Cervical dystonia

宫颈肌张力障碍
  • 文章类型: Journal Article
    小脑的经颅直流电刺激(tDCS)显示出治疗肌张力障碍的希望。在这种情况下,还制定了特定的运动康复计划。然而,尚未对这两种方法的组合进行评估以确定其治疗潜力.
    我们报告了一系列5例通过肉毒杆菌毒素注射控制不佳的宫颈肌张力障碍(CD)患者。最初通过单独使用小脑阳极tDCS(cer-atDCS)的每日重复疗程(3或5天)的方案进行治疗。第二次,cer-atDCS的附加协议与目标导向的运动训练练习(Mot-Training)计划相结合,专门用于治疗CD。在多伦多西部痉挛性斜颈评定量表(TWSTRS)上评估了该程序的临床影响。
    与基线相比,单独进行cer-atDCS后,TWSTRS总分改善的最大百分比平均为37%(p=0.147,无显著性),cer-atDCS联合Mot-Training后,平均为53%(p=0.014,显著性).联合方案后,TWSTRS疼痛和功能障碍子评分也有所改善。在单独或联合方案进行cer-atDCS后,TWSTRS反应量表评分为(+3)至(+5),对应于肌张力障碍和疼痛的中度至显著改善。这种改进在联合方案后比单独使用cer-atDCS后持续的时间更长(3.4vs.平均1.4个月,p=0.011)。
    与单独使用cer-atDCS相比,cer-atDCS与Mot-Training的结合产生了更大,更长时间的改善。这种联合治疗程序易于执行,并在CD的长期治疗中开辟了重要的前景。这些结果仍有待更大样本的随机假对照试验证实。
    UNASSIGNED: Transcranial Direct Current Stimulation (tDCS) of the cerebellum shows promise for the treatment of dystonia. Specific motor rehabilitation programs have also been developed in this context. However, the combination of these two approaches has not yet been evaluated to determine their therapeutic potential.
    UNASSIGNED: We report a series of 5 patients with cervical dystonia (CD) poorly controlled by botulinum toxin injections. They were initially treated by a protocol of repeated daily sessions (for 3 or 5 days) of cerebellar anodal tDCS (cer-atDCS) applied alone. In a second time, additional protocols of cer-atDCS were performed in combination with a program of goal-oriented motor training exercises (Mot-Training), specifically developed for the treatment of CD. The clinical impact of the procedures was assessed on the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS).
    UNASSIGNED: Compared to baseline, the maximum percentage of TWSTRS total score improvement was 37% on average after cer-atDCS performed alone (p = 0.147, not significant) and 53% on average after cer-atDCS combined with Mot-Training (p = 0.014, significant). The TWSTRS pain and functional handicap subscores also improved after the combined protocol. A score of (+3) to (+5) was rated on the TWSTRS response scale after cer-atDCS performed alone or the combined protocol, corresponding to a moderate to striking improvement on dystonia and pain. This improvement lasted longer after the combined protocol than after cer-atDCS alone (3.4 vs. 1.4 months on average, p = 0.011).
    UNASSIGNED: The combination of cer-atDCS with Mot-Training produced a greater and more prolonged improvement than the application of cer-atDCS alone. Such a combined therapeutic procedure is easy to perform and opens important perspectives in the long-term treatment of CD. These results remain to be confirmed by a randomized sham-controlled trial on a larger sample.
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  • 文章类型: Case Reports
    背景:宫颈肌张力障碍(CD)是一种通常发生在颈部肌肉中的肌张力障碍。由于颈部肌肉的间歇性或持续不自主收缩,头部和颈部扭曲和偏斜,并出现一些姿势异常。长期的姿势异常或疼痛会引起患者的负面情绪,这会影响他们的生活质量。
    方法:该病例报告包括一名37岁女性,她被诊断患有与焦虑和抑郁相关的CD;伴随的症状是头部和颈部向右倾斜约90°和精神障碍。经过两个疗程的针灸治疗,患者的头部和颈部可以保持在正常的位置,负面情绪可以得到缓解。
    结论:本病例提示针刺能有效改善CD患者的情绪状态和生活质量,使其成为有效的替代疗法。
    BACKGROUND: Cervical dystonia (CD) is a type of muscle tone disorder that usually occurs in the neck muscles. Due to the intermittent or continuous involuntary contraction of the neck muscles, the head and neck are twisted and skewed and some postural abnormalities occur. Long-term abnormal posture or pain can cause negative emotions in patients, which can affect their quality of life.
