肌萎缩侧索硬化症临床与电生理

目的:探讨肌萎缩侧索硬化症的临床与肌电图特点;方法:对30例肌萎缩侧索硬化症的临床及肌电图进行分析总结;结果:肌萎缩侧索硬化症表现为上下运动神经元同时受累,肌无力、肌萎缩、肌束颤动,伴腱反射亢进、病理征等,无感觉障碍,肌电图特点为广泛分布(多个肢体)的神经源性损害。结论:根据临床及肌电图并除外其它疾病时本病可确诊,肌电图的广泛神经源性损害有助于该病的早期诊断。     

肌萎缩侧索硬化症90例胸锁乳突肌肌电图的特点

目的　探讨胸锁乳突肌肌电图 (EMG)检测在肌萎缩侧索硬化症 (ALS)诊断中的价值。方法　对ALS患者均进行常规EMG检测 ,包括上肢、下肢及胸锁乳突肌 ,测定自发电位 ,募集相 ,运动单位电位的时限、波幅及多相波。比较有、无球部症状的患者之间胸锁乳突肌EMG改变的差异 ,以及不同部位即球部、颈部及腰骶部之间EMG检测结果的差异。结果　有球部症状的患者胸锁乳突肌EMG病变程度重于无球部症状者 (时限增宽幅度分别为 60 6 %± 2 1 2 %和 50 0 %± 1 9 2 % ,P <0 0 5)。胸锁乳突肌EMG自发电位、募集相、运动单位电位时限和波幅改变的程度均不同于上肢和下肢 ,存在显著性差异。结论　胸锁乳突肌EMG检测的异常与球部症状相关 ,而与肢体无关 ,其神经源性改变可反映球部下运动神经元病变     

肌萎缩侧索硬化患者肌电图与ALS-FRS-R的研究

目的研究肌萎缩侧索硬化(ALS)患者肌电图(EMG)相关肌肉小力收缩时运动单位动作电位(MUAP)的波幅(Amp)和时限(Lat)与肌萎缩侧索硬化功能评分(ALS-FRS-R)之间的相关性。方法 25例ALS患者分别进行ALS-FRS-R和EMG检查,分别记录并分析左右胫骨前肌、左右伸指总肌、腹直肌及胸锁乳突肌小力收缩时MUAP的Amp和Lat与ALS-FRS-R的相关性。结果 23例ALS患者右胫骨前肌小力收缩时Amp与ALS-FRS-R存在相关性,R2=0.173,P=0.043。左胫骨前肌、左右伸指总肌、腹直肌及胸锁乳突肌小力收缩时的Amp及左右胫骨前肌、左右伸指总肌、腹直肌及胸锁乳突肌小力收缩时的Lat与ALS-FRS-R不存在相关性(P>0.05)。结论 EMG中仅个别相关肌肉小力收缩时MUAP的Amp与ALS-FRS-R相关,因此EMG相关肌肉小力收缩时的Amp和Lat对ALS仅具有定性意义,不能反映ALS患者病情的严重程度。     

肌萎缩侧索硬化症152例下胸段脊旁肌肌电图特点

目的探讨下胸段脊旁肌肌电图在肌萎缩侧索硬化症(ALS)诊断中的应用价值。方法总结152例确诊ALS患者的临床及电生理资料,观察下胸段脊旁肌肌电图的特点,并分析其与病程、年龄、首发部位、呼吸困难、胸锁乳突肌肌电图、舌肌肌电图的相关性。结果152例ALS中,下胸段脊旁肌肌电图出现自发电位者125例(82．24%)；胸锁乳突肌肌电图呈神经源性损害83例(55．33%),其中出现自发电位者45例。通过Logistic多元回归分析提示脊旁肌肌电图与病程、年龄有关,而与首发部位、呼吸困难无明显相关性。结论脊旁肌肌电图与ALS患者的病程及年龄有关,且下胸段脊旁肌肌电图方便易行,在ALS中诊断价值较大。     

肌萎缩侧索硬化的诊断标准(草案)

肌萎缩侧索硬化 (ａｍｙｏｔｒｏｐｈｉｃｌａｔｅｒａｌｓｃｌｅｒｏｓｉｓ,ＡＬＳ)是成人运动神经元病中最常见的形式。其年发病率为 10万分之 2～ 3。以进行性上、下运动神经元损害为主。临床上表现为骨骼肌肉萎缩、无力、直至呼吸麻痹而死亡为特征。由于ＡＬＳ起病隐匿 ,早期症状不典型等原因 ,该病的诊断尚存在一定的困难。随着国内外对本病研究的深入 ,临床药物治疗和临床药物试验的开展 ,对本病的早期和正确诊断已很有必要。世界神经病学联盟于 1994年在西班牙首次提出ＡＬＳ的诊断标准 ,1998年春又对诊断标准进行了修订。我国中华医…     

