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1.
目的分析和探讨肌电图(EMG)肌源性损害合并神经源性损害的临床和电生理特点。方法检索作者医院EMG数据库,收集EMG表现为肌源性损害合并神经源性损害患者共71例,对其临床和电生理特点进行回顾性分析。结果在此组患者中,最常见的是结缔组织病,共63例(占88.7%)。其余依次为:肿瘤、进行性肌营养不良、AIDS、甲状腺功能亢进(甲亢)。在全部患者中,临床表现为近端无力者55例(占77.5%),有感觉症状体征者27例(占38.0%),肌肉萎缩者12例(占16.9%)。患者的三角肌52例(占73.2%)、股四头肌49例(占69.0%)EMG表现为肌源性损害,其中上下肢同时为肌源性损害者32例(占45.1%)。所合并存在的神经源性损害主要表现为多发性周围神经病(39例)、单神经病(27例)、颈或腰骶神经根病(5例)。结论EMG检查有助于检出临床下神经肌肉病变,电生理检查发现肌源性合并神经源性损害的同时分析肌病所合并周围神经病变类型将有助于揭示病变性质。  相似文献   

2.
肌电图干扰相自动分析在神经源性和肌源性损害中的应用   总被引:1,自引:0,他引:1  
目的 探讨肌电图干扰相自动分析(QIP技术在神经源性和肌源性疾病中的应用价值。方法 记录40例健康人、20例神经源损害和20例炎性肌病患者的肱二头肌在不同强度自主收缩时运动单位动作电位的反折数(NT)与平均波幅(MA)的比值,并以“云图”的方式表示。结果 神经疾病组和肌病组QIP阳性率分别为75%和65%,前者表现为NT:MA比值降低,后者表现为NT:MA比值升高。QIP的异常与临床上肌肉无力的程度有关。中重度肌肉无力的阳性率分别为89%和80%,高于轻度肌肉无力组。结论 尽管该方法不如常规同心圆针电极肌电图(EMG)敏感,但操作简单,重复性好,可作为初步筛选和疗铲判断的客观指标。  相似文献   

3.
目的探讨多发性肌炎(PM)、间质性肌炎(IM)与神经肌炎(NM)的临床特点及神经肌肉活检的诊断价值。方法回顾分析247例炎症性肌病的临床表现及神经肌肉活检结果。结果3组临床表现相似,为近端肌无力、肌痛等;均有不同程度的肌酶增高,但PM组增高明显;IM和NM具有PM的病理改变,但炎性程度不及PM,又各有其特异性,IM组为炎性细胞浸润间质,NM组神经活检多有髓鞘脱失、炎性细胞浸润。结论PM、IM、NM炎性肌病的临床表现相似,诊断困难,需结合神经肌肉活检等辅助检查才能作出正确的诊断。  相似文献   

4.
脂质沉积性肌病合并周围神经病的临床和神经电生理研究   总被引:2,自引:0,他引:2  
目的研究脂质沉积性肌病合并周围神经损害和不伴周围神经损害的临床及神经电生理特点。方法对19例病理证实的脂质沉积性肌病中7例合并周围神经损害(第一组)和12例不伴周围神经损害的患者(第二组)进行了常规肌电图、正中神经和胜后神经感觉传导速度及运动末端潜伏期测定。结果发现临床上两组不同卢、是前者病程较后者长(P<0.01);肌电图提示运动单位动作电位(MUAPS)时限第一组较第二组宽,去除多相波后更明显(P<0.01),部分合并神经源性损害;神经传导速度测定结果提示,第一组可见感觉和运动神经传导速度减慢及感觉神经动作电位波幅降低。结论对临床上有典型的肌病症状、电生理为肌源性损害合并神经源性损害或单纯神经源性损害应考虑脂质沉积性肌病的可能,肌肉和周围神经活检是非常必要的。  相似文献   

