运动神经元病及运动神经元病综合征

目的探讨运动神经元病综合征(MNDS)的病因、发病机制、诊断。方法回顾性分析90例运动神经元病(MND)及MNDS的临床特点和诊疗过程。结果MND38例(42.4%),对症及支持治疗,总有效率21.4%;MNDS52例(57.8%),病因为主治疗,总有效率61.5%。两者疗效差异显著(P<0.01)。结论明确MNDS的病因,对治疗及预后极为重要。     

Evaluation and rehabilitation of patients with adult motor neuron disease.

Adult motor neuron disease (amyotrophic lateral sclerosis [ALS]) is a neurodegenerative disorder characterized by loss of motor neurons in the cortex, brain stem, and spinal cord, manifested by upper and lower motor neuron signs and symptoms affecting bulbar, limb, and respiratory musculature. Clinically, the disease course is characterized by progressive weakness, atrophy, spasticity, dysarthria, dysphagia, and respiratory compromise, ultimately resulting in death or mechanical ventilation in the vast majority of patients. Patterns of presentation and pathological features of the disease, along with clinical and electrophysiologic criteria for diagnosis, are discussed in this review. Since 8% to 22% of patients survive more than 10 years without ventilator use, meticulous medical and rehabilitation management is extremely important to ensure optimal health and quality of life in these patients. Major issues in the care of individuals with ALS include weakness and spasticity, impairments in activities of daily living and mobility, communication deficits and dysphagia in those with bulbar involvement, respiratory compromise, fatigue and sleep disorders, pain, and psychosocial distress. Research in ALS changes rapidly, but is currently focused on potential etiologic factors such as glutamate excitotoxicity, role of oxidative stress, autoimmunity to calcium channels, and cytoskeletal abnormalities, as well as related treatment initiatives including glutamate modulators, neurotrophic factors, antioxidants, antiapoptotic factors, and gene therapy. Recently, mutations in the gene encoding Cu/Zn superoxide dismutase were identified in a subset of familial ALS patients. Riluzole, a glutamate antagonist and Na-channel blocker, became the only drug currently approved for treatment of ALS after studies showed a small positive effect on survival. Until a definitive treatment or cure for ALS is found, the multifaceted rehabilitation team approach remains the best hope for improving health and survival in this devastating illness.     

Nutrition and dietary supplements in motor neuron disease

Compromised nutrition leading to weight loss is a common and significant problem in the amyotrophic lateral sclerosis (ALS) patient population. The benefit of aggressive and early nutritional therapy can profoundly influence the disease course, quality of life, and survival. This article reviews the role of nutrition, both as sustenance and treatment for patients who have ALS. Self-medication with dietary supplements has become increasingly popular within this patient population. Despite their popularity, the efficacy of these compounds has been largely unsupported by formal clinical trials. Available data will be highlighted to provide a basis upon which to advise patients requesting guidance.     

Correlation of polyelectromyographic patterns and clinical upper motor neuron syndrome in hemiplegic stroke patients

OBJECTIVE: To investigate the correlation between movement patterns, measured by polyelectromyography (PEMG), and clinical upper motor neuron (UMN) syndrome in stroke patients. DESIGN: PEMG patterns, recorded from pairs of flexor-extensor muscles during voluntary maneuvers, and motor outcomes were assessed approximately 1 month after stroke (early stage). Motor outcomes were reassessed 6 months later (late stage). PARTICIPANTS: Thirty-nine hemiplegic stroke patients and 18 healthy control subjects. MAIN OUTCOME MEASURES: Passive stretch reflexes (PSRs), Brunnstrom's stages, and walking ability. RESULTS: Six PEMG patterns, varying from complete reciprocal to complete synchrony, were identified. Higher PEMG pattern scores were associated with better Brunnstrom's stages (r > .80), walking ability (r > .39), and some PSRs (r < -.37). PEMG patterns could separate patterns 1 and 2 from patterns 3 and 4 for patients with early Brunnstrom's stages 1 and 2. Patterns 1 and 2 (reduced agonist electromyographic activities) indicated weakness and resulted in the worst motor outcomes. Patterns 3 and 4 (cocontraction and coactivation) indicated spasticity and associated synergistic movements. Patients with patterns of 5 and 6 (reciprocal electromyographic activities) had more selective motor control. CONCLUSIONS: PEMG patterns correlate with clinical UMN syndromes and may allow treatment strategy planning on the basis of underlying motor control, as well as the prediction of final motor outcomes soon after stroke, even in patients who cannot move their legs initially.     