肌萎缩侧索硬化三例误诊分析

目的掌握肌萎缩侧索硬化(ALS)的诊断标准,以便早期准确诊断,避免误诊。方法分析3例ALS患者早期被误诊的临床资料。结果 3例患者均以下肢无力发病,逐渐波及上肢或对侧肢体,脊柱MR I示颈部或腰部椎间盘突出压迫硬膜囊,手术治疗后,症状无缓解,病情仍进行性加重,经肌电图检查证实为ALS。结论临床医师应熟知ALS的诊断标准,对患者详细询问病史、认真查体和电生理检查是减少ALS误诊的关键。     

肌萎缩侧索硬化诊断及治疗

<正> 概述:肌萎缩侧索硬化(ALS)是一种进展性神经肌肉疾病,由于上、下运动神经元变性导致球部、四肢、躯干的肌肉逐渐无力及萎缩,而动眼肌及括约肌不受累。发病率约1.5/10万,患病率4～6/10万,隐袭起病,进展缓慢,呈致死性,通常由于呼吸衰竭死亡。发病年龄平均55岁,从发病起平均存活3.5年,5年后有20％病人存活,10年有10％存活。5％患者对疾病有抵抗,在发病后可存活20年。一般发病年龄越早,存活时间越长。球部起病者存活期平均约2.2年,     

肌萎缩侧索硬化与颈椎病鉴别诊断方法的评估

目的 探讨早期肌萎缩侧索硬化（ＡＬＳ）与颈椎病鉴别诊断的临床首选方法。方法 对临床已确诊的５３例ＡＬＳ,１４例脊髓型颈椎病（ＣＳＭ）,２３例神经根型颈椎病（ＣＳＲ）患者的临床症状／体征和肌电图异常特征进行百分比统计及其比值比分析,对依据高比值比的临床症状／体征组合与肌电图所作出的鉴别诊断的可行性作比较。结果 以高比值比的临床症状／体征组合实施多步鉴别诊断的真阳性率９８％,真阴性率８６．５％,单凭肌电图难以作出鉴别诊断。结论 高比值比的临床症状／体征组合是ＡＬＳ与ＣＳＭ和ＣＳＲ鉴别诊断的首选临床方法。     

肌萎缩侧索硬化研究进展

<正>肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)是一种主要累及大脑皮质、脑干和脊髓运动神经元的慢性致死性神经系统变性疾病,临床表现为骨骼肌无力和萎缩,进行性加重。其病因、发病机制均不明确,迄今为止还未发现特效治疗方法,患者平均生存期仅3～5 y。其中5%～10%为家族性ALS(fA LS),90%～95%为散发性ALS(sA LS)。本文综述ALS在临床表现及相关生物标记物等方面的发展历程,重点介绍ALS神经电生理及神经影像等技术的应用,利于临床     

22例肌萎缩侧索硬化症的临床分析

目的：探讨肌萎缩侧索硬化（ALS）的临床特征,方法：对22例ALS患者的临床资料进行回顾性分析,结果：ALS在临床上隐袭起病居多,但也可呈亚急性起病。ALS的发展速度可能双往认为的更快,并发呼吸肌麻痹是ALS的长漏诊,EMG检查对ALS的确诊有重要意义,MRI检查对ALS的诊断有一定的辅助意义,结论：ALS是临床上呈性经过的慢性变性疾病,最常见的合并症以及最易导致误诊是颈椎病。EMG和MRI检查分别对ALS的诊断有确诊和鉴别诊断意义。     

肌萎缩侧索硬化症临床诊断进展

肌萎缩侧索硬化症为致命性神经系统变性疾病,主要累及锥体束、脑干和脊髓前角细胞,临床表现呈进行性加重的肌肉萎缩、无力及痉挛,以及认知损害等,与额颞叶痴呆的临床表现存在部分重叠。约有5%的患者为家族遗传性,临床表现与散发型相似。诊断主要基于患者临床表现、世界神经病学联盟公布的共识,同时排除临床表现相似的疾病。基因检测为加速诊断进程、早期干预提供了新的途径,部分基因突变与特异性表型相关,可据此进行预后评价和遗传学咨询。     

The physician-patient relationship in amyotrophic lateral sclerosis

The principal models of the physician-patient relationship are analysed in terms of their historical development. An outline is given of the clinical, psychological and ethical particularities of the approach to patients with amyotrophic lateral sclerosis. The peculiarities of this disease are so exclusive that they do not resemble other progressive diseases with a negative prognosis, and therefore require an equally exclusive approach to the physician-patient relationship. This approach should not only be informative, scientific and interpretative-deliberative, but most simultaneously be founded on a solid therapeutic alliance aimed at seeking the best interests of the patients while respecting their autonomy as well as their “good” (not only in the sense of physical well-being, but also in terms of respect for their personal values). This is the only way to confront the conflicts that inevitably arise (especially in advanced stages of the disease) without the risks associated with a desire to escape or to adopt extreme solutions (such as euthanasia and therapeutic insistence) and without the risk of burn-out. Received: 5 May 2000 / Accepted in revised form: 6 December 2000     

