危重病性肌病/危重病性多发性神经病的临床研究进展

危重病性肌病(CIM)和危重病性多发性神经病(CIP)是ICU患者的严重并发症之一,临床表现为弛缓性肢体无力和呼吸机撤离困难。CIM和/或CIP使患者机械通气时间延长、死亡率升高,并可导致长期功能障碍。其危险因素包括严重的基础疾病、多脏器功能衰竭、高血糖等。近期临床研究结果认为CIM和CIM合并CIP较CIP更多见。神经电生理检查是诊断CIM和/或CIP重要依据,直接肌肉刺激(DMS)可鉴别CIP和CIM。CIM的预后好于CIP及CIM合并CIP。CIM和/或CIP的预防和治疗方法包括减少四肢肌肉和呼吸肌制动、早期积极治疗败血症、早期肠内营养、强化胰岛素治疗和肌肉电刺激治疗等。     

糖尿病周围神经病电生理特点及与血糖水平的关系

目的研究糖尿病周围神经病的神经电生理特点以及与血糖水平的关系。方法分析2013年3月~2014年3月于本院神经内科住院的108例糖尿病周围神经病患者,测定其正中、尺、胫、腓总神经的运动传导速度(MCV)和复合肌肉动作电位波幅(CMAP),以及正中、尺、腓肠神经、腓浅神经的感觉传导速度(SCV)和感觉神经动作电位波幅(SNAP),比较上、下肢和运动、感觉神经异常情况,分析糖化血红蛋白(HbA1C)、餐后2 h血糖对神经传导速度(NCV)的影响。结果糖尿病患者下肢运动神经病变重于上肢,且差异明显(P<0.05)。感觉神经损害重于运动神经,且差异明显(P     

200例2型糖尿病患者神经传导速度检测分析

目的:探讨2型糖尿病(DM)患者周围神经病变的客观神经电生理特点。方法:分别对200例DM患者,其中有周围神经损害临床表现组(DM-I)100例和无周围神经损害临床表现组(DM-Ⅱ)100例,与50例正常成人进行运动神经传导速度(MCV)、感觉神经传导速度(SCV)、复合肌肉动作电位(CMAP)、感觉神经动作电位(SNAP)进行测定。结果:两组患者所测的MCV、SCV、CMAP、SNAP与正常对照组比较有显著差异,而DM-I组与DM-II所测的MCV、SCW、CMAP、SNAP比较亦有显著差异,下肢神经的4个参数总异常率高于上肢。结论:(1)神经传导速度的检测有助于糖尿病周围神经病的早期诊断。(2)DM并发周围神经损害在临床症状出现之前已有神经传导速度的改变。(3)下肢神经的总异常率高于上肢。     

危重病性多发性周围神经病1例报道(附文献复习)

目的:探讨危重病性多发性周围神经病(CIP)的临床特点。方法:回顾分析收治的1例CIP患者的临床表现及电生理特点,结合国外文献复习、总结CIP的特点。结果:CIP是一种急性起病,以对称性运动感觉神经轴索损害为主的CIP,常累及呼吸肌导致呼吸机脱机困难。CIP多发生于重症患者,与败血症和全身炎症反应综合征有关,其电生理特点为广泛而对称的运动感觉神经轴索损害。结论:电生理诊断是CIP诊断的重要依据,但临床上从症状学早期识别此病尤为关键。     

神经肌肉病患者电生理神经传导检测和超声下神经横截面面积的关系研究

目的探讨神经肌肉病患者电生理神经传导检测各参数和神经横截面面积(CSA)的关系。方法记录2022年3月3日至2023年5月4日就诊于北京天坛医院的21例神经肌肉病患者正中神经和尺神经不同位点的CSA, 同时进行常规神经传导检测, 分别记录每个位点附近正中神经和尺神经的运动神经传导速度(MCV)、复合肌肉动作电位(CMAP)波幅, 记录正中神经和尺神经腕部的感觉神经传导速度(SCV)及感觉神经动作电位波幅(SNAP)。分析同一位点MCV、CMAP波幅、SCV、SNAP波幅与CSA的关系。采用线性回归模型进行数据分析。结果共记录了180组同一位点的MCV和CSA, 其中正中神经102组, 尺神经78组;220组同一位点的CMAP波幅和CSA, 其中正中神经104组, 尺神经116组;60组腕部的SCV和CSA、SNAP波幅和CSA, 其中正中神经32组, 尺神经28组。正中神经和尺神经MCV与CSA的线性相关均有统计学意义(正中神经r2=0.10, 调整r2=0.09, P=0.001;尺神经r2=0.18, 调整r2=0.17, P<0.001)。CSA>10 mm2时, 正...     

