斜方肌肌电图在肌萎缩侧索硬化早期诊断中的应用

目的 探讨斜方肌肌电图在检测肌萎缩侧索硬化(ALS)延髓部下运动神经元损害中的价值.方法 对100例ALS患者、80例颈椎病患者和100名健康志愿者进行斜方肌肌电图检测,AKS组和颈椎病组同时进行胸锁乳突肌、第一骨间肌、腹直肌、胫前肌肌电图检测,比较3组肌电图的差异.颈椎病组中43例患者进行手术前后斜方肌肌电图比较.结果 ALS组中,病程≤8个月的患者斜方肌肌电图自发电位的出现率高于病程>8个月者[分别为21/30(70%)和28/70(40%),X~2=7.56,P=0.004];斜方肌肌电图与胸锁乳突肌肌电图异常率比较差异无统计学意义.ALS组[波幅(1086.9±152.6)μV,时限(17.2±6.5)ms,多相波23.6%±3.4%]与对照组[波幅(606.7±82.7)μV,时限(11.6±1.8)ms,多相波12.8%±2.2%;q=9.27、4.57、4.12,均P<0.01]、ALS组与颈椎病组[术前,波幅(615.7±90.3)μV,时限(12.1±2.0)ms,多相波13.5%±2.4%]间运动单位动作电位(MUAP)各参数比较,差异有统计学意义(q=8.32、4.25、4.23,均P<0.01).颈椎病患者手术前后斜方肌肌电图MUAP各参数无明显变化,5例术后发现少量自发电位.结论 斜方肌肌电图可作为检测ALS延髓部下运动神经元损害的辅助手段,特别是早期可见自发电位增多. 1.6±1.8)ms,多相波12.8%±2.2%;q=9.27、4.57、4.12,均P<0.01]、ALS组与颈椎病组[术前,波幅(615.7±90.3)μV,时限(12.1±2.0)ms,多相波13.5%±2.4%]间运动单位动作电位(MUAP)各参数比较,差异有统计学意义(q=8.32、4.25、4.23,均P< .01).颈椎病患者手术前后斜方肌肌电图MUAP各参数无明显变化,5例术后发现少量自发电位.结论 斜方肌肌电图可作为检测ALS延髓部下运动神经元损害的辅助手段,特别是早期可见自发电位增多. 1.6±1.8)ms,多相波12.8%±2.2%;q=9.27、4.57、4.12,均P<0.01]、ALS组与颈椎病组[术前,波幅(615.7±90.3)μV,时限(12.1±     

原发性侧索硬化2例报道及相关文献复习

目的 探讨原发性侧索硬化(PLS)的临床诊断、与经典的肌萎缩侧索硬化的关系、神经电生理特点、影像学特点及鉴别诊断.方法 分析我科收治的2例PLS患者的临床资料,并复习相关文献.结果 PLS是隐匿起病,进展缓慢的仅累及上运动神经元的神经退行性疾病,很多初诊为PLS的患者经过长期随访最后发展为肌萎缩侧索硬化,PLS的影像学表现多样,神经电生理检查容易早期发现下运动神经元损伤.结论 对于疑诊PLS的患者,明确是否具有局限性下运动神经元损伤症状十分重要,对患者进行时间的纵断随访对疾病最后的诊断和预后评估意义重大.     

广泛神经源性损害患者的临床分析

目的进一步研究肌萎缩性侧索硬化(ALS)的诊断及预后。方法2008年2月～2010年1月间因缓慢起病、逐渐进展的肌肉无力、萎缩、痉挛、束颤就诊,且针肌电图呈广泛神经源性损害者均纳入登记建档;记录首发起病部位、修订肌萎缩侧索硬化功能评分(ALSFRS-R)以及病程,并分析临床特点。结果(1)广泛神经源性损害患者共80例,19例(23.75%、19/80)为下运动神经元综合征(LMNS)患者,61例(76.25%、61/80)为不同诊断级别的ALS(Awaji-shi ma标准);其中临床肯定型24例(30%、24/80),临床拟诊型22例(27.5%、22/80),临床可能型15例(18.75%、15/80)(χ2=0.067,P=0.381);(2)在不同诊断级别的ALS中,首发起病部位在延髓者,临床肯定型的例数多于拟诊型和可能型(P0.05);(3)ALSFRS-R平均35.35±6.17,平均病程(27.7±22.2)年;与临床拟诊型和可能型患者组比较,肯定型组ALSFRS-R分值(31.00)更小,病程(1.64年)更短。结论广泛神经源性损害的患者中虽然不同诊断级别的ALS患者占大部分,但临床肯定型仅1/3;预后不良的因素为延髓起病、评分低和病程短。     