    METHODS: This case report included a 37-year-old woman who was diagnosed with CD associated with anxiety and depression; the accompanying symptoms were head and neck tilt of approximately 90° to the right and mental abnormality. After two courses of acupuncture treatment, the patient\'s head and neck can be maintained in a normal position, and the negative emotions can be relieved.
    CONCLUSIONS: This case indicates that acupuncture can effectively improve CD and the emotional state and quality of life of patients, making it an effective alternative treatment for the condition.
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  • 文章类型: Case Reports
    颈肌张力障碍并发脊髓型颈椎病是一种具有挑战性的病理,需要经过深思熟虑的管理。一名46岁的女性在这次演讲中被提到我们的中心。我们选择在C5至C7颈前路椎间盘切除术和融合(ACDF)之前进行双侧苍白球内深部脑刺激(DBS-GPi),以避免肌张力障碍运动对颈椎融合产生负面影响。在C5至C7ACDF后三个月和DBS-GPi后九个月对患者进行了随访,完全控制了震颤,没有放射学证据表明硬件松动或对准不良。虽然这种策略是成功的治疗我们的病人的颈脊髓病和颈肌张力障碍,需要进行更大规模的研究,以优化出现这些并发病变的患者的治疗.
    Cervical dystonia with concurrent cervical myelopathy is a challenging pathology that requires thoughtful management. A 46-year-old female was referred to our center with this presentation. We elected to perform bilateral globus pallidus internus deep brain stimulation (DBS-GPi) prior to C5 to C7 anterior cervical discectomy and fusion (ACDF) to avoid the potential for dystonic movements to negatively impact cervical fusion. The patient was followed up at three months post C5 to C7 ACDF and nine months post DBS-GPi with complete control of tremor and no radiographic evidence of hardware loosening or malalignment. Though this strategy was successful in treating both our patient\'s cervical myelopathy and cervical dystonia, larger studies need to be conducted to optimize the treatment of patients presenting with these concurrent pathologies.
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  • 文章类型: Case Reports
    共济失调-毛细血管扩张样障碍1(ATLD1)是一种罕见的神经退行性疾病,与早发性共济失调和动眼失用症有关。ATLD1的遗传测定是MRE11基因(减数分裂重组11基因)的突变,导致DNA双链断裂修复缺陷。ATLD1患者的临床特征与共济失调毛细血管扩张症(AT)相似,进展缓慢,表现温和。主要症状包括进行性小脑共济失调,动眼失用症,细胞对电离辐射过敏。面部运动障碍,肌张力障碍,构音障碍也有报道。在这里,我们介绍了一位45岁的女性,患有颈部和面部肌张力障碍,构音障碍和共济失调,结果证明是第一例无动眼失用症的ATLD,以肌张力障碍为主要表现。她多年来一直表现出这些非特异性症状,在整个外显子组测序确认诊断之前。
    Ataxia-telangiectasia-like disorder 1 (ATLD1) is a rare neurodegenerative disorder associated with early onset ataxia and oculomotor apraxia. The genetic determination of ATLD1 is a mutation in the MRE11 gene (meiotic recombination 11 gene), which causes DNA-double strand break repair deficits. Clinical features of patients with ATLD1 resemble those of ataxia telangiectasia (AT), with slower progression and milder presentation. Main symptoms include progressive cerebellar ataxia, oculomotor apraxia, cellular hypersensitivity to ionizing radiations. Facial dyskinesia, dystonia, dysarthria have also been reported. Here we present a 45-year old woman with cervical and facial dystonia, dysarthria and ataxia, who turned out to be the first case of ATLD without oculomotor apraxia, and with dystonia as a main manifestation of the disease. She had presented those non-specific symptoms for years, before whole exome sequencing confirmed the diagnosis.