慢性炎性脱髓鞘性多发神经根神经病的肌电图异常特点和诊断价值研究

目的探讨慢性炎性脱髓鞘性多发神经根神经病(CIDP)的肌电图特点和评价病情严重程度的价值。方法收集39例2018年9月至2020年12月收治的CIDP患者的临床资料。采用Hughes残疾评分评估患者病情严重程度。肌电图检查(1)4条运动神经:正中神经、尺神经、胫神经、腓神经的复合肌肉动作电位(CMAP)波幅、运动传导速度(MCV)和F波潜伏期;(2)3条感觉神经:正中神经、尺神经、腓肠神经的感觉神经传导速度(SCV)、动作电位(SNAP)波幅。根据电生理检测结果将患者分为A组(所测神经均可引出波形)和B组(≥1条神经测不出波形)。用Pearson相关性分析MCV、CMAP波幅和F波潜伏期与CIDP病情严重程度的相关性;用受试者工作特征曲线(ROC)评价MCV、CMAP及F波潜伏期对CIDP的诊断价值。结果与A组比较,B组Hughes残疾评分、脑神经受累、肢体无力、腱反射减弱、肌肉萎缩比例,MCV和CMAP波幅、F波潜伏期、SNAP波幅异常率以及F波潜伏期显著增加;而MCV、CMAP波幅均显著降低(均P0.05)。正中神经和尺神经MCV,胫神经CMAP波幅与病情严重程度呈显著负相关;正中神经、尺神经F波潜伏期与病情严重程度呈显著正相关(均P0.05)。ROC曲线分析结果显示,4条运动神经的MCV、CMAP及F波潜伏期均在CIDP的诊断中具有一定价值(均AUC0.07),3项指标联合可显著提高CIDP诊断的敏感度和特异度。结论 CIDP神经电生理表现为MCV明显减慢、CMAP波幅降低和F波潜伏期延长,神经损伤严重的CIDP患者此3项指标异常率更高,联合MCV、CMAP及F波潜伏期3项指标可提高CIDP诊断的敏感度和特异度。     

探讨神经电生理检查对腕管综合征手术治疗的指导意义

目的 探讨神经电生理检查对中、重度腕管综合征(CTS)患者手术治疗预后的疗效判定.方法 对33例中度及18例重度腕管综合征患者术前和术后3个月分别测定正中神经感觉神经传导速度( SCV)和运动神经末梢潜伏时(Mlat),示指到腕的正中神经感觉神经动作电位(SNAP),以及腕到拇对掌肌的复合肌肉动作电位(CMAP),将以上结果进行对比分析.结果 中度CTS组患者手术后SCV、M-lat、SNAP和CMAP均明显改善,差异有统计学意义(P＜0.01);而重度CTS组患者术后SCV、M-lat、SNAP和CMAP均无明显改变,差异无统计学意义(P＞0.05).结论 中度CTS组患者手术后恢复十分明显,而重度CTS组患者术后恢复较差.     

糖尿病性多发性神经病神经传导异常与临床评分相关性分析

目的分析糖尿病性多发性神经病患者神经电生理改变及其与临床评分的相关性。方法回顾性分析36例中日友好医院内分泌科住院的2型糖尿病性多发性神经病患者神经电生理异常特点并统计分析其与密歇根量表评分之间的相关性。结果本研究入组的36例糖尿病性多发性神经病患者中,周围神经传导检查下肢重于上肢(100%vs 83. 3%),感觉传导异常比运动传导异常多见(100%vs 83. 3%),以下肢感觉传导感觉神经动作电位(SNAP)波幅下降最为常见(36/36,100%);运动神经传导异常则以运动传导速度(MCV)减慢相对常见(30/36,83. 3%),复合肌肉动作电位(CMAP)波幅下降及运动传导远端潜伏期(DML)延长相对少见(11. 1%,5. 6%);神经损害电生理评分与密歇根量表评分之间具有显著相关(P 0. 01)。结论感觉传导波幅下降及轻度的运动传导速度减慢为常见的糖尿病性多发性神经病电生理改变特点;神经传导异常评分与密歇根量表评分具有显著相关,可用于疾病严重程度随访。     