多发性硬化患者脑脊液非髓鞘神经相关蛋白的检测及临床意义

目的:检测复发缓解型多发性硬化(RRMS)患者复发期脑脊液(CSF)和血清中tau、S100、14-3-3蛋白的水平,探讨其临床意义.方法:采用ELISA法检测30例RRMS患者(RRMS组)急性期CSF和血清的tau蛋白、S100蛋白和14-3-3蛋白水平,并与无中枢神经及周围神经损害的临床体征和影像学表现的其他疾病患者和健康志愿者(对照组)比较.对各观察指标与病程、IgG指数、扩展的残疾状况量表(EDSS)评分进行相关性分析.结果:RRMS组CSF tau蛋白明显高于对照组(P＜0.01);CSF S100蛋白、14-3-3蛋白与对照组比较差异无统计学意义(P＞0.05).RRMS组与对照组的血清tau、S100、14-3-3蛋白水平间没有显著差异(P＞ 0.05).CSF tau蛋白与IgG指数明显相关(r=-0.381,P=0.039),与病程、EDSS评分无明显相关(P＞0.05).结论:RRMS急性期存在轴索损伤,CSF tau蛋白与IgG指数相关;未发现神经元和胶质细胞损伤的生物指标有异常.     

运动神经元病患者的认知功能及其相关因素特征

目的：探讨运动神经元病患者是否存在认知功能障碍，分析患者的发病年龄、病程、受教育程度、延髓功能评分、肢体功能评分及运动能力总评分对认知功能的影响。方法：选择于2003—03／12在首都医科大学宣武医院神经内科就诊的运动神经元病患者32例为病例组，所有患者的诊断均符合世界神经病学联盟1998年修定的El Escorial的诊断标准，并行脑CT或脑MRI检查除外了可引起认知功能障碍的其他原发中枢神经系统疾病。选择年龄、性别、受教育程度与病例组匹配，均无既往精神神经疾病病史，且神经系统体检正常的同时期健康志愿者60例为正常对照组。采用扩充痴呆量表评定两组被调查对象的认知功能，包括8个分测验，按文盲至大学4种文化程度，判定标准分别为154，192，208，217分。采用汉密顿抑郁量表评定调查对象有无抑郁情绪，包括24个项目，采用0～4的5级评分。采用改良的Norris量表测定运动神经元病患者的延髓功能、肢体功能，评分范围：0分为最大损害，99分为健康。主要对病例组与正常对照：组扩充痴呆量表、汉密顿抑郁量表评分结果进行比较，采用多元相关与回归分析观察影响运动神经元病患者认知功能的相关因素，计量资料以-↑x&;#177;s表示，计数资料表示为率，两组计量资料之间均数的比较采用t检验，率的比较采用卡方检验。结果：所有调查对象全部进入结果分析。①汉密顿抑郁量表、扩充痴呆量表各项评分均数的比较：汉密顿抑郁量表评分结果显示，病例组与正常对照组抑郁症发病率无显著性差异（x^2=1．458，df=1，P=0．5944）。而病例组扩充性痴呆量表总分、学习、计算、注意、言语能力、抽象思维、记忆的评分均明显低于正常对照组（P〈0．05）。②影响运动神经元病患者认知功能的相关因素分析：运动神经元病患者延髓功能评分与扩充性痴呆量表总分及其抽象思维、记忆、言语分测试呈正相关（P〈0．05），其受教育程度与扩充性痴呆量表总分、记忆及学习分测试呈正相关（P〈0．05）。运动神经元病患者肢体功能评分、改良Norris量表总评分、发病年龄、病程与扩充性痴呆量表各项评分无明显相关性（P〉0．05）。结论：运动神经元病患者大多存在认知功能障碍，其认知功能障碍多具有皮质和皮质下损害的特点，并且受延髓功能损害程度、患者受教育程度等因素的影响，运动神经元病患者认知损害程度与其运动系统受损程度不平行，提示运动神经元病存在运动区域以外的脑组织受累。     

