肌萎缩侧索硬化的康复研究现况

肌萎缩侧索硬化（ALS）是一种致命的、进展的神经变性疾病,它主要损害大脑及脊髓的上下运动神经元,导致患者瘫痪、呼吸肌麻痹甚至死亡。目前该病发病机制尚不清楚,做到准确的早期诊断非常困难,更无有效的治疗措施去改善患者神经功能缺损的状况。康复治疗对ALS患者有着重要意义,它能帮助患者改善功能状况,提高生活质量。目前仍缺乏ALS康复相关的有力证据,未来需要对ALS康复的各个领域进行大规模的临床及基础研究,广泛建立ALS中心,规范并指导ALS患者的康复治疗。本综述全面探讨ALS的康复研究现况,强调了多学科管理的重要性。     

运动训练治疗肌萎缩侧索硬化症的研究进展

肌萎缩侧索硬化症(ALS)是一种累及上、下运动神经元的神经退行性疾病,目前尚无法治愈。针对ALS的临床管理较复杂,需要多学科合作,对症治疗及康复治疗是重要干预手段。运动训练能防止ALS患者肌肉废用性萎缩,同时也有助于患者心血管系统健康,被认为是ALS患者的一种潜在治疗方法,但仍存在一定争议。本文将阐述运动训练治疗ALS的机制、临床应用及对ALS的影响,以期为运动训练治疗ALS患者提供更多参考资料。     

基因检测在肌萎缩侧索硬化诊断中的意义

正肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)是一种持续进展的神经变性病,可导致肌无力、肌萎缩并最终死亡,患者的中位生存期为3~5年。ALS的发病率在欧洲和北美为每年(1.5~2.7)/100 000,患病率为(2.7~7.4)/100 000。目前虽然尚没有治愈ALS的方法,但大量研究表明,药物力如太、经皮胃造瘘、无创正压通气及对症支持治疗均     

肌萎缩侧索硬化干细胞治疗研究进展

<正>肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)是一种成年起病的致死性的慢性进行性神经变性疾病,临床上肢体和延髓的上、下运动神经元损害并存。除利鲁唑(riluzole)可延长存活时间数月外,目前尚无其他确切有效的临床治疗方法,患者诊断后平均存活期2.5~5.0年,最终死于呼吸衰竭。鉴于ALS的确切发病和进展的机制尚不明确,针对性靶向治疗的研究至今难以取     

肌萎缩侧索硬化症的神经传导速度研究新进展

肌萎缩侧索硬化症(ALS)是一种最普遍的成人运动神经元病,1869年由Charcot首次报道,是一种快速进展的、致命的中枢神经系统恶性变性病,主要累及大脑皮质、脑干和脊髓的神经元.这种广泛的神经元退化会导致肌无力、萎缩及痉挛.ALS病程是分阶段的,病因及发展过程形式多样,目前无有效的治疗措施来缓解其进展[1],利鲁唑也只能延长2～3个月的生命,80％～90％的患者从出现症状开始平均2～3年死亡[2]. 尽管目前ALS有适用的诊断标准,但因其临床特征多变性及缺乏生物学标记,诊断仍很困难.神经传导的研究对于诊断ALS是一种相对精确的方法,对于早期诊断、诊断疑似ALS排外其他原因引起的疾病方面很重要[3].在此就ALS的神经传导速度的研究现状予以综述.     

加味四君子方辅治肌萎缩侧索硬化症脾虚证的临床效果与安全性评价

目的评价加味四君子方在改善肌萎缩侧索硬化症(amyotrophic lateral sclerosis,ALS)脾虚证患者生活功能及延缓ALS疾病进展中的价值。方法采用随机、双盲、安慰剂对照的临床试验设计方案,将2013年1月—2015年12月上海中医药大学附属曙光医院50例ALS脾虚证患者均分为加味四君子方组和安慰剂组。加味四君子方组在西药治疗基础上加用加味四君子方;安慰剂组在西药治疗基础上加用安慰剂。两组均服药9个月。观察比较两组治疗前和治疗后3、6、9个月及停药后3个月时,ALS功能评分量表(ALSFRS-r)评分和ALS中医脾虚症状评分及治疗期间不良反应发生情况。结果经广义估计方程分析,两组治疗后ALSFRS-r评分、中医脾虚症状评分比较差异均有统计学意义(Waldχ~2=594.155,P0.05;Waldχ~2=452.196,P0.05),特别是在中医脾虚症状方面,加味四君子方组能有效延迟相关症状的恶化。对ALSFRS-r中精细动作、粗大运动评分的时间级别分析结果显示,两组间比较差异有统计学意义(Waldχ~2=166.976,P0.05;Waldχ~2=97.015,P0.05);但对延髓症状、呼吸症状评分的时间级别分析结果显示,两组间比较差异无统计学意义(Waldχ~2=0.007,P0.05;Waldχ~2=1.918,P0.05)。且两组治疗期间无严重不良反应事件发生。结论加味四君子方可以延缓ALS脾虚证患者神经功能恶化,对粗大动作、精细动作功能有良好的疗效。     