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  • 文章类型: Journal Article
    目标:非运动症状,比如睡眠障碍,疲劳,神经精神表现,认知障碍,和感觉异常,在特发性宫颈肌张力障碍(ICD)患者中已得到广泛报道。本研究旨在阐明ICD患者的自主神经系统(ANS)受累,这在文献中还不清楚。
    方法:我们进行了一项试点病例对照研究,以调查20例ICD患者和20例年龄性别匹配对照的ANS。复合自主系统量表31用于ANS临床评估。激光多普勒血流仪定量分析,应用于皮肤并从指数中记录,被用来在休息时测量,经过副交感神经激活(六个深呼吸)和两个交感神经刺激(等距手柄和心理计算),高频和低频振荡的功率,和低频/高频比。
    结果:ICD患者的临床自主神经失调症状高于对照组(p<0.05)。在休息时,在ICD患者中检测到比对照组更低的高频功率带,在≥57岁的年龄组中达到统计学上的显着差异(p<0.05)。在后一年龄组,ICD患者在休息时(p<0.05)和心理计算后(p<0.05)的低频/高频比率低于对照组。不管年龄,在手握过程中,ICD患者显示(i)低频/高频比率较低(p<0.05),(ii)与对照相比,低频振荡分量的增加相似,和(iii)稳定的高频振荡分量,相反,在对照组中有所下降。在深呼吸期间,两组之间没有发现差异。
    结论:ICD患者在临床和神经生理水平上表现出ANS功能障碍,反映了异常的副交感神经-交感神经相互作用,可能与异常的颈部姿势和神经递质改变有关。
    OBJECTIVE: Non-motor symptoms, such as sleep disturbances, fatigue, neuropsychiatric manifestations, cognitive impairment, and sensory abnormalities, have been widely reported in patients with idiopathic cervical dystonia (ICD). This study aimed to clarify the autonomic nervous system (ANS) involvement in ICD patients, which is still unclear in the literature.
    METHODS: We conducted a pilot case-control study to investigate ANS in twenty ICD patients and twenty age-sex-matched controls. The Composite Autonomic System Scale 31 was used for ANS clinical assessment. The laser Doppler flowmetry quantitative spectral analysis, applied to the skin and recorded from indices, was used to measure at rest, after a parasympathetic activation (six deep breathing) and two sympathetic stimuli (isometric handgrip and mental calculation), the power of high-frequency and low-frequency oscillations, and the low-frequency/high-frequency ratio.
    RESULTS: ICD patients manifested higher clinical dysautonomic symptoms than controls (p < 0.05). At rest, a lower high-frequency power band was detected among ICD patients than controls, reaching a statistically significant difference in the age group of ≥ 57-year-olds (p < 0.05). In the latter age group, ICD patients showed a lower low-frequency/high-frequency ratio than controls at rest (p < 0.05) and after mental calculation (p < 0.05). Regardless of age, during handgrip, ICD patients showed (i) lower low-frequency/high-frequency ratio (p < 0.05), (ii) similar increase of the low-frequency oscillatory component compared to controls, and (iii) stable high-frequency oscillatory component, which conversely decreased in controls. No differences between the two groups were detected during deep breathing.
    CONCLUSIONS: ICD patients showed ANS dysfunction at clinical and neurophysiological levels, reflecting an abnormal parasympathetic-sympathetic interaction likely related to abnormal neck posture and neurotransmitter alterations.