肌电图广泛神经源性损害和肌萎缩侧索硬化的诊断

目的 探讨肌电图广泛神经源性损害与肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)诊断之间的关系.方法 对2002年1月至2008年12月北京协和医院运动神经元疾病数据库进行回顾性分析,统计肌电图表现为广泛神经源性损害的疾病种类,总结ALS患者首次就诊时肌电图神经源性损害的分布区域和随诊后的结果,并对影响ALS初诊时肌电图表现的因素进行Logistic 回归分析.结果 在首次就诊时,共有298例患者的肌电图表现为广泛神经源性损害,其中ALS 192例(64.4％),进行性肌萎缩36例(12.1％),肯尼迪病13例(4.4％),平山病10例(3.4％),颈椎病或腰椎病9例(3.0％),脊髓性肌萎缩6例(1.3％),多灶性运动神经病5例(1.7％),ALS叠加综合征5例(1.7％),肌病4例(1.3％),遗传性运动神经病3例(1.0％),运动轴索性周围神经病3例(1.0％),脊髓灰质炎后综合征2例(0.7％),未能确定诊断者10例(3.4％).本数据库中,共有213例患者最后确诊为ALS,其中第1次肌电图检查时,8例(3.8％)表现为2个区域神经源性损害,13例(6.1％)表现为1个区域神经源性损害,经随诊3～24个月后,均发展为广泛神经源性损害.Logistic回归分析显示,ALS肌电图广泛神经源性损害的表现与病程、起病部位、发病年龄以及性别无关.结论 广泛神经源性损害的肌电图改变并非仅见于ALS;在疾病发生后一定时期内,ALS也可以仅有1个或2个区域的神经源性损害.     

单纤维肌电图在肌萎缩侧索硬化鉴别诊断中的价值

目的 探讨单纤维肌电图(SFEMG)技术在肌萎缩侧索硬化(ALS)鉴别诊断中的价值.方法 对我院收治的165例ALS患者和145例下运动神经元受累为主的非ALS疾病患者进行伸指总肌SFEMG测定,并测定伸指总肌肌力,按照伸指总肌肌力进行分组,分析不同组之间SFEMG改变的特点.结果 伸指总肌肌力正常者,ALS和非ALS组的平均颤抖(jitter)值分别为(66.1±20.1)、(38.0±9.2)μs(t=9.05),jitter＞55μs的百分比中位数分别为55%、0(Z=-7.81),阻滞所占百分比中位数分别为6.7%、0(Z=-6.93),ALS组各参数均明显高于非ALS组(均P＜0.01).伸指总肌肌力医学研究委员会(MRC)评分≤4者,ALS和非ALS组平均jitter值分别为(93.5±31.2)、(52.8±25.9)μs(t=9.37),jitter＞55μs的百分比中位数分别为86%、20%(Z=-8.46),阻滞所占百分比中位数分别为20%、0(Z=-7.25),ALS组各参数均明显高于非ALS组(均P＜0.01).在MRC评分＞4者,采用平均jitter＞55μ s诊断ALS的敏感性和特异性分别为70.2%和92.7%.结论 当采用SFEMG测定协助ALS的诊断和鉴别时,应尽量选择肌力正常的肌肉.平均jitter、jitter＞55μs的百分比和阻滞在ALS与其他下运动神经元疾病的鉴别诊断中具有重要价值.     

1例脊髓延髓肌萎缩症患者的临床分析

目的探讨脊髓延髓肌萎缩症(spinal and bulbar muscular atrophy,SBMA)的临床特征及诊断。方法对1例四肢无力伴言语含糊的患者进行临床、实验室、神经电生理、基因学检查。结果男性患者,慢性起病,表现为四肢无力,缓慢进行性进展,逐渐出现言语含糊、肌肉萎缩,血清激酶水平升高,肌电图EMG提示神经源性损害。结论脊髓延髓肌萎缩症的临床特征与肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)极其相似,通过病史、查体、实验室、神经电生理、基因检测有利于疾病的诊断。     

