振动感觉阈值(VPT)在糖尿病周围神经病变(DPN)中的诊断价值

 目的 探讨以振动感觉阈值(vibrating perception threshold,VPT)检查为主联合糖尿病神经病变症状评分(diabetic neuropathy symptom,DNS)、足外观、温度觉、痛觉及触觉5种简易检查方法在糖尿病周围神经病变(diabetic peripheral neuropathy,DPN)中的诊断价值。方法 217例2型糖尿病患者记录DNS,同时行足外观检查、VPT、温度觉、痛觉、半定量音叉、10g尼龙丝检查和肌电图神经传导速度(nerve conducting velocity,NCV)测定。按神经电生理检查结果,分为不合并周围神经病变组(n=130),合并周围神经病变组 (n=87),比较两组的基本情况和代谢指标。计算5种联合检查方法的敏感度、特异度、阳性预测值、阴性预测值、准确度、Youden指数以及 Kappa值(κ值),与NCV检查进行相关性和一致性分析。结果 两组间年龄、糖尿病病程、VPT值有显著差异,DPN组明显高于无DPN组(P<0.05)。VPT与正中运动神经传导速度(motor conducting velocity,MCV)、尺神经MCV、腓总神经MCV、腓浅神经感觉性传导速度(sensory conducting velocity,SCV)、胫神经MCV之间均呈负相关(P<0.05)。5种联合检查方法与NCV检查的秩相关分析均呈显著正相关(P<0.01),VPT联合DNS检查与NCV的相关性最好(r=0.799)。VPT联合DNS检查的敏感度、特异度、准确度分别为74.4%、100%、89.9%,与NCV检查高度一致(κ值为0.780)。结论 VPT联合DNS是准确筛查和诊断DPN的适用方法。     

Charcot–marie–tooth disease with intermediate conduction velocities caused by a novel mutation in the MPZ gene

Charcot–Marie–Tooth (CMT) disease is a heterogeneous group of inherited sensory and motor neuropathies. Mutations in the gene that encodes for myelin protein zero (MPZ) can produce different phenotypes: CMT1 (with low conduction velocities), CMT2 (less frequent and with unaffected conduction velocities), and CMTID (with intermediate conduction velocities). We report a study of seven patients from a four‐generation family. All the affected members of the family had a typical CMT phenotype, but three of them had calf hypertrophy. The nerve conduction velocities (NCV) in all of them were between 35 and 43 m/s. Molecular study revealed the novel mutation Lys214Met in the MPZ gene. Molecular study of the MPZ gene would be useful in cases of CMT in families with intermediate NCV, especially if no mutations in the GJB‐1 gene are found or there is male‐to‐male transmission. Muscle Nerve, 2010     

Maladie de Charcot-Marie-Tooth associée au gène de la périaxine (CMT4F) : description clinique, électrophysiologique et génétique de 24 patients

Autosomal recessive Charcot-Marie-Tooth disease (AR-CMT) is often characterized by onset in early childhood and severe phenotype compared to the dominant forms. CMT disease associated with periaxin gene (PRX) is rare and characterized by demyelination limited to the major peripheral nerves. Following the discovery of a high frequency of a specific periaxin gene mutation (E1085fsX4 homozygote) in the Reunion Island, we examined all French patients known as carriers of the periaxin gene mutation. There were 24 patients. Eighteen were from the Reunion Island (6 families and 10 sporadic cases). The six remaining patients were in two families, each with two affected individuals, and two sporadic cases. The series included 17 female and seven male patients. Walking was acquired late, on average at 3.4 ± 1.6 years. One patient never learned to walk. The Charcot Marie Tooth Neuropathy Score (CMTNS) averaged 24.5 ± 8.1. Seven patients had been wheelchair-bound since the age of 24 ± 22. Other symptoms were: scoliosis most often observed after the age of 12 years and sometimes complicated by a restrictive respiratory syndrome; foot deformity in 24 patients; strabismus; glaucoma; myopia. When conduction recordings are available, median nerve motor conduction was slow (< 10 m/s), associated with a major lengthening of distal latencies. Study of the periaxin gene should be considered in patients with severe demyelinating neuropathy associated with early infantile scoliosis. This disease leads to major disability (29% of patients in this series were wheelchair-bound) and to respiratory insufficiency. Genetic counselling is highly recommended for consanguineous families.     

