagal hypertonia, of which the central mechanisms remain unclear. This study tests the hypotheses that endothelin-1-mediated brainstem glial activation produces asthmatic airway vagal hypertonia via enhanced action of adenosine 5\'-triphosphate on neuronal purinergic P2X4 receptors. A rat model of asthma was prepared using ovalbumin. Airway vagal tone was evaluated by the recurrent laryngeal discharge and plethysmographic measurement of pulmonary function. The changes in the brainstem were examined using ELISA, Western blot, luciferin-luciferase, quantitative reverse transcription-polymerase chain reaction, enzyme activity assay and immunofluorescent staining, respectively. The results showed that in the medulla of rats, endothelin receptor type B and P2X4 receptors were primarily expressed in astrocytes and neurons, respectively, and both of which, along with endothelin-1 content, were significantly increased after ovalbumin sensitization. Ovalbumin sensitization significantly increased recurrent laryngeal discharge, which was blocked by acute intracisternal injection of P2X4 receptor antagonist 5-BDBD, knockdown of brainstem P2X4 receptors, and chronic intraperitoneal injection of endothelin receptor type B antagonist BQ788, respectively. Ovalbumin sensitization activated microglia and astrocytes and significantly decreased ecto-5\'-nucleotidase activity in the medulla, and all of which, together with the increase of medullary P2X4 receptor expression and decrease of pulmonary function, were reversed by chronic BQ788 treatment. These results demonstrated that in rats, allergic airway challenge activates both microglia and astrocytes in the medulla via enhanced endothelin-1/endothelin receptor type B signaling, which subsequently causes airway vagal hypertonia via augmented adenosine 5\'-triphosphate/P2X4 receptor signaling in central neurons of airway vagal reflex.
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  • 文章类型: Journal Article
    目前尚无脊髓损伤(SCI)后过度肌张力的按需和局部治疗。这里,我们检查了中胸患者腿部高张力的减少,使用商用经皮电刺激器(TES)以50或150Hz的频率施加到下背部,以及可能的机制使腿部张力双侧降低。在没有TES的情况下和在TES期间,将阴极(T11-L2)和阳极(L3-L5)放置在脊柱上方(中线,MID)或中线左侧10厘米(横向,LAT)仅对活跃的底层皮肤和肌肉传入,用摆锤测试同时测量两条腿。在与应用的LATTES相对的右腿中检查了本体感觉(H反射)和皮肤神经反射(CMR)传入介导的脊柱反射。双腿的高张力消失,但仅在胸腰椎TES期间消失,甚至在LATTES期间。音调的明显减少反映在从完全伸展位置释放后,两个小腿首先下降到更大的距离。在MID和LATTES期间增加了172.8%和94.2%,分别,与没有TES相比。MID和LAT(左)TES都增加了H反射,但减少了第一次爆发,并延长了随后爆发的时间,在右腿的皮肤神经反射中。胸腰椎TES是一种有前途的方法来减少腿部高张力的慢性,运动性完全SCI而不激活脊髓结构,并且可能通过促进本体感受输入而起作用,从而激活具有双侧投射的兴奋性中间神经元,进而招募复发性抑制性神经元。NEW&NOTEWORTHY我们提出了概念证明,下背部的表面刺激可以减少运动完全的参与者的严重腿部过度紧张,胸椎脊髓损伤(SCI),但仅在施加刺激期间。我们建议胸腰椎经皮电刺激(TES)激活皮肤和肌肉传入神经可能会招募具有双侧投射的兴奋性脊髓中间神经元,从而招募复发性抑制网络,以按需抑制正在进行的非自愿运动神经元活动。
    On demand and localized treatment for excessive muscle tone after spinal cord injury (SCI) is currently not available. Here, we examine the reduction in leg hypertonus in a person with mid-thoracic, motor complete SCI using a commercial transcutaneous electrical stimulator (TES) applied at 50 or 150 Hz to the lower back and the possible mechanisms producing this bilateral reduction in leg tone. Hypertonus of knee extensors without and during TES, with both cathode (T11-L2) and anode (L3-L5) placed over the spinal column (midline, MID) or 10 cm to the left of midline (lateral, LAT) to only active underlying skin and muscle afferents, was simultaneously measured in both legs with the pendulum test. Spinal reflexes mediated by proprioceptive (H-reflex) and cutaneomuscular reflex (CMR) afferents were examined in the right leg opposite to the applied LAT TES. Hypertonus disappeared in both legs but only during thoracolumbar TES, and even during LAT TES. The marked reduction in tone was reflected in the greater distance both lower legs first dropped to after being released from a fully extended position, increasing by 172.8% and 94.2% during MID and LAT TES, respectively, compared with without TES. Both MID and LAT (left) TES increased H-reflexes but decreased the first burst, and lengthened the onset of subsequent bursts, in the cutaneomuscular reflex of the right leg. Thoracolumbar TES is a promising method to decrease leg hypertonus in chronic, motor complete SCI without activating spinal cord structures and may work by facilitating proprioceptive inputs that activate excitatory interneurons with bilateral projections that in turn recruit recurrent inhibitory neurons.NEW & NOTEWORTHY We present proof of concept that surface stimulation of the lower back can reduce severe leg hypertonus in a participant with motor complete, thoracic spinal cord injury (SCI) but only during the applied stimulation. We propose that activation of skin and muscle afferents from thoracolumbar transcutaneous electrical stimulation (TES) may recruit excitatory spinal interneurons with bilateral projections that in turn recruit recurrent inhibitory networks to provide on demand suppression of ongoing involuntary motoneuron activity.