Clinical features of pain in amyotrophic lateral sclerosis: A clinical challenge

Pain in amyotrophic lateral sclerosis (ALS) is paradoxical in this disease of the upper and lower motor neurons. As such, it remains an underestimated and neglected clinical problem because it is poorly identified by physicians, its mechanisms are numerous and its treatments are generally not effective. Pain may be primary in the form of cramps, spasticity and neuropathy, or secondary as nociceptive pain, and may arise before the first motor symptoms. It may also lead to depression and, in all cases, affect patients’ daily activities and quality of life. Given the high frequency of pain in ALS, the use of analgesic or sedative drugs is necessary and should reduce the course of the disease. Nevertheless, it is important to understand the pathophysiological mechanisms of pain in ALS, and to train physicians how to detect ALS pain early on and provide dedicated treatments. In France, the implementation of ALS centers is a positive response to the public-health problem resulting from this disorder.     

Respiratory function in amyotrophic lateral sclerosis

Abstract. The aim of this study was to examine the vital capacity (FVC) and forced expiratory volume in 1 s (FEV 1) in relation to the site of amyotrophic lateral sclerosis (ALS) onset and the duration of the disease. Respiratory involvement is the principal cause of death in ALS patients. The study was conducted at the Department of Neurology, University School of Medicine in Lublin. The study comprised 18 ALS patients. The average duration of ALS was 12 months. The patients were divided into two groups according to the site of ALS onset and into two groups according to the duration of the disease. FVC was significantly higher in the group of patients with a limb onset than in the group of patients with a bulbar onset of the disease. The study has shown respiratory function disturbances in ALS patients. FVC significantly depends on the site of ALS onset but not on the duration of the disease.     

Clinical features of amyotrophic lateral sclerosis

The clinical features and course of amyotrophic lateral sclerosis are discussed. The data on a series of 116 patients are compared with those of the literature. The following points emerge: 1) when the disease starts before the age of 50, the prognosis is often less poor than usual; 2) the forms with spinal, and especially cervical, onset appear to be less rapid than bulbar forms; 3) in 20% of the patients survival is over 5 years. There may be some unknown factor that increases the resistance of some subjects to the disease.

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Sommario Sono discusse le caratteristiche cliniche ed evolutive della Sclerosi Laterale Amiotrofica. In particolare i dati emersi dallo studio di una casistica di 116 pazienti vengono confrontati con quelli della letteratura. Dall'analisi emergono alcuni dati: 1) quando la malattia esordisce prima dei 50 anni l' evoluzione è frequentemente meno rapida; 2) le forme ad esordio spinale ed in particolare quelle con inizio a carico del midollo cervicale appaiono meno rapide di quelle ad esordio bulbare; 3) nel 20% dei pazienti la sopravvivenza è superiore ai 5 anni. è quindi prospettata la possibilità dell' esistenza di uno sconosciuto fattore di ?resistenza” alla malattia.

     

An open-randomized clinical trial of selegiline in amyotrophic lateral sclerosis

Based on the hypothesis that free radicals play a general role in the neurodegenerative process in motor neuron disease, we tested selegiline in a group of patients affected by amyotrophic lateral sclerosis (ALS) to examine whether it might modify the progression of the disease. Patients were admitted if they were 25–80 years old and had a confirmed diagnosis of ALS with symptoms lasting no longer than 24 months. Patients with familial ALS, pure progressive bulbar palsy, primary lateral sclerosis or progressive muscle atrophy were excluded; a total of 111 patients were recruited. Fifty-three patients were randomly assigned to receive the drug (selegiline 10 mg/day orally for 6 months) and the remaining 58 were considered ALS controls. Mortality was similar in the two groups (4 and 5 patients respectively), though the difference was not statistically significant. Among the survivors, mean MRC and Norris disability scores and forced vital capacity were fairly similar in the two groups at all times and no statistically significant difference between treated and untreated patients was found. The results did not change when the data were related to age, duration and characteristics of onset of the disease. The rate of progression was significantly more rapid in patients with bulbar symptoms in both groups. Our data do not show any significant effect of selegiline in modifying the progression of ALS.     

Minocycline in amyotrophic lateral sclerosis: a pilot study

Abstract Recent studies indicate that minocycline exerts neuroprotective effects in vitro and in vivo, and suggest that the drug may represent a novel therapeutic approach to amyotrophic lateral sclerosis (ALS). In this study we investigated the safety of combined treatment with minocycline and riluzole in ALS. Twenty ALS patients were randomised into two groups and administered either riluzole (50 mg b.i.d.) or riluzole and minocycline (100 mg i.d.) for 6 months. Disease progression was measured by means of the ALS-Functional Rating Scale score at monthly intervals. Respiratory function was measured at the beginning of the study and repeated after 3 and 6 months of treatment. Combined treatment with minocycline and riluzole was not followed by significant side effects. This pilot study shows that minocycline and riluzole can be taken safely together. Further trials are needed to assess efficacy of such treatment.