肌萎缩侧索硬化症患者跌倒发生现状及影响因素分析

目的调查肌萎缩侧索硬化症(ALS)患者跌倒发生现状并分析影响因素。方法采用便利抽样法, 选取2023年2—4月北京大学第三医院神经内科收治的110例ALS患者作为研究对象。采用自设的患者一般资料及疾病相关调查表、Morse跌倒风险评估量表进行问卷调查。结果共纳入100例ALS患者, ALS患者跌倒的发生率为45.0%(45/100);二项Logistic回归分析结果显示, 步态异常、跌倒高风险、下肢肌力异常是ALS患者跌倒的危险因素(P<0.05)。结论步态异常、跌倒高风险、下肢肌力异常是ALS患者跌倒的独立影响因素, 是预防和管理ALS患者跌倒的重要方面。     

骨髓间充质干细胞治疗肌萎缩侧索硬化症的护理

目的观察骨髓间充质干细胞（MSC）治疗肌萎缩侧索硬化症（ALS）的临床效果,探讨护理方法。方法在无菌条件下采集患者骨髓液,密度梯度离心法分离,获取单个核细胞层,接种于培养瓶中,加入培养液,在5％二氧化碳细胞培养箱培养3d,微生物检测安全后静脉输注给患者。结果治疗后能延迟肌萎缩侧索硬化症患者的病情进展,有效8例,无效1例。结论MSC治疗肌萎缩侧索硬化症的临床效果有效。     

肌萎缩侧索硬化症的扩散张量成像研究

目的采用DTI技术,定量检测肌萎缩侧索硬化症（ALS）患者脑内上运动神经元（UMN）的受累情况。方法运用GE Signa3.0T磁共振成像系统,对15例临床确诊及拟诊的ALS患者和12例性别、年龄相当的正常对照者进行了全脑DTI扫描。分别计算双侧大脑皮层下白质、皮质脊髓束及胼胝体各部的各向异性分数（FA）和平均扩散系数（MD）,并与多种临床参数进行相关分析。结果ALS患者脑内的FA值在双侧中央前后回皮层下白质、放射冠、内囊后肢后部、大脑脚及胼胝体各部较正常对照者显著下降;而相应部位的MD值无显著变化。FA值与ALSFRS及最大手指敲击率呈正相关,与病程、年龄及病情进展速度呈负相关;MD值与ALSFRS、病情进展速度及年龄呈负相关,而与其他临床指标之间无显著相关性。结论DTI可以通过定量检测ALS患者脑内FA及MD值的变化帮助确定上运动神经元的受累情况,尤其是在临床诊断不是很明确时。     

基于体素形态学和表面形态计量学MRI观察肌萎缩侧索硬化患者大脑结构改变

目的 探讨肌萎缩侧索硬化（ALS）患者大脑形态结构的改变,以期寻找有效的早期诊断影像学标记。方法 采集27例临床确诊ALS患者（ALS组）和27名健康志愿者（对照组）的3D-T1W脑结构像,分别以基于体素形态学（VBM）和基于表面形态计量学（SBM）方法对全脑结构进行分析。将2种算法得到的ALS组有显著差异脑区的灰质体积与临床修订版ALS功能评分量表（ALSFRS-R）评分分别进行相关分析。结果 VBM分析结果显示,与对照组比较,ALS组双侧中央前回、左侧背侧丘脑、右侧辅助运动区、左侧中央沟盖、双侧中央后回等运动相关脑区灰质体积显著减小,双侧额叶、颞叶、枕叶及海马旁回等运动区以外的脑区灰质体积亦缩小。SBM分析结果显示,与对照组比较,ALS组右侧BA4a、BA4p区灰质体积和皮层厚度降低。ALS组右侧BA4a区灰质体积与ALSFRS-R评分呈正相关（r=0.418,P=0.038）。结论 ALS患者大脑运动区和运动区域以外脑结构均有所改变,BA4a区的灰质体积及皮层厚度有望作为生物学标记物,用于监测疾病病程进展。     