5.
目的:探讨神经肌炎的临床特点以及肌电图(EMG)、神经传导速度(NCV)的诊断价值。方法:分析13例神经肌炎临床表现、EMG、NCV结果。结果:13例患者均以肌肉受累为主要临床表现;11例并发有神经病变的症状及体征。13例患者NCV均有异常。所检测的104根神经NCV异常率为57.7%。F波异常率为26.9%。11例针极EMG呈神经源性与肌源性混合损害,2例呈神经源性损害。结论:EMG、NCV是有价值的电生理诊断方法。确诊神经肌炎需结合临床表现和EMG结果。  相似文献   

6.
15例神经肌炎临床分析   总被引:1,自引:0,他引:1  
目的 探讨神经肌炎的临床特点、实验室检查及诊断标准。方法 对15例神经肌炎患者的临床表现及实验室检查的资料进行分析。结果 发现此病患者有如下特点:15例患者均有不同程度肌无力,2例伴有肌肉酸痛;所有患者均有不同程度血清肌酶升高,其中10例超过正常5倍;全部患者肌电图异常,其中13例呈神经或肌肉神经混合损害,2例呈肌源性损害;14例肌活检中13例呈肌炎性改变,1例病程超过3年者呈肌病改变。结论 临床表现结合实验室检查可以诊断神经肌炎,早期诊断治疗与预后有关。  相似文献   

7.
目的分析脂质沉积性肌病(LSM)的临床及病理特点,探讨脂质沉积性肌病被误诊的原因。方法分析8例确诊为脂质沉积性肌病患者的临床特点、实验室检查、神经电生理及病理学资料。结果LSM的临床特点是四肢近端肌无力和运动不耐受;肌酶谱轻中度甚至重度升高;肌电图表现肌源性损害、神经源性损害或混合性损害;肌肉病理学检查显示肌纤维中大小不等的空泡形成;7例0R0染色显示Ⅰ型肌纤维中大量脂质颗粒沉积,1例显示脂肪成分正常者电镜检查可见肌纤维内大量脂滴沉积,呈串珠状排列。2例进行神经活检,1例正常,1例光镜下部分髓鞘变薄,崩解脱失。电镜下髓鞘板层分离,雪旺氏细胞内出现脂滴沉积。确诊的LSM曾被误诊为多发性肌炎、肢带型肌营养不良、脊肌萎缩症、胶原血管病、慢性吉兰-巴雷综合征、病毒性心肌炎。结论LSM很易被误诊为其他肌病或神经源性肌病,肌肉活检及组化OR0染色可能染色不成功造成阴性结果,电镜检查是诊断LSM的主要依据。  相似文献   

8.
目的探讨强直性肌营养不良(DM)的临床特点,以提高对DM疾病的认识及诊断水平。方法对一DM家系确诊的5例患者临床资料进行收集及回顾性分析,包括患者基本资料、临床表现、血液生化、心电图、肌电图及肌肉活检等。结果 5例DM患者均为慢性病程,以肌强直、肌无力、肌萎缩为主要表现,伴有眼部、心脏、内分泌和生殖、神经等多系统损害如白内障、心律失常、脱发、阳痿、习惯性流产、智能减退等,血清肌酶轻度增高或正常,肌电图具有特征性肌强直放电和肌源性损害,肌肉活检呈非特异性肌病特征。结论 DM是一种以肌强直、肌无力、肌萎缩为主要表现的多系统损害的遗传性疾病,临床表现复杂多样,识别DM的临床特点有助于提高对其诊断水平。  相似文献   

9.
目的分析肌病伴管聚集这种罕见的以细胞器异常为主的先天性肌肉病的临床和病理特点。方法通过临床、电生理及病理分析对本病进行诊断。结果本例临床具有特殊性,既有发作性负荷性肌肉病的特点,又有类重症肌无力的表现;肌电图可见有些肌群有神经源性损害,而重频刺激未见递增和递减现象;病理检查发现肌肉组织具有典型的改变,而且还有神经肌肉接头和周围神经受损。结论本病是一种多系统受损害的疾病  相似文献   