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  • 文章类型: Case Reports
    目前的病例报告描述了一种罕见的肩胛骨提肌(LS)插入,广泛地附着在多个结构上,包括后上锯齿,颈韧带,颈椎的第6和第7个棘突.该腱膜的最上层部分与C7水平的韧带融合。将头部的中部和下部插入(或融合)到菱形小肌的肌腱以及锯齿后上肌的纤维中。因此,认识到这种解剖变化,其特征是LS附加滑移连接到第七椎骨和其他肌肉结构,这不仅对解剖学专家至关重要,而且对在颈后端进行与颈或肩疼痛和颈肌张力障碍相关的手术的外科医生也至关重要。
    The current case report describes an uncommon insertion of the levator scapulae (LS), which broadly attaches to multiple structures, including the serratus posterior superior, ligamentum nuchae, and the 6th and 7th spinous processes of the cervical vertebrae. The most superior portion of this aponeurosis merged with the ligamentum nuchae at the C7 level. The middle and inferior portions of the head were inserted (or fused) into the tendon of the rhomboid minor as well as the fibers of the serratus posterior superior muscle. Consequently, recognizing this anatomical variation, characterized by an additional slip of the LS attaching to the 7th vertebra and other muscular structures, is crucial not only for anatomists but also for surgeons performing procedures on the posterior neck related to cervical or shoulder pain and cervical dystonia.
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  • 文章类型: Case Reports
    颈肌张力障碍,以颈椎肌肉的不自主收缩为特征,是成人肌张力障碍最常见的形式。在患有难治性宫颈肌张力障碍的患者中,我们根据术前18F-氟代脱氧葡萄糖(18F-FDG)正电子发射断层扫描/计算机断层扫描(PET/CT)对C3-C6脊神经后支的左斜头肌切开术和选择性外周神经支配(SPD)进行了切开术.病人65岁,有着平淡无奇病史的右撇子.他的头不由自主地向左旋转。药物和肉毒杆菌毒素注射无效,并考虑手术治疗。18F-FDGPET/CT显像提示左斜头炎有FDG摄取,右胸锁乳突,和左脾炎。在全身麻醉下进行左斜头炎的肌切开术和C3-C6脊神经后支的SPD。在6个月的随访中,患者的多伦多西部痉挛性斜颈评定量表评分从35分提高到9分。此病例表明,术前18F-FDGPET/CT可有效识别肌张力障碍并确定宫颈肌张力障碍的手术策略。
    Cervical dystonia, characterized by the involuntary contraction of cervical muscles, is the most common form of adult dystonia. In a patient with intractable cervical dystonia, we carried out a myotomy of the left obliquus capitis inferior and selective peripheral denervation (SPD) of the posterior branches of the C3-C6 spinal nerves based on preoperative 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT). The patient was a 65-year-old, right-handed man with an unremarkable medical history. His head rotated involuntarily to the left. Medication and botulinum toxin injections were ineffective, and surgical treatment was considered. 18F-FDG PET/CT imaging revealed FDG uptake in the left obliquus capitis inferior, right sternocleidomastoideus, and left splenius capitis. Myotomy of the left obliquus capitis inferior and SPD of the posterior branches of the C3-C6 spinal nerves was performed under general anesthesia. During the 6-month follow-up, the patient\'s Toronto Western Spasmodic Torticollis Rating Scale score improved from 35 to 9. This case shows that preoperative 18F-FDG PET/CT is effective in identifying dystonic muscles and determining the surgical strategy for cervical dystonia.
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  • 文章类型: Case Reports
    UNASSIGNED:关于苍白球切开术对宫颈肌张力障碍的长期影响的报道仍然很少。
    UNASSIGNED:我们报告一例通过单侧苍白球切开术成功治疗的宫颈肌张力障碍。该患者是一名29岁的男性,没有宫颈肌张力障碍的既往病史和家族史。在28岁的时候,随着右肩抬高,颈部向右旋转发展并逐渐恶化。在症状对重复注射肉毒杆菌毒素和口服药物没有反应后,他做了左侧苍白球切开术,导致颈部肌张力障碍和肩抬高的显着改善,而没有手术并发症。在3个月的评估中,症状完全好转。多伦多西部痉挛性斜颈评定量表评分从手术前的39分显着提高到术后7年评估的0分。
    UNASSIGNED:此病例提示单侧苍白球切开术可作为宫颈肌张力障碍的替代治疗选择。
    UNASSIGNED: Reports on the long-term effects of pallidotomy for cervical dystonia remain scarce.