单纤维肌电图在肌萎缩侧索硬化和颈椎病鉴别诊断中的价值

目的 探讨单纤维肌电图(SFEMG)技术在肌萎缩侧索硬化(ALS)与神经根型和脊髓型颈椎病鉴别诊断中的价值.方法 对61例ALS伴有MRI颈椎病表现、59例ALS不伴MRI颈椎病表现、55例神经根型和脊髓型颈椎病患者进行伸指总肌SFEMG测定,分析不同组之间SFEMG改变的特点.结果 在3组患者中,平均颤抖(jitter)值分别为(81.2±25.9)、(91.6±32.4)、(40.9±11.8) μs,jitter>55 μs的百分比M50分别为73%、80%、5%,阻滞所占百分比M50分别为10%、20%、0,纤维密度分别为2.9±0.5、2.9±0.6、2.4±0.6.ALS伴和不伴MRI颈椎病变2组之间各参数比较差异无统计学意义.两组ALS患者合并后[平均jitter值(86.3±29.6)μs,jitter>55μs的百分比M50为80%,阻滞所占百分比M50为14%,纤维密度2.9±0.5]再与颈椎病组比较,各参数均明显高于颈椎病组(分别为t=14.49,Z=8.96、7.68,t=5.83,均P=0.000).在经随诊而确诊的18例ALS患者中,初诊时肌电图仅有1个节段的神经源性损害,在伸指总肌肌力和常规肌电图均正常情况下,有16例SFEMG可见纤维密度增高,13例jitter增宽,6例可见阻滞.结论 ALS伴或不伴MRI颈椎病变的SFEMG均表现为jitter明显增宽,可伴有阻滞,纤维密度增高,与神经根型和(或)脊髓型颈椎病患者明显不同.SFEMG测定有助于ALS与颈椎病的鉴别诊断.     

肌萎缩侧索硬化患者神经电生理分析研究进展

<正>临床神经电生理检查在肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)的早期诊断和鉴别诊断中发挥关键作用,主要包括同心圆针肌电图(concentric needle electromyography,CNEMG)和神经传导测定,而诱发电位,单纤维肌电图,巨肌电图,重复神经刺激技术,运动单位数目估计,H反射等也有一定辅助诊断作用。本文对神经电生理在ALS中的应用及最新研究进展进行阐述。1同心圆针肌电图测定CNEMG是判定下运动神经元(lower motor neuron,LMN)损害的主要检查方法。在ALS,肌电图表现为进行     

中央前回质子磁共振波谱在肌萎缩侧索硬化患者临床病情评估中的价值

目的 以质子磁共振波谱(1H-MRS)研究肌萎缩侧索硬化(ALS)的上运动神经元病损情况,以期探寻一种评估病情和疾病进程的指标.方法 对110例ALS和24例下运动神经元综合征(LMNS)患者以及89名健康志愿者进行双侧中央前回单体素1H-MRS检查.以上运动神经元受损体征和反射评分、ALS功能评估量表(ALS-FRS)和APPEL ALS量表(AARS)定量评定患者临床症状和体征.结果 与对照组(1.62±0.18)相比,LMNS患者(1.60±0.17)的NAA/Cr无改变,但ALS患者(1.40±0.25)与两组相比均明显降低(与对照组比较,t=-5.007,P=0.000;与LMNS组比较,t=-2.660,P=0.009);在不同分级的ALS患者中,确诊ALS患者较拟诊ALS者和可能ALS者降低更为明显(与拟诊组比较,t=-2.626,P=0.010;与可能组比较,t=-2.537,P=0.013).结合ALS患者临床表现进一步分析发现,上运动神经元体征明显的患者其NAA/Cr较不明显的患者降低更为明显,差异有统计学意义(t=-2.827,P=0.006),相关分析显示,NAA/Cr与患者的反射评分、ALS-FRS、AARS及其各分项均存在显著相关性(P＜0.05).结论 ALS中央前回1H-MRS检测在一定程度上反映患者上运动神经元受损情况,可作为评估患者病情程度的一项临床指标,但其对该病早期诊断和鉴别诊断的价值有限.     

肌萎缩侧索硬化下运动神经元损伤临床评估方法进展

肌萎缩侧索硬化(amytrophic lateral sclerosis,ALS)是选择性累及上下运动神经元的神经变性疾病,下运动神经元损伤与该病的起病、病程进展和预后有密切联系,因此下运动神经元损伤评估对ALS具有重要意义。临床上除传统肌电图以外,复合肌肉动作电位、束颤电位、神经生理指数、运动单位估数和指数、单纤维肌电图、重复电刺激、阈值跟踪等电生理技术以及神经肌肉超声和磁共振检查均已成为评估下运动神经元损伤新方法,本文对上述方法的研究和应用进展进行综述,期望为临床上ALS确立诊断、评估病情、判断预后,以及科研和药物试验提供帮助。     