10.
目的 通过研究神经肌炎患者的临床及病理特点来探讨神经肌炎能否作为一种独立的疾病实体。方法 分析26例神经肌炎的临床和病理改变。结果 神经肌炎的主要临床表现为肢体无力、疼痛、肌萎缩。15例肌酶正常,11例肌酶增高,以CK增高为主。肌电图可表现为神经源性损害和/或肌源性损害。病理活检可见神经肌肉大量炎性细胞浸润,神经脱髓鞘改变。结论 神经肌炎与多发性肌炎相比有其独特之处,神经肌炎作为一种独立的疾病,诊断主要依靠肌电图和病理。  相似文献   

11.
OBJECTIVE: Little is known about the relation and sensitivity of macro-EMG (MA-EMG) compared with concentric-needle EMG (CN-EMG) in the detection of neuromuscular disorders. METHODS: CN-EMGs and MA-EMGs were recorded from the right brachial biceps muscle of 40 healthy subjects, aged 17-83 years, 20 patients with neurogenic disorders, aged 25-75 years, and 20 patients with myopathy, aged 18-76 years. Motor unit action potentials (MUAPs) were examined. RESULTS: In patients with neurogenic disorders CN-MUAP duration, CN-MUAP amplitude, percent polyphasia, MA-MUAP amplitude, MA-MUAP area and fibre density were significantly increased. In patients with myopathy, only fibre density was significantly increased. In patients with neurogenic disorders, the sensitivity of CN-EMG was 80%, and that of MA-EMG 85%. In myopathies, the sensitivity was 50% for each technique. Pooling the results of both EMG techniques, the sensitivity increased to 90% in patients with neurogenic disorders, and to 65% in myogenic disease. CONCLUSIONS: MA-EMG has a similar sensitivity in the detection of neuromuscular disorders as CN-EMG. Particularly when myopathy is suspected, both techniques should be applied if one is unrevealing.  相似文献   

12.
Reports show wide variability of electromyography (EMG) in detecting pediatric neuromuscular disorders. The study's aim was to determine EMG/nerve conduction study accuracy compared to muscle biopsy and final clinical diagnosis, and sensitivity for myopathic motor unit potential detection in childhood. Of 550 EMG/nerve conduction studies performed by the same examiner from a pediatric neuromuscular service, 27 children (ages 6 days to 16 years [10 boys; M:F, 1:1.7]) with muscle biopsies and final clinical diagnoses were compared retrospectively. Final clinical diagnoses were congenital myopathies (5 of 27,18%), nonspecific myopathies (biopsy myopathic, final diagnosis uncertain; 6 of 27, 22%), congenital myasthenic syndrome (3 of 27, 11%), juvenile myasthenia gravis (1 of 27, 4%), arthrogryposis multiplex congenita (2 of 27, 7%), hereditary motor and sensory neuropathy (1 of 27, 4%), bilateral peroneal neuropathies (1 of 27, 4%), and normal (8 of 27, 30%). There were no muscular dystrophy or spinal muscular atrophy patients. EMG/nerve conduction studies had a 74% agreement with final clinical diagnoses and 100% agreement in neurogenic, neuromuscular junction, and normal categories. Muscle biopsies concurred with final diagnoses in 87%, and 100% in myopathic and normal categories. In congenital myasthenic syndrome, muscle biopsies showed mild variation in fiber size in 2 of 3 children and were normal in 1 of 3. EMG sensitivity for detecting myopathic motor unit potentials in myopathies was 4 of 11 (36%), greater over 2 years of age (3 of 4, 75%), compared to infants less than 2 years (1 of 7, 14%), not statistically significant (P = .0879). EMGs false-negative for myopathy in infants < 2 years of age were frequently neurogenic (3 of 6, 50%). In congenital myopathies EMG detected myopathic motor unit potentials in 40%, with false-negative results neurogenic (20%) or normal (40%). Because our study has no additional tests for active myopathies, for example Duchenne muscular dystrophy genetic testing, our sensitivity for myopathies is lower than if we used a more global view. In conclusion, EMG detection rate of myopathic motor unit potentials at a young age was low, improving in children over 2 years of age. In neurogenic and neuromuscular junction disorders, the EMG has a very high detection rate. In children with mild to moderate neurogenic EMG findings and normal nerve conduction, a myopathy should always be considered.  相似文献   