糖尿病周围神经病病情分级与电生理的相关性

目的探讨糖尿病周围神经病病情分级与电生理的相关性。方法依据糖尿病性周围神经病的诊断标准确定入选对象;依据糖尿病周围神经病病情分级对入选对象进行临床分级;应用丹麦产DANTEC CANTATA型肌电图仪,进行运动神经和感觉神经传导功能检查。结果腓肠神经、正中神经诱发感觉动作电位波幅(SNAP)和腓总神经复合肌肉动作电位波幅(CMAP)随病情分级的升高而明显减低(P<0.05);腓肠神经、正中神经感觉传导速度(SCV)和腓总神经、正中神经运动传导速度(MCV)3级与1、2两级比较显著减慢(P<0.05)。结论神经电生理改变,尤其感觉神经电生理改变,易此作为糖尿病周围神经病情程度评定的指标。     

微量长春新碱误行鞘内注射致腰骶神经根损害11例报告

目的 探讨治疗中枢神经系统(CNS)白血病时微量硫酸长春新碱鞘内注射后导致神经系统损害的I临床、影像及电生理特点.方法收集11例确诊病例临床、MRI、肌电图(EMG)及神经传导等详细资料进行综合分析.结果 所有患者均有不同程度双下肢无力,除1例肌无力最轻者外均有不同程度感觉障碍及尿便障碍.行行腰骶段MRI平扫结果8例均未见异常.其中2例行增强扫描亦未见异常.电生理检查结果双下肢异常10例:EMG表现为神经源性损害;运动神经传导部分神经未引出波形,能引出者复合肌肉动作电位(CMAP)波幅均明显降低(0.1～2.7 mV),2例胫神经运动传导速度(MCV)轻度减慢;感觉传导速度(SCV)及感觉神经动作电位(SNAP)均正常.结论 从电生理检查结果分析此组腰骶神经根损伤可能主要为轴索损害.患者病情相对较轻,病变部位相对局限于邻近给药部位,可能与注入长春新碱量较少有关.     

Critical illness polyneuropathy and myopathy: a major cause of muscle weakness and paralysis

Critical illness polyneuropathy (CIP) and myopathy (CIM) are complications of critical illness that present with muscle weakness and failure to wean from the ventilator. In addition to prolonging mechanical ventilation and hospitalisation, CIP and CIM increase hospital mortality in patients who are critically ill and cause chronic disability in survivors of critical illness. Structural changes associated with CIP and CIM include axonal nerve degeneration, muscle myosin loss, and muscle necrosis. Functional changes can cause electrical inexcitability of nerves and muscles with reversible muscle weakness. Microvascular changes and cytopathic hypoxia might disrupt energy supply and use. An acquired sodium channelopathy causing reduced muscle membrane and nerve excitability is a possible unifying mechanism underlying CIP and CIM. The diagnosis of CIP, CIM, or combined CIP and CIM relies on clinical, electrophysiological, and muscle biopsy investigations. Control of hyperglycaemia might reduce the severity of these complications of critical illness, and early rehabilitation in the intensive care unit might improve the functional recovery and independence of patients.     

Approach to critical illness myopathy and polyneuropathy in the older SARS-CoV-2 patients

One of the major concerns of the health care community and the public surrounding the SARS-CoV-2 pandemic is the availability and use of ventilators. Unprecedented surges of patients presented to intensive care units across the country, with older adults making up a large proportion of the patient population. This paper illustrates contemporary approaches to critical illness myopathy (CIM), critical illness polyneuropathy (CIP), and critical illness polyneuromyopathy (CIPNM) in older patients, including incidence, risk factors, mechanisms for pathology, diagnosis, contemporary treatment approaches, and outcomes. We hope that the following analysis may help educate clinicians and ultimately decrease the duration of the mechanical ventilation required by these patients, resulting in improved clinical outcomes and an increase in ventilator availability for other patients in need.     

Risk factors for critical illness polyneuromyopathy

Although numerous clinical, laboratory, and pharmacological variables have been reported as significant risk factors for critical illness polyneuromyopathy (CIPM), there is still no consensus on the aetiology of this condition.Objectives of the study were to assess the clinical and electrophysiological incidence and risk factors for CIPM.A cohort of critically ill patients was observed prospectively for a one-month period and the association between neuromuscular involvement and various potential risk factors was evaluated. Sixty one critically ill patients completed the follow-up (30 women, 31 men, median age 59 years).CIPM development was detected clinically in 17 patients (27.9 %) and electrophysiologically in 35 patients (57.4 %). CIPM was significantly associated with the presence and duration of systemic inflammatory response syndrome and the severity of multiple, respiratory, central nervous, and cardiovascular organ failures. The median duration of mechanical ventilation was significantly longer in patients with CIPM than in those without (16 vs 3 days, p<0.001). Independent predictors of CIPM obtainable within the 1(st) week of critical illness were the admission sequential organ failure assessment score (odds ratio [OR], 1.15; 95% confidence interval [CI], 1.02-1.36), the 1(st) week total sequential organ failure assessment scores (OR, 1.14; 95 % CI, 1.06-1.46) and the 1(st) week duration of systemic inflammatory response syndrome (OR, 1.05; 95% CI, 1.01-1.15). They were able to correctly predict the development of CIPM at the end of the 1(st) week in about 80% of critically ill cases.In conclusion, the presence and duration of systemic inflammatory response syndrome and the severity of multiple and several organ failures are associated with increased risk of the development of CIPM.     