消渴清颗粒治疗糖尿病周围神经病变疗效分析

目的：观察消渴清颗粒治疗糖尿病周围神经病变（DPN）的临床疗效。方法：将40例DPN患者随机分为消渴清颗粒治疗组（21例）和对照组（19例）,在常规治疗的基础上治疗组加用消渴清颗粒1袋/次（6 g）,3次/d;对照组用甲钴胺胶囊0.5 mg/次,3次/d,2组连续治疗3个月后观察神经传导速度（NCV）及临床症状变化。结果：治疗组在治疗后疼痛、麻木及感觉异常总有效率为85.71%,对照组为63.16%,2组比较差异有统计学意义（P〈0.05）;治疗组神经传导速度与治疗前比较明显提高（P〈0.01）。结论：消渴清颗粒是治疗糖尿病周围神经病变的有效药物。     

人神经生长因子治疗糖尿病性多发性神经病的疗效和安全性

目的:考察人神经生长因子(hNGF)对糖尿病性多发性神经病(DPN)的疗效和安全性.方法:采用随机双盲对照研究,60例DPN患者分hNGF治疗组30例,给予hNGF 4mL·d-1(1 000U)肌内注射,对照组30例给予安慰剂4mL肌内注射,2组疗程均为12周.主要观察指标为神经体征(密西根糖尿病神经评分,MDNS),辅助观察指标为神经症状(自觉感觉异常)和神经传导速度(NCV).结果:治疗84d后2组中度和重度患者的MDNS评分显效率比较差异均有显著性(P<0.05),治疗后84d和168d,治疗组NCV异常率均显著低于对照组(44.00% vs 72.67%,P<0.01;39.23% vs 62.86%,P<0.01).治疗组疗效确切,未发现严重不良反应.结论:hNGF治疗DPN安全有效,其疗效和NCV的改善与hNGF治疗开始时间、疗程长短、DPN病变的程度密切相关.     

Encephalitis associated with motor polyneuropathy

We describe three patients with acute encephalitis and motor-dominant polyneuropathy. All three patients were in their middle age and presented with features of encephalopathy with flu like illness. Cerebrospinal fluid (CSF) studies showed lymphocytic pleocytosis with increased protein. Brain magnetic resonance imagings (MRIs) showed symmetric high signal intensities in bilateral mesial temporal lobes and bilateral thalami in all patients. All three patients became quadriplegic and areflexic within two hospital days (HDs) and electrophysiologic studies revealed motor polyneuropathy. Viral or atypical bacterial infection was suspected, but no causative agent could be found. One patient expired on the twentieth hospital day, and the other two patients showed no or little improvement. To our knowledge, this combination of acute encephalitis with motor polyneuropathy has not been reported.     

皮肤交感反应在小纤维神经病测定中的应用

目的：观察和分析皮肤交感反应（SSR）在小纤维神经病测定中的应用效果。方法：选取我院2013年7月～2014年10月接受神经传导速度（NCV ）检测正常的患者30例（观察组）,均存在小纤维损害症状,并选择同时期45例健康人员（对照组）进行比较,刺激强度为35mA,脉宽为0．2ms,刺激右侧正中神经,并详细记录波形变化、潜伏期、波幅等情况,对其SSR进行测定。结果：观察组患者SSR引出率为97％;足部潜伏期观察组患者与对照组比较差异有统计学意义（ P＜0．05）,两组受检者手部SSR指标无明显差异（P＞0．05）。结论：NCV正常患者的足部SSR存在长度依赖性损害表现,可能会患有交感节后C类小纤维损害,SSR为临床疾病的预防与诊断提供科学的参考资料。     

前列腺素E1在动态负荷下对大鼠受压马尾神经电生理的影响

目的：研究动态因素下前列腺索E1对大鼠马尾神经电生理变化的影响。方法：分4组，每组6只。除正常对照组，其他三个实验组使用硅胶片插入L4及L6椎管造成横截面积减小30％的双节段狭窄。压迫施加2h后，三个实验组分别施加动态负荷(高频率刺激和/或压迫追加)6min。PGE1压迫增加组在施加动态负荷前给与前列腺素E1。结果：压迫2h后，各实验组神经传导速度均显著下降。动态负荷下，HFS褐压迫增加组出现了神经传导阻滞，而应用了前列腺累E1的PGE4压迫增加组则部分避免了其发生。结论：受压状态中的马尾神经在动态负荷下，前列腺索E1可以防止神经传导阻滞的发生，对神经传导功有保护作用，它可以作为一种非手术治疗腰椎管狭窄症的选择。     

活血通络法治疗糖尿病周围神经病变的临床研究

目的评价活血通络法对糖尿病周围神经病变(DPN)的疗效.方法将60例DPN患者随机分为2组,在基础治疗相同的情况下,治疗组30例采用水蛭注射液4 mL,溶于0.9%氯化钠溶液250 mL静脉滴注,同时采用熏洗舒痹汤熏洗患处;对照组30例采用弥可保肌肉注射,每次500μg.观察治疗前后DPN患者的症状、体征的改善情况及神经传导速度的变化.结果治疗组显效率为56.67%,对照组为16.67%(P＜0.01).治疗组总有效率为86.67%,对照组为56.67%(P＜0.05).对神经传导速度改善,治疗组明显优于对照组(P＜0.01).结论活血通络法能够减轻DPN患者的临床症状,改善病变神经传导速度,对防治DPN有较好的疗效.