13.
脂肪累积肌肉病的病理和肌电图改变之间的关系   总被引:1,自引:0,他引:1  
目的探讨脂肪累积性肌肉病和线粒体脂肪累积性肌肉病的肌肉活检病理改变和肌电图改变之间的关系。方法对33例肌活检确诊患者的肌肉病理改变分为单纯脂肪累积性肌肉病和线粒体-脂肪累积性肌肉病两组,对比两者肌纤维内脂肪沉积的程度以及肌电图改变的差异。结果33.3%为线粒体-脂肪累积性肌肉病,66.7%为单纯脂肪累积性肌肉病。前者肌纤维内脂肪沉积程度显著,7例出现小组样分布的小角状萎缩的肌纤维,后者脂肪沉积的程度相对轻,只有3例患者出现小角状萎缩的肌纤维。在前者81.8%的患者肌电图为肌源性损害、18.2%为混合性损害、45.5%合并周围神经损害。在后者59.1%的患者出现肌源性损害、13.6%出现混合或神经源损害、27.3%肌电图正常、22.7%合并周围神经损害。结论和单纯脂肪累积性肌肉病比较,线粒体-脂肪累积性肌肉病的脂肪滴沉积程度更显著以及更多的患者出现肌纤维萎缩,更易导致骨骼肌的电生理改变以及合并周围神经损害。  相似文献   

14.
Electromyography (EMG), histology, and histochemistry were related in 264 patients with neuromuscular disorders classified according to history and clinical and other laboratory findings. Electromyography and histological and histochemical abnormalities were divided in specific and nonspecific criteria. Specific histochemical criteria alone identified 28% of neurogenic lesions. Criteria of myopathy, obtained from the pattern of electrical activity during 30% of maximal effort, helped to delineate a myopathy when the only abnormality was an increased incidence of polyphasic potentials together with a pattern of full recuitment during maximal effort. Histology, histochemistry, or both, and EMG were concordant with clinical findings in 77% of 188 patients with myopathy and in 91% of 64 patients with neurogenic lesions. The electromyogram was concordant with the clinical classification in 87% of patients with myopathy and in 91% of patients with neurogenic impairment. The biopsy was in agreement with or contributed to the classification in 79% of patients with myopathy and in 92% of patients with neuropathy.  相似文献   

15.
目的探讨Nonaka肌病的临床、肌肉病理及肌肉磁共振特点。方法入选2例患者,女性1例,男性1例,临床表现均以双下肢远端肌肉无力、萎缩为主,双上肢仅轻度受累。血清肌酸激酶轻度升高,肌电图提示肌源性损害,神经传导速度均正常。对患者完善大腿及小腿肌肉磁共振检查,并予以左上肢肱二头肌活检,进行组织学、酶组织化学及免疫组织化学染色,抽取外周静脉血2mL送基因公司进行遗传性肌肉病相关基因测序。结果肌肉病理提示,肌纤维肥大、萎缩、再生,肌纤维内可见镶边空泡,符合肌病样病理改变。肌肉MRI提示,大腿股四头肌脂肪化程度较轻,尤其是股外侧肌未受累及,大腿后组肌群及小腿胫前肌、胫后肌脂肪化程度严重。基因结果均提示GNE基因突变。结论 Nonaka肌病是一种与GNE基因突变相关的常染色体隐性遗传性远端肌病,临床表现特点为胫前肌首先受累,而股四头肌早期不受累。病理改变特点为肌纤维内镶边空泡形成。肌肉MRI可提示肌肉脂肪化的程度及分布规律,为诊断提供依据。  相似文献   