The demand for prehospital advanced life support and the appropriateness of dispatch in Taipei

INTRODUCTION: Implementing prehospital advanced life support (ALS) services requires more medical and societal resources in training and equipment. The actual demand for ALS services in our communities was not clear. To ensure good use of expensive resources, it is important to evaluate the demand and appropriateness of ALS services before full-scale implementation takes place. OBJECTIVE: To evaluate the rate and characteristics of demand for ALS, and the appropriateness of ALS dispatch of the emergency medical service (EMS) system in metropolitan Taipei City. METHODS: A retrospective, cross-sectional analysis of the EMS records of Taipei City Fire Department from April 1999 to December 2000 was conducted. Stratified random sampling of all EMS records in the second week of January, April, July and October of 2000 were obtained, along with the corresponding ALS dispatch records. Retrospective ALS demand criteria, including the chief complaints, mechanisms of injury/illness, initial vital signs and types of care rendered, were developed to estimate the rate of ALS demand. ALS demand is expressed as the percentage of cases fulfilling ALS criteria over the total number of EMS cases. Appropriate ALS dispatches were those ALS dispatches determined as fulfilling the ALS demand criteria. RESULTS: Among the sampled 5433 EMS cases, 490 (9.02%) were determined as a demand for ALS care. ALS demands varied from region to region, and were higher during winter months and afternoon rush hours. There were 175 actual ALS dispatches, accounting for 3.22% of the sampled EMS services. The triage performance was suboptimal: the appropriateness of ALS dispatch was 37.14%; the overtriage rate was 72.86%. CONCLUSION: Around nine percent of EMS calls demand ALS services. The current triage performance for proper ALS dispatch was suboptimal. A correct ALS dispatch protocol and more dispatcher training programmes should be established in the communities to ensure best use of valuable ALS resources.     

Does advanced life support provide benefits to patients?: A literature review

INTRODUCTION: Emergency medical services have invested substantial resources to establish advanced life support (ALS) programs. However, it is unclear whether ALS care provides better outcomes to patients compared to basic life support (BLS) care. OBJECTIVE: To evaluate the current evidence regarding the benefits of ALS. METHODS: Electronic medical databases were searched to identify articles that directly compared ALS versus BLS care. A total of 455 articles were found. Articles were excluded for the following reasons: (1) the article was not written in English; (2) BLS response was not compared to an ALS response; (3) a physician or nurse was included as part of the ALS response; (4) it was an aeromedical response; or (5) defibrillation was included in the ALS, but not the BLS, scope of care. Twenty-one articles met the inclusion criteria for this literature review. RESULTS: Results were divided into four categories: (1) trauma; (2) cardiac arrest; (3) myocardial infarction; and (4) altered mental status. Trauma: The majority of articles showed that ALS provided no benefits over BLS in urban trauma patients. In fact, most studies showed higher mortality rates for trauma patients receiving ALS care. Further research is needed to evaluate the benefits of ALS for rural trauma patients, and whether ALS care improves outcomes in subgroups of urban trauma patents. Cardiac Arrest: Cardiac arrest studies show that early CPR plus early defibrillation provide the greatest improvement in survival. However, most cardiac arrest research includes defibrillation as an ALS skill which has now moved into the BLS scope of care. The 2004 multi-center OPALS study provided good evidence that ALS does not improve cardiac arrest survival over early defibrillation. Further research is needed to address whether any ALS interventions improve cardiac arrest outcome. Myocardial Infarction: Only one study directly compared the outcome of BLS and ALS care on myocardial infarction. The study found no difference in outcomes between BLS and ALS care in an urban setting. Advanced Life Support: Only one study directly compared the outcome of BLS and ALS care on patients with altered mental status. The study found that the same number of patients had improved to "alert" on arrival at the emergency department, but there was a decreased length of emergency department stay for patients treated by ALS for hypoglycemia. Limitations: This review article does not take into account the benefits of 2005 ALS interventions, such as thrombolytics, dextrose, or nitroglycerin, since no studies directly compared these interventions to BLS care. Furthermore, only one study in this literature review was a large, multi-center trial. CONCLUSIONS: ALS shows little, if any, benefits for urban trauma patients. Cardiac arrest studies show that ALS does not provide additional benefits over BLS-defibrillation care, but more research is needed in this area. In two small studies, ALS care did not provide benefits over BLS care for patients with myocardial infarctions or altered mental status. Larger-scale studies are needed to evaluate which specific ALS interventions improve patient outcomes.     