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  • 文章类型: Case Reports
    鞘内注射巴氯芬(ITB)治疗,对于不合适的常规痉挛药物候选药物,是口服途径给药的优选方法。由于其生物利用度提高,ITB确保在目标地点更有效地交付。
    目前缺乏关于使用ITB治疗治疗痉挛型肌张力障碍非卧床患者的确凿证据。ITB泵植入前,患者通常接受ITB推注试验,以排除潜在的不良反应,并验证对高渗问题的治疗效果.在这份报告中,我们重点介绍一例痉挛性肌张力障碍,特别关注在ITB注射试验后在改良Ashworth量表(MAS)评分和步态模式方面均显示显著改善的非卧床患者.
    本病例报告概述了一名67岁男性的病史,诊断为左侧偏瘫和痉挛性肌张力障碍,由于他的右丘脑颅内出血的第二次发作。开始了ITB注射试验,因为患者不适合继续注射肉毒杆菌毒素和口服药物。这是由于上肢和下肢持续发生痉挛性肌张力障碍。患者接受了为期四天的ITB注射试验,剂量逐渐增加,改善MAS评分和步态参数,包括节奏,步长,步进时间,步幅长度,步伐时间增加了。特别是,运动学步态分析表明,在僵硬的膝关节步态模式下,在摆动阶段膝关节屈曲增加有了显着改善。这些结果表明痉挛相关症状逐渐减少,表明ITB注射试验的积极作用。患者最终接受了ITB泵植入。
    在这位患有痉挛型肌张力障碍的中风后患者中,ITB治疗已证明有效和实质性的痉挛管理,随着步态模式的改善。
    UNASSIGNED: Intrathecal baclofen (ITB) therapy, a viable alternative for unsuitable candidates of conventional spasticity medications, is a preferred method of administration over the oral route. Owing to its enhanced bioavailability, ITB ensures a more effective delivery at the target site.
    UNASSIGNED: There is a lack of conclusive evidence regarding the use of ITB treatment in managing ambulatory patients with spastic dystonia. Before ITB pump implantation, patients commonly undergo an ITB bolus injection trial to rule out potential adverse reactions and verify the therapeutic effects on hypertonic issues. In this report, we highlight a case of spastic dystonia, particularly focusing on an ambulatory patient who demonstrated significant improvement in both the modified Ashworth scale (MAS) score and gait pattern following the ITB injection trial.
    UNASSIGNED: This case report outlines the medical history of a 67-year-old male diagnosed with left-side hemiplegia and spastic dystonia, resulting from his second episode of intracranial hemorrhage in the right thalamus. An ITB injection trial was initiated because the patient was not suitable for continued botulinum toxin injections and oral medications. This was due to the persistent occurrence of spastic dystonia in both the upper and lower extremities. The patient underwent a four-day ITB injection trial with progressively increasing doses, resulting in improved MAS scores and gait parameters, including cadence, step length, step time, stride length, and stride time were increased. Particularly, kinematic gait analysis demonstrates a substantial improvement of increased knee flexion in the swing phase in stiff knee gait pattern. These findings indicated a gradual reduction in spasticity-related symptoms, signifying the positive effect of the ITB injection trial. The patient eventually received an ITB pump implantation.
    UNASSIGNED: In this post-stroke patient with spastic dystonia, ITB therapy has demonstrated effective and substantial management of spasticity, along with improvement in gait patterns.