Prolonged compound muscle action potential duration in critical illness myopathy

Critical illness myopathy (CIM) is a frequent cause of generalized weakness in the intensive care unit. Prolonged compound muscle action potential (CMAP) durations have been described in this patient population, and this study presents further data on CMAP duration in normal controls and patients with CIM. The findings highlight the importance of testing multiple nerve muscle combinations in weak, critically ill patients. Recognition of this pattern, which has not been widely described, can facilitate the diagnosis of CIM. Muscle Nerve, 2009     

Neuromuscular disorders in critically ill patients: review and update

Neuromuscular disorders that are diagnosed in the intensive care unit (ICU) usually cause substantial limb weakness and contribute to ventilatory dysfunction. Although some lead to ICU admission, ICU-acquired disorders, mainly critical illness myopathy (CIM) and critical illness polyneuropathy (CIP), are more frequent and are associated with considerable morbidity. Approximately 25% to 45% of patients admitted to the ICU develop CIM, CIP, or both. Their clinical features often overlap; therefore, nerve conduction studies and electromyography are particularly helpful diagnostically, and more sophisticated electrodiagnostic studies and histopathologic evaluation are required in some circumstances. A number of prospective studies have identified risk factors for CIP and CIM, but their limitations often include the inability to separate CIM from CIP. Animal models reveal evidence of a channelopathy in both CIM and CIP, and human studies also identified axonal degeneration in CIP and myosin loss in CIM. Outcomes are variable. They tend to be better with CIM, and some patients have longstanding disabilities. Future studies of well-characterized patients with CIP and CIM should refine our understanding of risk factors, outcomes, and pathogenic mechanisms, leading to better interventions.     

Age related axonal neuropathy in spinocerebellar ataxia type 3/Machado-Joseph disease (SCA3/MJD)

To identify determinants of peripheral involvement in spinocerebellar ataxia type 3/Machado-Joseph disease (SCA3/MJD) the influence of CAG repeat length, age of onset, disease duration and age on the results of nerve conduction studies was analysed in 58 patients with SCA3/MJD. Patients with SCA3/MJD showed marked reduction of compound muscle action potential (CMAP) and sensory nerve action potential (SNAP) amplitudes indicating axonal neuropathy of both motor and sensory fibres. In addition, there was moderate slowing of nerve conduction suggestive of mild peripheral demyelination. Multivariate regression showed that CMAP and SNAP amplitudes decreased with age, but were not affected by CAG repeat length, age of onset, or disease duration. The age related decline of CMAP and SNAP amplitudes in SCA3/MJD was greater than in normal subjects. The data suggest that the degree of peripheral damage in SCA3/MJD does not depend on CAG repeat length, age of onset, or disease duration, but is mainly related to the time period over which the SCA3/MJD mutation exerts its effect.     

Critical illness polyneuropathy and myopathy]

Critical Illness Polyneuropathy (CIP) and Myopathy (CIM), either singly or in combination, are a common complication of critical illness. Both disorders may lead to severe weakness and require mechanical ventilation. CIP, as initially described by Bolton et al., in 1984, is a sensorimotor polyneuropathy that is often a complication of sepsis and multiorgan failure. In Japan, Horinouchi et al., first reported a case in 1994. CIM has been referred to by a number of different terms (acute quadriplegic myopathy, thick filament myopathy, acute necrotizing myopathy of intensive care, rapidly evolving myopathy with myosin-deficiency fibers) in the literature. A variety of serious problems (e.g., pneumonia, severe asthma, and lung or liver transplantation) and the concomitant use of high-dose intravenous corticosteroids and nondepolarizing neuromuscular blocking agents predispose to CIM. In Japan, Kawada et al., reported a first case as acute quadriplegic myopathy in 2000. There is no specific treatment for CIP and CIM. Minimizing the use of corticosteroids and nondepolarizing neuromuscular blocking agents in a critical illness setting may prove helpful in preventing the occurrence of these disorders. The prognosis is directly related to the age of the patient and the seriousness of the underlying illness.     

What is the best diagnostic index of conduction block and temporal dispersion?