To lead or not to lead? Prospective controlled study of emergency nurses' provision of advanced life support team leadership

Background and objectives: In many emergency departments advanced life support (ALS) trained nurses do not assume a lead role in advanced resuscitation. This study investigated whether emergency nurses with previous ALS training provided good team leadership in a simulated cardiac arrest situation. Methods: A prospective study was conducted at five emergency departments and one nurses'' association meeting. All participants went through the same scenario. Details recorded included baseline blood pressure and pulse rate, time in post, time of ALS training, and subjective stress score (1 = hardly stressed; 10 = extremely stressed). Scoring took into account scenario understanding, rhythm recognition, time to defibrillation, appropriateness of interventions, and theoretical knowledge. Results: Of 57 participants, 20 were ALS trained nurses, 19 were ALS trained emergency senior house officers (SHOs), and 18 were emergency SHOs without formal ALS training. The overall mean score for doctors without ALS training was 69.5%, compared with 72.3% for ALS trained doctors and 73.7% for ALS trained nurses. Nurses found the experience less stressful (subjective stress score 5.78/10) compared with doctors without ALS training (6.5/10). The mean time taken to defibrillate from the appearance of a shockable rhythm on the monitor by the nurses and those SHOs without ALS training was 42 and 40.8 seconds, respectively. Conclusion: ALS trained nurses performed as well as ALS trained and non ALS trained emergency SHOs in a simulated cardiac arrest situation and had greater awareness of the potentially reversible causes of cardiac arrest. Thus if a senior or middle grade doctor is not available to lead the resuscitation team, it may be appropriate for experienced nursing staff with ALS training to act as ALS team leaders rather than SHOs.     

Emerging mechanisms of molecular pathology in ALS

Amyotrophic lateral sclerosis (ALS) is a devastating degenerative disease characterized by progressive loss of motor neurons in the motor cortex, brainstem, and spinal cord. Although defined as a motor disorder, ALS can arise concurrently with frontotemporal lobal dementia (FTLD). ALS begins focally but disseminates to cause paralysis and death. About 10% of ALS cases are caused by gene mutations, and more than 40 ALS-associated genes have been identified. While important questions about the biology of this disease remain unanswered, investigations of ALS genes have delineated pathogenic roles for (a) perturbations in protein stability and degradation, (b) altered homeostasis of critical RNA- and DNA-binding proteins, (c) impaired cytoskeleton function, and (d) non-neuronal cells as modifiers of the ALS phenotype. The rapidity of progress in ALS genetics and the subsequent acquisition of insights into the molecular biology of these genes provide grounds for optimism that meaningful therapies for ALS are attainable.     

Early respiratory insufficiency in the ALS patient: a case study.

Respiratory insufficiency is a problem that develops in nearly all people diagnosed with amyotrophic lateral sclerosis (ALS). Noninvasive positive pressure ventilation (NIPPV) is the treatment of choice for ALS patients with respiratory insufficiency. Forced vital capacity (FVC) is the test most commonly used to qualify ALS patients for NIPPV; however, some research suggests FVC may not be the best tool to measure early respiratory insufficiency in all patients with ALS. This case study introduces an ALS patient who had normal FVC results, symptoms of respiratory insufficiency, and abnormal nocturnal oximetry. After NIPPV initiation, the patient reported improved sleep and less daytime fatigue, which he associated with the start of NIPPV treatment.     