运动神经元病患者的认知功能及其相关因素特征

目的:探讨运动神经元病患者是否存在认知功能障碍,分析患者的发病年龄、病程、受教育程度、延髓功能评分、肢体功能评分及运动能力总评分对认知功能的影响。方法:选择于2003-03/12在首都医科大学宣武医院神经内科就诊的运动神经元病患者32例为病例组,所有患者的诊断均符合世界神经病学联盟1998年修定的ElEscorial的诊断标准,并行脑CT或脑MRI检查除外了可引起认知功能障碍的其他原发中枢神经系统疾病。选择年龄、性别、受教育程度与病例组匹配,均无既往精神神经疾病病史,且神经系统体检正常的同时期健康志愿者60例为正常对照组。采用扩充痴呆量表评定两组被调查对象的认知功能,包括8个分测验,按文盲至大学4种文化程度,判定标准分别为154,192,208,217分。采用汉密顿抑郁量表评定调查对象有无抑郁情绪,包括24个项目,采用0～4的5级评分。采用改良的Norris量表测定运动神经元病患者的延髓功能、肢体功能,评分范围:0分为最大损害,99分为健康。主要对病例组与正常对照组扩充痴呆量表、汉密顿抑郁量表评分结果进行比较,采用多元相关与回归分析观察影响运动神经元病患者认知功能的相关因素,计量资料以x±s表示,计数资料表示为率,两组计量资料之间均数的比较采用t检验,率的比较采用卡方检验。结果:所有调查对象全部进入结果分析。①汉密顿抑郁量表、扩充痴呆量表各项评分均数的比较:汉密顿抑郁量表评分结果显示,病例组与正常对照组抑郁症发病率无显著性差异(χ2=1.458,df=1,P=0.5944)。而病例组扩充性痴呆量表总分、学习、计算、注意、言语能力、抽象思维、记忆的评分均明显低于正常对照组(P<0.05)。②影响运动神经元病患者认知功能的相关因素分析:运动神经元病患者延髓功能评分与扩充性痴呆量表总分及其抽象思维、记忆、言语分测试呈正相关(P<0.05),其受教育程度与扩充性痴呆量表总分、记忆及学习分测试呈正相关(P<0.05)。运动神经元病患者肢体功能评分、改良Norris量表总评分、发病年龄、病程与扩充性痴呆量表各项评分无明显相关性(P>0.05)。结论:运动神经元病患者大多存在认知功能障碍,其认知功能障碍多具有皮质和皮质下损害的特点,并且受延髓功能损害程度、患者受教育程度等因素的影响,运动神经元病患者认知损害程度与其运动系统受损程度不平行,提示运动神经元病存在运动区域以外的脑组织受累。     

针灸治疗运动神经元病的现状

目的:对运动神经元病现状进行系统文献回顾,以期为临床治疗提供参考资料,并探讨进一步发展针刺治疗的策略。资料来源:应用网站检索http://www.chkd.cnki.net1994/2003年中国医学核心期刊的相关文献。检索词:运动神经元病,针刺。资料选择:选择运动神经元病针刺治疗相关文献100篇,排除重复性文献。资料提炼:在100篇文献,符合纳入标准的有53篇,对53篇文献进行分类整理,15篇选为参考文献。资料综合:中医学将运动神经元病归属于“痿证”范畴,认为本虚为主,治疗上以培肝、健脾(胃)、固肾之本为基本原则。针灸治疗此病有一定疗效。在临床上,多采用头针、体针,也有伍用或单用穴位注射方法。然而,多数报道缺乏严格的临床研究设计、随机对照、统一的疗效判定标准和随访,且临床观察病例数较少,故针灸治疗此病的客观疗效尚待进一步科学验证。结论:针刺治疗运动神经元病显示了一定疗效,但仍需要开展大样本随机对照试验对针灸治疗进行科学评价,总结针灸治疗此病规范化方案,为运动神经元病的治疗提供科学有效的针灸疗法。     