Epilepsy and anomalies of neuronal migration: MRI and clinical aspects

Neuronal migration disorders are the result of disturbed brain development. In such disorders, neurons are abnormally located. In diagnosing these conditions, magnetic resonance imaging is superior to any other imaging technique. This enables us to improve our knowledge of the clinical correlates of neuronal migration. With reference to migrational disorder, a retrospective study of all 303 patients with epileptic seizures referred for magnetic resonance imaging during a 3-year period was performed, 13 patients (aged 12-41, mean age 27) were identified. They represent 4.3% of the entire study group. Of the patients with known epilepsy, 6.7% and of the mentally retarded, 13.7% had migrational disorders. Four patients had schizencephaly as the dominant finding, one was classified as hemimegalencephaly, 2 had isolated heterotopias, and 6 had localized pachy- and/or poly-microgyria. The clinical pictures are complex. Ectopias of grey matter are recognised foci of epilepsy, but from an epileptological and a clinical viewpoint little attention has been given to these disorders. The present study shows that malmigration is not rare in epilepsy patients, especially not in the mentally retarded.     

Classification of methods in transcranial Electrical Stimulation (tES) and evolving strategy from historical approaches to contemporary innovations

Transcranial Electrical Stimulation (tES) encompasses all methods of non-invasive current application to the brain used in research and clinical practice. We present the first comprehensive and technical review, explaining the evolution of tES in both terminology and dosage over the past 100 years of research to present day. Current transcranial Pulsed Current Stimulation (tPCS) approaches such as Cranial Electrotherapy Stimulation (CES) descended from Electrosleep (ES) through Cranial Electro-stimulation Therapy (CET), Transcerebral Electrotherapy (TCET), and NeuroElectric Therapy (NET) while others like Transcutaneous Cranial Electrical Stimulation (TCES) descended from Electroanesthesia (EA) through Limoge, and Interferential Stimulation. Prior to a contemporary resurgence in interest, variations of transcranial Direct Current Stimulation were explored intermittently, including Polarizing current, Galvanic Vestibular Stimulation (GVS), and Transcranial Micropolarization. The development of these approaches alongside Electroconvulsive Therapy (ECT) and pharmacological developments are considered. Both the roots and unique features of contemporary approaches such as transcranial Alternating Current Stimulation (tACS) and transcranial Random Noise Stimulation (tRNS) are discussed. Trends and incremental developments in electrode montage and waveform spanning decades are presented leading to the present day. Commercial devices, seminal conferences, and regulatory decisions are noted. We conclude with six rules on how increasing medical and technological sophistication may now be leveraged for broader success and adoption of tES.     

Hepatic Considerations in the Use of Antiepileptic Drugs

Summary: Virtually all of the major antiepileptic drugs (AEDs) can cause hepatotoxicity, although fatal hepatic reactions are rare. The mechanisms, incidences, and risk profiles for such reactions differ from drug to drug. With carbamazepine and phenytoin, hepatotoxicity may be due to drug hypersensitivity. Although the profiles of patients at risk have not been well-defined for these two antiepileptic drugs, it would appear from reports in the literature that older adolescents and adults are at higher risk than children of developing serious or fatal hepatotoxicity. Once hepatotoxicity develops, mortality rates are 10–38% with phenytoin and 25% for carbamazepine. The risk profile for valproate fatal hepatotoxicity has been more clearly defined. Those at primary risk of fatal hepatic dysfunction are children under the age of 2 years who are receiving multiple anticonvulsants and also have significant medical problems in addition to severe epilepsy. The risk is considerably lower for patients over the age of 2 years on valproate monotherapy. In contrast to the risk profile with other AEDs, adults receiving valproate as monotherapy have the lowest risk of hepatotoxicity. Fatal hepatic dysfunction coincident with valproate may be the result of aberrant drug metabolism. Concomitant use of AEDs that induce microsomal P450 enzymes (e.g., phenytoin and phenobarbital) may enhance the production of a toxic metabolite, and hence the greater risk of hepatotoxicity with polypharmacy.     

Vascular Malformations and Epilepsy: Clinical Considerations and Basic Mechanisms

Summary: Vascular malformations (VMs) are associated with epilepsy. The natural history of the various VMs, clinical presentation, and tendency to provoke epilepsy determine treatment strategies. Investigations have probed the mechanisms of epileptogenesis associated with these lesions. Electrophysiologic changes are associated with epileptogenic cortex adjacent to VMs. Putative pathophysiologic mechanisms of epileptogenesis include neuronal cell loss, glial proliferation and abnormal glial physiology, altered neurotransmitter levels, free radical formation, and aberrant second messenger physiology.     