    UNASSIGNED: We report a case of cervical dystonia successfully treated by unilateral pallidotomy. The patient was a 29-year-old man without past medical and family history of cervical dystonia. At the age of 28 years, neck rotation to the right with right shoulder elevation developed and gradually became worse. After symptoms failed to respond to repetitive botulinum toxin injections and oral medications, he underwent left pallidotomy, which resulted in significant improvement of cervical dystonia and shoulder elevation without surgical complications. At the 3-month evaluation, the symptoms completely improved. The Toronto Western Spasmodic Torticollis Rating Scale score dramatically improved from 39 points before surgery to 0 points at 7-year postoperative evaluation.
    UNASSIGNED: This case suggests that unilateral pallidotomy can be an alternative treatment option for cervical dystonia.
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  • 文章类型: Journal Article
    介绍颈肌张力障碍(CD),一种罕见的疾病,是肌张力障碍最常见的形式,运动障碍.慢性疼痛可能导致日常生活活动和生活质量受损,感知到的耻辱,行走困难,和/或缺乏对运动的控制。对CD中行走困难的治疗方法的研究尚无定论。整骨手法医学(OMM)已用于改善其他健康状况下的步态生物力学。步行时的足部进展角(FPA)表明功能性步态异常,会增加膝关节损伤和骨关节炎的风险。目的本研究的目的是通过识别和解决躯体功能障碍,测试使用针对CD设计的OMM序列进行的每周五次治疗是否可以改善CD患者的异常步态生物力学。方法在这个前瞻性病例系列中,在40岁之前独立走动有CD症状的个体,不是由于外伤,使用经过验证的严重程度量表(多伦多西部痉挛性斜颈评定量表[TWSTRsI])和影响生活质量的症状(宫颈肌张力障碍影响谱[CDIP-58])进行评估,体检,和FPA之前和之后五周的OMM治疗。在临床步态实验室中,通过九台摄像机以参与者选择的步行速度在一个步态周期的三次试验中收集三维全身位置数据,从而捕获了下半身关节的运动范围和角度。步态周期期间的FPA波形通过ViconNexus和Polygon应用进行定量。将治疗前和治疗后的结果与建立的健康步态波形进行比较,并通过重复测量ANOVA(α=0.05)进行测试。结果与先前发表的年龄-性别匹配的健康波形相比,在需要下肢内旋的步态周期阶段,CD中的预处理波形具有平均5.13°的过量FPA。五次治疗后内旋改善96%,平均0.21°(p=0.041)的过量FPA。平均TWSTR和CDIP-58评分改善。在体检时,C2椎骨的旋转方向与颈部肌肉高渗性相对。患有前斜颈的患者存在垂直的蝶骨基底软骨综合菌株。参与者具有同侧颈前外侧肌和腹壁前外侧肌张力增高。所有患者均患有骨盆躯体功能障碍,左侧相对于右侧高于右侧,并且下肢内旋受限(即,旋光功能障碍)。结论治疗后FPA明显改善。该OMM序列具有良好的耐受性,并且可用于改善患有CD的个体的步态运动学。随机化,控制,需要长期研究来确定有效性。
    Introduction Cervical dystonia (CD), a rare disorder, is the most common form of dystonia, a movement disorder. Impairments in activities of daily living and quality of life may result from chronic pain, perceived stigma, difficulty walking, and/or lack of control over movements. Studies of treatments for difficulty walking in CD have been inconclusive. Osteopathic manipulative medicine (OMM) has been used to improve gait biomechanics in other health conditions. Foot progression angle (FPA) while walking indicates functional gait abnormalities that increase the risk of knee injury and osteoarthritis. Objective The aim of this study is to test if five-weekly treatments using an OMM sequence designed for CD improved abnormal gait biomechanics in individuals with CD by identifying and addressing somatic dysfunctions. Methods In this prospective case series, independently ambulating individuals with CD symptom onset before the age of 40 years, not due to traumatic injury, were evaluated utilizing validated scales for severity (Toronto western spasmodic torticollis rating scale [TWSTRsI]) and symptoms affecting quality of life (Cervical Dystonia Impact Profile [CDIP-58]), physical examination, and FPA before and after five-weekly OMM treatments. Lower body joint range of motion and angles were captured in a clinical gait lab by nine cameras collecting three-dimensional Whole-body position data during three