In order to find the best diagnostic index of conduction block and abnormal temporal dispersion, the amplitude, duration, and area of the compound muscle action potentials (CMAP) were studied in 40 normal controls and 28 patients with acquired demyelinating neuropathies. In the normal subjects, there was a substantial difference among the various nerves in the degree of CMAP amplitude reduction and CMAP duration prolongation with proximal stimulation, and thus different criteria should be used for conduction block or abnormal temporal dispersion for a given nerve. In 28 patients with demyelinating neuropathy, 58 of 207 (28%) tested nerve segments showed nerve conduction velocity (NCV) evidence of demyelination. To identify “demyelination” in these segments, conduction block was best detected by the total area method in 71% of cases, and abnormal temporal dispersion was bast by the negative-peak duration method. This study showed that the best diagnostic index for conduction block is the total area method and for abnormal temporal dispersion, the negative-peak duration method. © 1994 John & Sons, Inc.     

Neuromuscular manifestations of critical illness

Critical illness, more precisely defined as the systemic inflammatory response syndrome (SIRS), occurs in 20%-50% of patients who have been on mechanical ventilation for more than 1 week in an intensive care unit. Critical illness polyneuropathy (CIP) and myopathy (CIM), singly or in combination, occur commonly in these patients and present as limb weakness and difficulty in weaning from the ventilator. Critical illness myopathy can be subdivided into thick-filament (myosin) loss, cachectic myopathy, acute rhabdomyolysis, and acute necrotizing myopathy of intensive care. SIRS is the predominant underlying factor in CIP and is likely a factor in CIM even though the effects of neuromuscular blocking agents and steroids predominate in CIM. Identification and characterization of the polyneuropathy and myopathy depend upon neurological examination, electrophysiological studies, measurement of serum creatine kinase, and, if features suggest a myopathy, muscle biopsy. The information is valuable in deciding treatment and prognosis.     

Long-term neuromuscular sequelae of critical illness

In this observational study, we analyzed the long-term neuromuscular deficits of survivors of critical illness. Intensive care unit-acquired muscular weakness (ICU-AW) is a very common complication of critical illness. Critical illness polyneuropathy (CIP) and critical illness myopathy (CIM) are two main contributors to ICU-AW. ICU-AW is associated with an increased mortality and leads to rehabilitation problems. However, the long-term outcome of ICU-AW and factors influencing it are not well known. We analyzed the medical records of 490 survivors of critical illness, aged 18–75 years and located in the area of the study center. Intensive care unit (ICU) survivors with comorbidities that might influence neuromuscular follow-up examinations, muscle strength, or results of nerve conduction studies, such as renal or hepatic insufficiency, diabetes mellitus, or vitamin deficiency were excluded. A total of 51 patients were finally included in the study. Six to 24 months after discharge from the ICU, we measured the Medical Research Council (MRC) sum score, the Overall Disability Sum score (ODSS), and also performed nerve conduction studies and EMG. For all ICU survivors, the median MRC sum score was 60 (range 47–60) and the median ODSS score was 0 (range 0–8). CIP was diagnosed in 21 patients (41 %). No patient was diagnosed with CIM. Patients with diagnosis of CIP showed a higher median ODSS scores 1 (range 0–8) versus 0 (range 0–5); p < 0.001 and lower median MRC sum scores 56 (range 47–60) versus 60 (range 58–60); p < 0.001. The three main outcome variables MRC sum score, ODSS score and diagnosis of CIP were not related to age, gender, or diagnosis of sepsis. The MRC sum score (r = ?0.33; p = 0.02) and the ODSS score (r = 0.31; p = 0.029) were correlated with the APACHE score. There was a trend for an increased APACHE score in patients with diagnosis of CIP 19 (range 6–33) versus 16.5 (range 6–28); p = 0.065. Patients with the diagnosis of CIP had more days of ICU treatment 11 days (range 2–74) versus 4 days (range 1–61); p = 0.015, and had more days of ventilator support 8 days (range 1–59) versus 2 days (range 1–46); p = 0.006. The MRC sum score and the ODSS score were correlated with the days of ICU treatment and with the days of ventilator support. The neuromuscular long-term consequences of critical illness were not severe in our study population. As patients with concomitant diseases and old patients were excluded from this study the result of an overall favorable prognosis of ICU-acquired weakness may not be true for other patient’s case-mix. Risk factors for the development of long-term critical illness neuropathy are duration of ICU treatment, duration of ventilator support, and a high APACHE score, but not diagnosis of sepsis. Although ICU-AW can be serious complication of ICU treatment, this should not influence therapeutic decisions, given its favorable long-term prognosis, at least in relatively young patients with no concomitant diseases.