Neonatal Seizures: Problems in Diagnosis and Classification

Summary: The clinical identification of neonatal seizures is critical for the recognition of brain dysfunction; however, diagnosis is often difficult because of the poorly organized and varied nature of these behaviors. Current classification systems are limited in their ability to communicate motor, autonomic, and electroencephalo-graphic features of seizures precisely and to provide a basis for uniform effective diagnosis, therapy, and determination of prognosis. Recent investigations of neonates, utilizing bedside electroencephalographic/polygraphic/ video monitoring techniques, have provided the basis for improved diagnosis and classification of seizures in the newborn. These studies have demonstrated that not all clinical phenomena currently considered to be seizures require electrocortical epileptiform activity for their initiation or elaboration. In addition, the specific clinical character of the phenomena considered to be seizures, the clinical state of the infant, and the character of the EEG indicate the probable pathophysiological mechanisms involved and suggest probable etiologies, prognosis, and therapy. Similarities between animal models that demonstrate reflex physiology and neonates with motor automatisms and tonic posturing suggest that these clinical behaviors may not be epileptic in origin but, rather, primitive movements of progression and posture mediated by brainstem mechanisms. Although not all clinical behaviors currently considered to be neonatal seizures may have similar pathophysiological mechanisms, they are clinically significant because they all indicate brain dysfunction.     

Valproate Monotherapy in the Management of Generalized and Partial Seizures

Summary: For decades, therapeutic tradition has promoted the concept of polypharmacy in the management of epilepsy. In recent years, however, studies have shown that, for most patients, monotherapy can provide comparable or better seizure control than administration of multiple anticonvulsants, while diminishing the potential for adverse reactions, drug interactions, and poor compliance. Valproate is an important monotherapeutic agent that is highly effective in the control of idiopathic primary and secondarily generalized epilepsies, and partial seizures that do not generalize. Comparative studies have found that valproate is at least as effective as phenytoin and carbamazepine in the treatment of generalized and partial seizures. Given the similar efficacy, other factors such as pharmacokinetics and side effects may therefore determine anticonvulsant selection for monotherapy.     

Carbamazepine Efficacy and Utilization in Children

Summary: Carbamazepine is effective for preventing partial and generalized tonic-clonic seizures in children. Although absence epilepsies are more common in children than adults, an estimated 80% of children with epilepsy have seizure types or epilepsies that are potentially responsive to carbamazepine. The differential diagnosis of ictal staring is an especially important issue in children because absence and atypical absence seizures are more prevalent in children than adults. Age-related pharmacokinetic differences and drug interactions are major considerations in children. On average, children have higher clearance rates of carbamazepine, shorter half-lives, and higher ratios of carbamazepine-10, 11-epoxide to carbamazepine than adults. In addition, children with severe epilepsy are more likely to require multiple-drug therapy, which can lead to complex drug interactions. When carbamazepine is administered along with valproate, drug protein binding interactions can cause intermittent side effects.     

Un nouveau cadre conceptuel de travail pour la psychiatrie

In an attempt to place psychiatric thinking and the training of future psychiatrists more centrally into the context of modern biology, the author outlines the beginnings of a new intellectual framework for psychiatry that derives from current biological thinking about the relationship of mind to brain. The purpose of this framework is twofold. First, it is designed to emphasize that the professional requirements for future psychiatrists will demand a greater knowledge of the structure and functioning of the brain than is currently available in most training programs. Second, it is designed to illustrate that the unique domain which psychiatry occupies within academic medicine, the analysis of the interaction between social and biological determinants of behavior, can best be studied by also having a full understanding of the biological components of behavior.     

Special Pharmacokinetic Considerations in Children

Summary: Pediatric patients have greater degrees of pharmacokinetic variability and unpredictability than adults. This variability results from the effects of pharmacogenetics, age and growth, prior and current comedication, and disease. Newborns with seizures have the least predictable dosage requirements, and their needs change as drug-eliminating mechanisms mature in the neonatal period. Infants have the highest relative capacities to eliminate antiepileptics of any age group and require the largest relative doses. In addition to age-related trends, children demonstrate the same drug-specific, pharmacokinetic phenomena that adults do, including nonlinear phenytoin elimination, nonlinear valproate binding, and autoinduction of carbamazepine. Intercurrent illness and drug interactions further modify the age-related pharmacokinetic patterns in children and make dosage requirements even more unpredictable. Recent studies have shown that febrile illness can affect drug elimination, sometimes decreasing drug levels by 50% or more. Intermittent treatment with benzodiazepines administered either orally or rectally can be an important adjunct and help minimize this type of problem for children with marginally controlled epilepsy. Intermittent benzodiazepines are also helpful for children who have febrile seizures and who need only occasional antiepileptic protection.