运动神经元病患者神经传导阻滞的研究

目的 探讨运动神经元病(MND)患者神经传导阻滞(CB)的诊断标准。方法对86例确诊的MND患者(MND组)、60例正常人(正常组)，14例多灶性运动神经病患者(MMN组)分别进行运动神经传导速度(MCV)测定，采用由近端至远端分段刺激，记录各段刺激引发的复合肌肉动作电位(CMAP)的波幅、时限、面积，并根据不同的诊断标准判定CB。结果MND组中，根据美国电诊断医学会(AAEM)标准有10条神经存在CB(3％)，传统标准有2条神经存在cB(0．5％)，Gustav Pfeiffer标准无神经存在CB；MMN组在各种标准下均存在CB(阳性率分别为96．4％，89．3％，83．9％)；正常组均无CB。结论对MND患者进行CB判定时，根据CMAP波幅的不同，所采用的标准也应不同。     

精神分裂症患者脑脊液单胺类神经递质检测与疗效的相关性研究

目的　探讨精神分裂症患者脑脊液 (CSF)内多巴胺 (DA)、高香草酸 (HVA)含量的变化及其临床意义。方法　 86例首发精神分裂症 ,用利培酮治疗 6个月 ,于治疗前及治疗后第 6个月末采用高效液相色谱 -电化学 (HPLC -ECD)法检测CSF中的DA及HVA含量 ,并与 32名健康人进行对照 ,患者组同时用PANSS量表评定其临床疗效。结果　患者组治疗前CSF中DA和HVA含量分别为 (3.2 3± 0 .36 ) / μmol/L和 (1.99± 0 .4 9) μmol/L ,显著高于对照组 (2 .4 4± 0 .32 ) μmol/L和 (1.4 1± 0 .37)βmol/L ,(P均 <0 .0 1) ;治疗后第 6个月末 ,分别为 (2 .4 9± 0 .35 ) μmol/L和 (1.4 2± 0 .2 8) μmol/L。其前后降低值与PANSS量表总分的减分差值里显著正相关。结论　精神分裂症患者CSF内DA和HVA含量显著增高 ,利培酮对精神分裂症的疗效与DA和HVA含量的变化密切相关 ,同时进一步验证了精神分裂症患者中枢神经系统DA功能亢进的假说     

Exercise effect on creatine phosphokinase elevation in motor neuron disease.

Creatine phosphokinase (CPK) is usually elevated in inflammatory and degenerative muscle disease but is usually reported as normal in neuropathic diseases. A review of the literature indicates, however, that it is elevated in 50 to 75% of patients who have motor neuron diseases, and that these elevations are usually 5 to 6 times normal. The effect of bedrest and moderate exercise on serial CPK values in a patient with motor neuron disease is assessed. Bedrest for 24 hours decreased his CPK to 0.58 of the baseline value; 5 hours following moderate exercise it increased to 1.71 of the basal value. This phenomenon appears to be a response to exercise stimuli rather than to the disease process itself. CPK values up O 1000 IU/L are compatible with the diagnosis of amyotrophic lateral sclerosis (ALS). In patients whose CPK values seem atypical, it is best to have them rest 48 hours before repeating the test.     

肌萎灵注射液治疗运动神经元病的效果观察

目的:探讨肌萎灵注射液治疗运动神经元病的疗效。方法:选择河北以岭医院门诊2000-03/2002-10确诊为运动神经元病的患者852例,随机分为治疗组710例和对照组142例,两组均住院治疗观察3个月。治疗组应用肌萎灵注射液48mL加入50g/L葡萄糖或生理盐水250mL静脉滴注,对照组应用神经营养因子2000BU溶于250mL生理盐水静脉滴注,全组病例,1次/d,25d为1个疗程,休息5～7d,继续下一个疗程。结果:治疗组显效率为22.2%,总有效率为86.4%,肌萎灵注射液能显著改善运动神经元患者的临床症状,如肌肉萎缩、无力、饮食呛咳、吞咽呼吸困难等,而对照组则显示无效。结论:肌萎灵注射液对运动神经元疾病有明显的治疗作用。     