16.
目的探讨运动神经元病(MND)患者胸锁乳突肌(SCM)肌电图的特征。方法回顾性分析461例MND患者及349例非MND患者的临床和肌电图资料。结果MND组SCM肌电图异常率(60.3%)显著高于非MND组(4.6%)(P<0.01);确诊级MND患者SCM肌电图的异常率(77.4%)明显高于其他诊断级(均P<0.01);MND组中,SCM肌电图的异常率(60.3%)低于上、下肢体肌肉的肌电图(93.2%、84.4%)(均P<0.001);MND患者SCM肌电图异常以自发电位(42.5%)和轻收缩时运动单位电位时限增宽(43.2%)最为常见;MND组有延髓症状者SCM肌电图的异常率(71.7%)明显高于无延髓症状者(54.3%)(P<0.05)。结论MND患者SCM肌电图异常率及其特异性高,为延髓肌受累的指征,有助于MND的诊断与鉴别诊断。  相似文献   

17.
Different clinical aspects of debrancher deficiency myopathy   总被引:1,自引:0,他引:1       下载免费PDF全文
OBJECTIVE: To characterise the main clinical phenotypes of debrancher deficiency myopathy and to increase awareness for this probably underdiagnosed disorder. METHODS: The diagnosis of debrancher deficiency was established by laboratory tests, EMG, and muscle and liver biopsy. RESULTS: Four patients with debrancher deficiency myopathy were identified in the Tyrol, a federal state of Austria with half a million inhabitants. Clinical appearance was highly variable. The following phenotypes were differentiated: (1) adult onset distal myopathy; (2) subacute myopathy of the respiratory muscles; (3) severe generalised myopathy; and (4) minimal variant myopathy. Exercise intolerance was uncommon. The clinical course was complicated by advanced liver dysfunction in two patients and by severe cardiomyopathy in one. All had raised creatine kinase concentrations (263 to 810 U/l), myogenic and neurogenic features on EMG, and markedly decreased debrancher enzyme activities in muscle or liver biopsy specimens. The findings were substantiated by a review of 79 previously published cases with neuromuscular debrancher deficiency. CONCLUSIONS: This study illustrates the heterogeneity of neuromuscular manifestations in debrancher deficiency. Based on the clinical appearance, age at onset, and course of disease four phenotypes may be defined which differ in prognosis, frequency of complications, and response to therapy.  相似文献   

18.
目的探讨脂质沉积性肌病(LSM)的临床和肌肉病理特点。方法分析2例LSM的临床特点、辅助检查及肌肉病理资料。结果 LSM的临床表现为进行性肌无力,以近端肌无力为主,伴肌萎缩;发作期血清肌酶轻、中度升高,血脂升高或正常,肌电图多为肌源性损害,合并神经源性损害;肌肉病理为I型纤维受累重,肌纤维内散在大小不等的圆形空泡或缺损(脂质颗粒),线粒体受压变形;对激素治疗反应好。结论临床表现和肌酶检查对LSM的诊断具有重要意义,确诊依靠肌肉病理活检,早期治疗效果好。  相似文献   

19.
The studied group included 179 individuals: a control group of 82 subjects, myopathy patients (55) and neurogenic atrophy cases (42). The records were obtained from 4 muscles (biceps, 1st interosseous muscles, quadriceps muscle, anterior tibialis muscle) using the method of automatic EMG analysis and quantitative conventional method. The obtained results demonstrated that in the control group Anops analysis showed no significant differences in the electromyographic parameters of a given muscle in three age groups (15-30, 31-45 and 46-60 years). The values of the mean duration of single potentials were calculated by the automatic method and in the control group they were shorter (by a mean value of 15%) than the mean duration of these potentials calculated by the conventional method. In cases of neuromuscular diseases the obtained measurements differentiated the normal records from the pathological ones, and myogenic from neurogenic lesions. The EMG parameters in the automatic analysis differentiating most evidently normal EMG from pathological EMG were: amplitude and mean duration of single potentials. These results seem to demonstrate the usefulness of automatic analysis of EMG records.  相似文献   

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