High ultrasound variability in chronic immune-mediated neuropathies. Review of the literature and personal observations

Chronic immune-mediated neuropathies show high clinical variability. Diagnosis is based on clinical and neurophysiological studies, but recently ultrasound (US) of peripheral nerves has been shown to provide useful morphological information. US has already been shown to crucially influence diagnosis and clinical care in entrapment neuropathies, in traumatic nerve lesions and in tumors. The role of US in the evaluation of polyneuropathies is still not clearly defined, but increasing attention has recently been focused on the immune-mediated neuropathies and specific US measures (namely the intra- and inter-nerve cross-sectional area variability) have been developed. The aim of the current paper is to make a review of the available nerve US studies and provide data from personal observations in the most common chronic immune-mediated neuropathies.     

Nerve ultrasound in a case of chronic inflammatory demyelinating neuropathy

Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common acquired immune‐mediated inflammatory disorder of the peripheral nervous system. The diagnosis is based mainly on the clinical presentation and electrophysiological detection of demyelination. Methods: Several MRI studies have demonstrated hypertrophy and abnormal enhancement of spinal nerve roots or brachial plexus in CIDP, but there have been only anecdotal reports of similar sonographic findings. Results: This article reports the sonographic findings of a CIDP case and includes a review of the literature and previously reported cases. Conclusions: This case report highlights the importance of sonography in the localization and recognition of focal nerve enlargements in patients with CIDP. This method could be a helpful tool in the diagnosis of conduction block in CIDP, especially in cases where a nerve segment cannot be explored easily with the inching technique. Systematic data are needed to confirm this observation. Muscle Nerve 47:443‐446, 2013     

Prevalence of neuromuscular disorders in Qena governorate/Egypt: population-based survey

Background: Few epidemiological studies of the prevalence of neuromuscular disorders have been undertaken. The aim of the study was to estimate the prevalence of the most common types of neuromuscular disorders in Qena governorate/Egypt.

Methods: A random sample was taken from 11 districts, involving 9303 inhabitants with 57.3% urban residents and 42.7% rural residence. Patients were diagnosed using a screening questionnaire for the diagnosis of neuromuscular disorders. All positive cases were referred to Qena University hospital where they underwent full clinical, electrophysiological, and laboratory investigations.

Results: Out of 9303 participants 448 cases were identified positive during survey. Four hundred and twenty-six cases proved to have neuromuscular disorders giving a crude prevalence rate (CPR) of 4.57%; 408 cases had definite neuropathy and 18 cases had muscular disorders equivalent to CPR of 4.39% and 193/10⁵ respectively. There was a higher prevalence in the rural than urban population. The CPR of focal compression neuropathies was 1.8%, with the majority of cases having carpal tunnel syndrome (CPR = 1.67%). CPR of diabetic neuropathy was 1.67%. The CPR of compressive radiculopathy was 0.34%. Traumatic nerve injury had a CPR 0.06%. The lifetime prevalence of Bell’s palsy was 0.16%. Hereditary motor and sensory neuropathy had a CPR 0.08%. The CPR of idiopathic neuropathy was 0.09% and Infective Leprotic neuropathy was 0.04%. Five patients were diagnosed as having muscular dystrophy and another 5 patients had myotonia with CPR of 54/10⁵ for each. Two cases of myasthenia gravis and another two cases with systemic myopathy were recorded giving a CPR of 21/10⁵.

Conclusion: The overall CPR of neuromuscular disorders in the general population in Qena governorate/Egypt was higher than reported in other countries.     

1H-MR波谱检测腓肠肌细胞内脂质浓度用于早期诊断2型糖尿病模型大鼠周围神经病变

目的 观察¹H-MR波谱(MRS)检测腓肠肌肌细胞内脂质(IMCL)浓度对早期诊断2型糖尿病(T2DM)模型大鼠周围神经病变(DPN)的价值。方法 将30只雄性SD大鼠随机分为糖尿病(DM)组和正常组,每组15只。对DM组以高糖高脂饲养4周联合单次腹腔注射1%链脲佐菌素(STZ)溶液45 mg/kg体质量建立T2DM模型,正常组则以普通饲料饲养4周联合注射1% STZ柠檬酸-柠檬酸钠缓冲液作为对照。于建模成功后第4、8周采集2组大鼠右后肢腓肠肌¹H-MRS,检测其IMCL浓度;于建模成功后第8周处死动物,检测2组右坐骨神经运动神经传导速度(MNCV)和感觉神经传导速度(SNCV),之后行病理学检查,观察坐骨神经组织变化,判断是否发生DPN;评估腓肠肌IMCL浓度对早期诊断DPN的价值。结果 DM组15只均成功建立T2DM大鼠模型,建模成功后每周DM组大鼠空腹血糖及体质量均高于正常组(P均＜0.05)。建模成功后第4、8周,DM组大鼠右后肢腓肠肌IMCL浓度均显著高于正常组(P均＜0.01),且建模成功后第8周DM组及正常组大鼠IMCL浓度均明显高于第4周(P均＜0.01)。建模成功后第8周,DM组大鼠均发生DPN,其右坐骨神经MNCV及SNCV均明显低于正常组(P 均＜0.001);病理结果示神经纤维异常变化。结论 ¹H-MRS检测腓肠肌IMCL对早期诊断T2DM大鼠DPN具有一定价值。     

