Electrophysiological findings in immune checkpoint inhibitor-related peripheral neuropathy

ObjectiveTo report the electrodiagnostic features of immune checkpoint inhibitor (ICI)-related neuropathy.MethodsWe retrospectively reviewed clinical presentations and electrodiagnostic features of 23 patients studied after receiving immune checkpoint inhibitors (ICIs). The presentations for electrodiagnostic evaluation included an acute neuropathy or neuromuscular junction disorder. We applied established electrodiagnostic criteria for polyneuropathy and acute demyelinating neuropathy.ResultsWe identified acute demyelinating neuropathy (13 cases), axonal sensory motor neuropathy (5), pure sensory neuropathy (4) and mononeuropathy (1). 13 patients had acute demyelinating neuropathy confirmed by demonstrating demyelination in 2 or more nerves; 3 additional patients had demyelination in only one nerve. Analysis of motor nerve conduction parameters revealed demyelination involving median and ulnar nerve distal motor latencies as well as median, ulnar and peroneal nerve conduction velocities. Conduction block was found in median, ulnar and peroneal nerves. The remaining one-third patients without demyelination had acute painful axonal neuropathy. Coexisting myopathic changes (6) and neuromuscular junction dysfunction (4) were also identified.ConclusionsOur findings suggest that, while immune-mediated motor nerve demyelination is the primary underlying mechanism of ICI-related neuropathy, axonal painful neuropathy can also be an important presentation. Early recognition and effective intervention may reduce morbidity and permanent disability.SignificanceElectrophysiological studies might be useful in the evaluation of ICI-related neuropathy.     

The utility of Guillain‐Barré syndrome prognostic models in Malaysian patients

Guillain‐Barré syndrome (GBS) is an acute immune‐mediated neuropathy that has variable disease course and outcome. The Erasmus GBS outcome score (EGOS), modified EGOS (mEGOS), and Erasmus GBS respiratory insufficiency score (EGRIS) are prognostic models designed to predict the functional outcome of GBS patients at 6 months (EGOS and mEGOS) and the need for mechanical ventilation within a week of admission (EGRIS). The models were primarily developed in the Dutch GBS population, and thus the usefulness of these models in other GBS cohorts is less clear. In the current study, we aimed to validate mEGOS, EGOS, and EGRIS in Malaysian GBS patients. A total of 107 patients with GBS and its variants were consecutively recruited. Patients with GBS and Miller Fisher syndrome (MFS) were analysed separately. In the GBS cohort, high mEGOS and EGOS scores were significantly correlated with poor outcome at 6 months (mEGOS on admission: r = .381, P = .005; mEGOS at day 7 of admission: r = .507, P < .001; EGOS: r = .484, P < .001). However, there were no significant correlations between mEGOS or EGOS and outcome in patients with MFS (mEGOS on admission: r = .152, P = .523; mEGOS at day 7 of admission: r = .008, P = .973; EGOS: r = .110; P = .644). The score of EGRIS for GBS patients with mechanical ventilation was significantly higher than those patients without mechanical ventilation (4 ± 2 vs 3 ± 1; P < .001). We conclude that mEGOS and EGOS are clinically useful and relevant to the Malaysian GBS population but not in patients with classic MFS. EGRIS could be used to predict the need for mechanical ventilation in our local GBS patients.     

Increased Risk for Group B Streptococcus Sepsis in Young Infants Exposed to HIV,Soweto, South Africa, 2004–2008

Although group B Streptococcus (GBS) is a leading cause of severe invasive disease in young infants worldwide, epidemiologic data and knowledge about risk factors for the disease are lacking from low- to middle-income countries. To determine the epidemiology of invasive GBS disease among young infants in a setting with high maternal HIV infection, we conducted hospital-based surveillance during 2004–2008 in Soweto, South Africa. Overall GBS incidence was 2.72 cases/1,000 live births (1.50 and 1.22, respectively, among infants with early-onset disease [EOD] and late-onset [LOD] disease). Risk for EOD and LOD was higher for HIV-exposed than HIV-unexposed infants. GBS serotypes Ia and III accounted for 84.0% of cases, and 16.9% of infected infants died. We estimate that use of trivalent GBS vaccine (serotypes Ia, Ib, and III) could prevent 2,105 invasive GBS cases and 278 deaths annually among infants in South Africa; therefore, vaccination of all pregnant women in this country should be explored.     