Optimizing care of patients with ALS. Steps to early detection and improved quality of life

Primary care physicians should be alert for early, purely motor neurologic signs and symptoms of ALS, a progressive and ultimately fatal motor neuron disease. Because many neuromuscular disorders mimic ALS, careful differential diagnosis is essential. All patients with signs of motor neuron dysfunction need prompt referral to a neurologist at a regional ALS care center to ensure that they receive an accurate diagnosis and disease-specific multidisciplinary care. Once the diagnosis is confirmed, primary care physicians should work closely with the ALS center to ensure continuity of care and provide emotional support for both patient and family. Patients may also benefit from enrollment in treatment trials and the ALS CARE database. Riluzole, the only medication approved for treatment of ALS, has been shown to slow disease progression and prolong survival. Such benefits have provided new hope to patients and have spurred investigators to search for other, more effective medications for use alone or in combination.     

Long-term survivors with artificial liver support in fulminant hepatic failure.

Clinical ability of artificial liver support (ALS) has been improved greatly in recent years which has allowed us to encounter long-term survivors with fulminant hepatic failure (FHF) whose liver function has been almost completely lost. This suggests that application of ALS in patients with FHF gains time while awaiting transplantation as well as time for functional recovery and regeneration of the liver graft following receipt of the graft with marginal function and/or size. Thus, ALS will contribute greatly to extending the indications for liver transplantation and increase the number of patients receiving and benefiting from this treatment. On the other hand, introduction of ALS prolongs the duration of intensive treatment which increases the risk of infection and increases medical costs. In addition, when to discontinue intensive treatment of patients whose level of consciousness is maintained only by ALS is controversial. Thus, further investigation will be needed to establish a consensus on indications for long-term ALS in FHF.     

Amyotrophic lateral sclerosis: current understanding.

Amyotrophic lateral sclerosis (ALS), or motor neuron disease (MND) as it is usually termed in the United Kingdom, is a fatal degenerative disease resulting in progressive weakness and wasting of voluntary muscles. The disease is caused by degeneration of upper motor neurons in the motor cortex and of lower motor neurons in the brainstem and spinal cord. This combined loss of function causes spastic paralysis, flaccid muscle weakness, wasting, and fasciculations. The disease process spares the sensory, autonomic, and oculomotor neurons. ALS is the most common of the MND syndromes in adults. Although the cause of ALS is unknown, there is evidence that the excitatory neurotransmitter glutamate plays an important role in neuronal cell death in the disease. Several risk factors, such as exposure to welding and soldering, inhalation of lead vapor, exposure to chemicals, and electrical trauma are postulated as contributing to the pathogenesis of ALS. About 90% of all ALS patients have the sporadic form. Approximately 20% of all familial ALS cases are associated with mutations of the copper/zinc superoxide dismutase-1 gene. What is not clear is what factors contribute to the causation of the more common sporadic cases. The drug riluzole has neuroprotective effects in ALS and is the only disease-specific treatment available to date. Riluzole has been approved by the National Institute for Clinical Excellence for use in the National Health Service of the United Kingdom. Other treatments are aimed at managing the devastating symptoms of ALS.     

Advanced life support skills undertaken by nurses--UK survey.

Many nurses successfully complete the Resuscitation Council (UK), European Resuscitation Council, advanced life support (ALS) Provider Course. Acquiring ALS provider status is not necessarily a licence to practice and individual hospital policy determines which skills these nurses can then perform without direct medical supervision. This postal survey aimed to determine which ALS skills are utilised by nurse ALS providers working in a variety of clinical areas within acute hospitals in the UK. A questionnaire was sent to the Resuscitation Officer or Nursing Director of all acute hospital groups in the UK. Almost 261 (87%) of the questionnaires were completed and returned. Nurse ALS providers in 99% of coronary care units, 89% of intensive care units, and 88% of accident and emergency departments undertook manual defibrillation. The majority of hospitals ran compulsory in-house training sessions for intravenous cannulation. Laryngeal mask insertion by nurse ALS providers was permitted in 19% of coronary care units and in the wards of 16% of the responding hospitals. Tracheal intubation by nurse ALS providers working in coronary care units, intensive care units and emergency departments was permitted by 11% of the responding hospitals. This survey has demonstrated that many acute hospitals do not permit nurse ALS providers to use a number of the skills taught on the ALS provider course. General ward-based nurse ALS providers, in particular, are restricted in the ALS skills, they are permitted to use. It would be more efficient for nurses to be trained and assessed specifically in skills they are then permitted to use. Having been assessed in a given skill and achieved a nationally recognised standard, nurse ALS providers should be permitted to use it in clinical practice.