亚临床甲状腺功能减退症对糖尿病心脏自主神经病变的影响研究

背景糖尿病心脏自主神经病变（DCAN）是糖尿病常见且严重的慢性并发症之一,但目前关于甲状腺功能与DCAN关系的研究报道较少。目的探讨亚临床甲状腺功能减退症（SCH）对DCAN的影响。方法选取2019年10月至2020年10月在首都医科大学附属北京友谊医院住院及门诊就诊的2型糖尿病患者564例。所有患者均完成Ewing试验。收集患者一般资料、体格检查结果、实验室检查结果,并依据Ewing试验评估并筛查DCAN。相关性分析采用Pearson相关分析和Spearman秩相关分析,采用多因素Logistic逐步回归分析探究2型糖尿病患者并发DCAN的影响因素。结果564例2型糖尿病患者中,129例并发DCAN（DCAN组）,435例未并发DCAN（对照组）,DCAN发生率为22.9%。2型糖尿病患者中合并SCH者84例（14.9%）,DCAN组患者中合并SCH者36例（28.0%）,对照组患者中合并SCH者48例（11.0%）,DCAN组患者合并SCH患病率高于对照组（χ2=22.346,P<0.001）。2型糖尿病并发DCAN患者Valsalva试验动作期间最大R-R间距与最小R-R间距比值（VAL R-R比值）与糖尿病病程、糖化血红蛋白（HbA1c）、空腹血糖（FBG）、低密度脂蛋白胆固醇（LDL-C）、三酰甘油（TC）、超敏C反应蛋白（hs-CRP）、促甲状腺激素（TSH）、尿微量白蛋白/肌酐（UACR）呈负相关,与舒张压（DBP）呈正相关（P<0.05）。多因素Logistic逐步回归分析结果显示,SCH〔OR=1.717,95%CI（1.246,2.365）〕是2型糖尿病患者并发DCAN的独立影响因素（P<0.05）。结论2型糖尿病患者TSH水平与DCAN相关,且SCH是2型糖尿病患者并发DCAN的独立影响因素。     

聚乙二醇对盐酸伊立替康脂质体体外释放及在不同稀释介质中稳定性的影响

目的:考察不同聚乙二醇含量对盐酸伊立替康脂质体体外释放特性及在不同稀释介质中稳定性的影响。方法:采用乙二胺四乙酸铵梯度法,以聚乙二醇2000含量分别为0、8、14、20、26 mg/ml的聚乙二醇2000-二硬脂酰磷脂酰乙醇胺(m PEG2000-DSPE)制备盐酸伊立替康脂质体,测定其包封率和粒径,评价其体外24 h内的累积释放度和在生理氯化钠溶液与5%葡萄糖注射液中的稳定性。结果:随着m PEG2000-DSPE含量的增加,盐酸伊立替康脂质体体外24 h累积释放度逐渐减小;以生理氯化钠溶液及5%葡萄糖注射液稀释后,随着m PEG2000-DSPE含量的增加,盐酸伊立替康脂质体的包封率和粒径变化均减小;当聚乙二醇2000质量浓度增加至20、26 mg/ml时,脂质体包封率和粒径基本不再变化。结论:m PEG2000-DSPE的加入可减慢盐酸伊立替康脂质体的体外释放,提高其在不同稀释介质中的稳定性。     

高频超声评价2型糖尿病患者下肢周围神经病变临床观察

目的探讨高频超声用于评价2型糖尿病合并周围神经病变患者下肢股神经和隐神经异常改变的临床价值。方法共81例2型糖尿病患者分为无周围神经病变组(40例)和合并周围神经病变组(41例),通过彩色多普勒超声观察股神经和隐神经内部回声及连续性,测量并比较神经宽径、厚径和横截面积。结果正常对照组受试者股神经和隐神经横切面扫描呈略高回声,内部呈细小筛网格状,纵切面扫描内部呈条状低回声和平行线样排列的条状略高回声;无周围神经病变组患者内部回声略减低,细小筛网格状结构模糊,纵切面条状低回声结构欠清晰;合并周围神经病变组患者内部回声明显减低,细小筛网格状结构显示不清,纵切面条状低回声结构模糊。与正常对照组相比,糖尿病组患者股神经宽径(均P=0.000)、厚径(P=0.023,0.036)和横截面积(均P=0.000)增大;隐神经宽径(P=0.010,0.014)、厚径(均P=0.001)和横截面积(P=0.002,0.004)增大;合并周围神经病变组患者股神经和隐神经厚径(P=0.048,0.012)和横截面积(P=0.031,0.034)较无周围神经病变组增大。结论高频超声可以清晰地显示2型糖尿病合并周围神经病变患者股神经和隐神经主干内部回声及各径线的异常改变,为临床诊断提供客观依据,具有重要临床意义。     

Multifocal motor neuropathy with high titers of anti‐MAG antibodies

Multifocal motor neuropathy (MMN) and anti‐myelin‐associated glycoprotein (anti‐MAG)‐associated neuropathy are clinically and electrophysiologically distinct entities. We describe a patient with characteristic features of both neuropathies, raising the possibility of an overlap syndrome. A 49‐year‐old patient reported a history of slowly progressive predominantly distal tetraparesis, with mild sensory deficits. Nerve conduction studies demonstrated persistent motor conduction blocks outside compression sites, typical of MMN. Laboratory findings revealed persistently high titers of anti‐MAG immunoglobulin Mλ (IgMλ) paraprotein in the context of a monoclonal gammapathy of unknown significance. Skin biopsy of distal lower limb revealed IgM positive terminal nerve perineurium deposits. This case suggests that the distinction between subtypes of chronic inflammatory neuropathies may not be as clear as initially thought, and that the pattern of pathogenicity of anti‐MAG antibodies may vary.