丙种球蛋白联合甲泼尼松龙治疗小儿吉兰－巴雷综合征呼吸肌麻痹的临床研究

目的：研究丙种球蛋白联合甲泼尼松龙治疗小儿吉兰－巴雷综合征（GBS ）呼吸肌麻痹的临床疗效,企为临床治疗此病提供更有效的方法。方法：从在我院进行治疗的小儿GBS呼吸肌麻痹患者中,选取64例患者,并签订知情协议书。按照是否具有使用丙种球蛋白治疗的经济能力将患者分为实验组和对照组。实验组使用丙种球蛋白联合甲泼尼松龙治疗,对照组单纯使用甲泼尼松龙治疗,分别观察两组患者的休斯（H ug hes ）评分、疗效和不良反应。结果：试验组患者治疗的有效率为94％,显著高于对照组的69％（ P＝0．010）;在患者接受治疗后15 d ,试验组患者的 H ug hes评分是（2．69±1．23）分,明显低于对照组患者（3．34±0．85）分（P＝0．017）,且在患者接受治疗后6个月时,实验组患者的H ug hes评分是（1．29±0．94）分,亦明显低于对照组患者的（1．87±0．76）分（ P＝0．028）;实验组不良反应的发生率为16％,而对照组不良反应的发生率为22％（ P＝0．522）,差异不具有统计学意义。结论：与单纯使用甲泼尼松龙治疗小儿GBS呼吸肌麻痹相比,使用丙种球蛋白联合甲泼尼松龙治疗的疗效更为显著,且安全性较高,有利于患者病情的早期康复。     

吉兰-巴雷综合征复合肌肉动作电位随访观察分析

目的探讨吉兰-巴雷综合征(GBS)患者肌力变化与肌电图复合肌肉动作电位的关系。方法选取6例GBS患者,分别于入院以及发病2 m、3 m、6 m进行神经功能评分(Hughes评分)及肌电图检查,观察肌力与复合肌肉动作电位的变化特点。结果发病2 m、3 m、6 m Hughes评分结果较入院时明显好转(P<0.05);神经传导速度的好转于2 m出现(P<0.01),运动神经波幅于发病后3 m出现好转(P<0.05),至发病后6 m仍有部分神经未引出波形。结论神经功能的好转与肌电图的变化并不平行,电生理的恢复滞后于临床症状的恢复。     

围产期孕妇生殖道B族链球菌感染和耐药性及不良妊娠结局的研究

目的 通过分析围产期孕妇生殖道B族链球菌(GBS)的感染和耐药性及不良妊娠结局,为临床医师制定有效的预防和治疗措施提供依据.方法 2013年1月至2015年2月,对795例围产期孕妇生殖道分泌物进行GBS培养鉴定与药敏试验,并观察临床症状及不良妊娠结局,对结果进行统计学分析.结果 795例孕妇中共检出GBS携带者256例,带菌率为32.2％.＜30岁组(28.9％)与≥30岁组(42.3％)的带菌率差异具有统计学意义(x2=19.095,P＜0.01).GBS阳性者与GBS阴性者的临床症状发生率(18.8％ vs 8.0％)差异具有统计学意义(x 2=39.514,P＜ 0.01).10种抗菌药物(万古霉素、利奈唑胺、青霉素、氨苄西林、头孢曲松、呋喃妥因、左氧氟沙星、克林霉素、红霉素及四环素)耐药率分别为:0％、0％、0.6％、3.1％、6.6％、9.6％、21.9％、23.8％、29.9％及58.1％.D-抑菌圈试验阳性率为23.9％.GBS阳性组与GBS阴性组比较,胎膜早破、早产、宫内感染及新生儿感染发生率的差异均有统计学意义(P＜0.01).结论 该区围产期孕妇GBS带菌率较高,且高龄者易于感染;围产期孕妇感染GBS可增加不良妊娠结局的发生,应据药敏试验结果选择敏感性抗生素予以临床干预.     

Guidelines for single fiber EMG

This document is the consensus of international experts on the current status of Single Fiber EMG (SFEMG) and the measurement of neuromuscular jitter with concentric needle electrodes (CNE – CN-jitter). The panel of authors was chosen based on their particular interests and previous publications within a specific area of SFEMG or CN-jitter. Each member of the panel was asked to submit a section on their particular area of interest and these submissions were circulated among the panel members for edits and comments. This process continued until a consensus was reached. Donald Sanders and Erik Stålberg then edited the final document.     

Guillain-Barré Syndrome Surveillance during National Influenza Vaccination Campaign,New York,USA, 2009

The New York State Department of Health (NYSDOH) collected information about hospitalized patients with Guillain-Barré syndrome (GBS) during October 2009–May 2010, statewide (excluding New York City), to examine a possible relationship with influenza A(H1N1)pdm09 vaccination. NYSDOH established a Clinical Network of neurologists and 150 hospital neurology units. Hospital discharge data from the Statewide Planning and Research Cooperative System (SPARCS) were used to evaluate completeness of reporting from the Clinical Network. A total of 140 confirmed or probable GBS cases were identified: 81 (58%) from both systems, 10 (7%) from Clinical Network only, and 49 (35%) from SPARCS-only. Capture–recapture methods estimated that 6 cases might have been missed by both systems. Clinical Network median reporting time was 12 days versus 131 days for SPARCS. In public health emergencies in New York State, a Clinical Network may provide timely data, but in our study such data were less complete than traditional hospital discharge data.