trials of one gait cycle at participant-selected walking speed. The FPA waveforms during the gait cycle were quantified by Vicon Nexus and Polygon applications. Pretreatment and posttreatment results were compared to established healthy gait waveforms and tested by repeated measures ANOVA (α=0.05). Results Pretreatment waveforms in CD had a mean 5.13° of excess FPA during gait cycle phases requiring lower-extremity pronation compared to previously published age-gender-matched healthy waveforms. There was 96% improvement in pronation after five treatments, with a mean 0.21° (p=0.041) of excess FPA. Mean TWSTRs and CDIP-58 scores improved. On physical examination, the rotational direction of C2 vertebrae was contralateral to neck muscle hypertonicity. Vertical sphenobasilar synchondrosis strains were present in those with anterotorticollis. Participants had ipsilateral anterolateral neck muscle and anterolateral abdominal wall muscle hypertonicity. All patients had pelvic somatic dysfunctions with left-side superior relative to right-side and restriction from lower-extremity pronation (i.e., supination dysfunctions). Conclusion The FPA was significantly improved after treatment. This OMM sequence was well tolerated and may be useful for improving gait kinematics in individuals with CD. Randomized, controlled, long-term studies are needed to determine effectiveness.
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  • 文章类型: Case Reports
    背景:宫颈肌张力障碍(CD)是一种罕见且难以治疗的疾病。提供各种神经外科手术选择,每个人都有自己的优点和缺点。我们使用改良的McKenzie-Dandy手术对选择性外周神经支配(SPD)失败的CD患者进行了调查。
    方法:一名42岁男子出现左侧旋转斜颈3年。在接受各种口服药物治疗和反复注射肉毒杆菌毒素后,他被转诊接受手术,但效果不佳。对于第一个操作,患者接受了SPD(伯特兰改良手术);不幸的是,术后结果不令人满意,手术被认为是失败的。6个月后,他的症状没有改善,进行了改良的麦肯齐-丹迪手术。手术后立即,他经历了令人满意的结果。在从暂时的吞咽困难中恢复1个月后,他能够恢复正常的活动和就业。他只抱怨轻微的颈部疼痛,随访3年后未观察到复发。
    结论:对于SPD失败的患者,修改后的麦肯齐-丹迪程序是一个可行和有效的选择。该程序在正确执行时相对安全,长期效果可以维持。
    BACKGROUND: Cervical dystonia (CD) is a rare and difficult-to-treat disorder. Various neurosurgical options are available, each with its own set of advantages and disadvantages. We investigated using the modified McKenzie-Dandy operation for a patient with CD who failed selective peripheral denervation (SPD).
    METHODS: A 42-year-old man presented left-sided rotational torticollis for 3 years. He was referred for surgery after treating with a variety of oral medications and repeated botulinum toxin injections that became ineffective. For the first operation, the patient underwent SPD (modified Bertrand\'s operation); unfortunately, the postoperative outcome was unsatisfactory, and the operation was considered a failure. After his symptoms did not improve after 6 months, the modified McKenzie-Dandy operation was performed. Immediately following surgery, he experienced satisfactory outcomes. He was able to resume his normal activities and employment after 1 month after recovering from his temporary swallowing difficulties. He only complained of minor neck pain and no recurrence was observed after 3 years follow-up.
    CONCLUSIONS: For patients who have failed SPD, a modified McKenzie-Dandy procedure is a feasible and effective option. The procedure is relatively safe when performed properly, and the long-term effects can be maintained.
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