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  • 文章类型: Journal Article
    目的:5%至12%的儿童发生腰穿后头痛。这项研究的目的是确定进行鞘内巴氯芬试验的高渗症患儿腰椎穿刺后头痛的频率和预测因素。方法:这是一项回顾性的单中心回顾,对2013-2022年接受鞘内巴氯芬试验的所有43名患有高张力和/或运动障碍的儿童(<18岁)进行研究。通过双向配对t检验和Fisher精确检验评估腰椎穿刺后头痛的预测因素。结果:7名受试者(16.3%)出现腰椎穿刺后头痛。在腰椎穿刺后出现头痛的患者中,3需要紧急护理或住院治疗。其中一人误诊为便秘。没有测量开口压力的16名患者从随后的分析中排除。在27名患者中,有记录的打开压力,平均开放压力为24.0cmH2O(SD6.5),5(18.5%)的开放压力升高(>28cmH2O)。腰椎穿刺后头痛患者的平均开口压力较高(28.6vs22.4cmH2O,P=.014)。打开压力升高的患者中有60%出现腰椎穿刺后头痛。将巴氯芬泵放置在4例(80%)开放压力升高的患者和6例(85.7%)腰椎穿刺后头痛的患者中,无并发症。解释:鞘内注射巴氯芬试验后腰椎穿刺后头痛的风险高于文献报道,可能是因为打开压力升高的速率更大。医生可以使用打开压力来预测腰椎穿刺后头痛的风险,并应向家人介绍症状。打开压力升高或腰椎穿刺后头痛可能不排除巴氯芬泵的放置。
    Aims: Post-lumbar puncture headache occurs in 5% to 12% of children. The purpose of this study was to determine the frequency and predictors of post-lumbar puncture headache in children with hypertonia undergoing lumbar puncture for intrathecal baclofen trial. Methods: This was a retrospective single-center review of all 43 children (<18 years) with hypertonia and/or dyskinesia undergoing intrathecal baclofen trial from 2013-2022. Predictors of post-lumbar puncture headache were evaluated via 2-way paired t test and Fisher exact test. Results: Seven subjects (16.3%) developed post-lumbar puncture headache. Of patients who developed post-lumbar puncture headache, 3 required emergency care or hospitalization. One was misdiagnosed with constipation. The 16 patients without opening pressure measured were excluded from subsequent analyses. Of the 27 patients with documented opening pressure, the mean opening pressure was 24.0 cm H2O (SD 6.5) and 5 (18.5%) had elevated opening pressure (>28 cm H2O). Mean opening pressure was higher for those with post-lumbar puncture headache (28.6 vs 22.4 cm H2O, P = .014). Sixty percent of patients with elevated opening pressure developed post-lumbar puncture headache. Baclofen pumps were placed in 4 (80%) patients with elevated opening pressure and 6 (85.7%) with post-lumbar puncture headaches without complications. Interpretation: The risk of post-lumbar puncture headache after intrathecal baclofen trial was higher than reported in the literature, likely because of greater rates of elevated opening pressure. Physicians may use opening pressure to predict risk for post-lumbar puncture headache and should educate families about symptoms. Elevated opening pressure or post-lumbar puncture headache may not preclude baclofen pump placement.
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  • 文章类型: Case Reports
    由原肌球蛋白基因TPM2和TPM3的致病变异引起的肌病患者通常会出现肌肉张力减退和无力,他们的肌肉活检通常显示纤维大小不均衡和线虫体。这里,我们描述了一系列具有过度收缩分子表型的患者,高肌肉张力,和大多数无线虫体的非特异性肌病活检结果。其中三个病人有三丝,而在一个病人身上,她手指的远端关节在手腕伸展时弯曲。在患有罕见TPM3致病变异的患者的一次活检中,观察到岩心和微矿,TPM3引起的肌病的一个不寻常的发现。变体改变原肌球蛋白和肌动蛋白之间的保守接触位点。
    Patients with myopathies caused by pathogenic variants in tropomyosin genes TPM2 and TPM3 usually have muscle hypotonia and weakness, their muscle biopsies often showing fibre size disproportion and nemaline bodies. Here, we describe a series of patients with hypercontractile molecular phenotypes, high muscle tone, and mostly non-specific myopathic biopsy findings without nemaline bodies. Three of the patients had trismus, whilst in one patient, the distal joints of her fingers flexed on extension of the wrists. In one biopsy from a patient with a rare TPM3 pathogenic variant, cores and minicores were observed, an unusual finding in TPM3-caused myopathy. The variants alter conserved contact sites between tropomyosin and actin.
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