老年反流性食管炎患者的临床特点及食管运动功能分析

【摘要】目的探讨老年反流性食管炎（RE）患者的临床表现、内镜、幽门螺杆菌（Up）感染及食管运动功能特点,为老年RE患者的治疗提供理论依据。方法选取我院近3年来经内镜诊断并行食管测压及食管24hpH值监测的老年RE患者56例与同期检出的中青年RE患者58例,分析两组患者的临床表现、内镜、Hp感染及食管运动功能特点。结果老年组反酸、胃灼热的发生率明显低于中青年组（P〈0．05）。老年组轻中度食管炎发生率低于中青年组,重度食管炎发生率高于中青年组,差异无统计学意义（P〉0．05）。老年组食管裂孔疝（HH）合并率显著高于中青年组（P〈0．05）。老年组伴发Barrett食管（BE）7例（12．5％）,中青年组3例（5．2％）,差异无统计学意义（P〉0．05）。老年组Hp阳性率29．6％;中青年组Hp阳性率26．4％,差异无统计学意义（P〉0．05）。老年组下食管括约肌压力（LESP）、食管体部压力明显低于中青年组（P〈O．05）。反流〉5min次数老年组明显高于中青年组（P〈0．05）。结论老年RE患者的典型症状发生率明显低于中青年人,非典型症状高于中青年人。RE食管黏膜破损程度随年龄增加而加重。老年RE患者HH的发生率增加,BE发生率较中青年人有增高趋势,Hp感染率与中青年RE患者相近。老年人RE患者抗反流能力减弱、食管酸廓清能力下降明显,可出现更严重的食管运动功能障碍。     

Human stem cell grafts as therapies for motor neuron disease

Stem cell transplantation, a widely accepted therapeutic approach to diseases of modular organs such as the heart and the endocrine pancreas, remains a controversial option for the nervous system. However, historical advances in the 1990s on key aspects of neural plasticity and the appreciation of the selective vulnerability of nerve cells and systems in degenerative and traumatic diseases require a re-examination of this scepticism. The successes and failures with prior trophic factor therapies are particularly enlightening; many lessons from this era can be creatively absorbed as the early outcomes with stem cell grafts in animal models of disease are evaluated. Stem cell grafting into spinal cord, traditionally a doomed enterprise because of lack of neurogenicity in this region and multiple early failures, is becoming promising again with recent findings from the authors' laboratory and elsewhere that specific types of cells and methods of culture are associated with much improved biological and functional outcomes.     

自体骨髓干细胞移植治疗运动神经元病的护理

运动神经元病是一种病因不明的选择性侵犯脊髓前角细胞、脑干后角运动神经元皮质锥体细胞以及锥体束的慢性进行性变性疾病。主要通过口服药物抑制中枢系统谷氨酸的释放，减少兴奋性递质的毒性作用，延迟病人发生呼吸功能障碍时间和延长存活期。近年来，随着分子生物学和细胞生物工程技术的发展以及对骨髓干细胞研究认识的深入，人们发现骨髓干细胞可以分化为心肌细胞、血管内皮细胞和神经元细胞等，而且体内移植可以改善病人呼吸和吞咽功能，为运动神经元病病人带来新的希望。我院于2006年3月--2006年10月先后为10例运动神经元病病人进行自体骨髓干细胞移植治疗。现报告如下。     

Immunodeficiency associated with motor neuron disease treated with intravenous immunoglobulin

A man with symptoms of amyotrophic lateral sclerosis and immunodeficiency was treated with intravenous immunoglobulin. After four weekly intravenous injections of 2.5 gm of immunoglobulin, his condition showed progressive improvement when measured by clinical, neurological, and physiological parameters. There was a noticeable increase in general physical well-being, an 80% reduction in fasciculations, and the normalization of sensory and motor nerve conduction velocities.