Diagnostic sensitivity of electrophysiology and ultrasonography in ulnar neuropathies of different severity

ObjectiveTo assess the diagnostic performance of electrophysiology and nerve ultrasound in ulnar neuropathies of varying clinical severity in 135 consecutive patients.MethodsClinical severity of ulnar neuropathy was graded on a 4 point scale from very mild (symptoms only) to severe (marked atrophy of intrinsic hand muscles). Sensitivity and localization ability of electrophysiology and nerve ultrasound were assessed for each point of the scale.ResultsUltrasound had higher sensitivity than electrophysiology in clinically very mild (20% and 3% for ultrasound and electrophysiology, respectively) and mild (62% and 47% for ultrasound and electrophysiology, respectively) neuropathies, had greater localizing ability in axonal ulnar neuropathies, and identified nerve hypermobility.Ultrasound nerve cross-sectional area had strong positive correlation with both clinical and electrophysiological severity scores, but with significant overlap across the severity groups.ConclusionThe diagnostic work-up of ulnar neuropathies was improved by using both electrophysiology and ultrasound at all levels of clinical severity. Ultrasound increased the diagnostic yield in very mild and mild neuropathies, localized all the ulnar neuropathies with abnormal non-localizing electrophysiology and identified nerve hypermobility.SignificanceThis is the first detailed analysis of the diagnostic performance of electrophysiology and ultrasound in ulnar neuropathies of varying severity.     

高压氧综合治疗糖尿病性胃轻瘫患者的疗效观察

目的观察高压氧（HBO）联合吗丁啉综合治疗对糖尿病性胃轻瘫患者胃动素及胃排空时间的影响。方法采用随机数字表法将2000年10月至2004年10月间连续人院的糖尿病性胃轻瘫患者112例随机分为高压氧组和对照组各46例，HBO组在给予吗丁啉（每次10mg，每天3次，连用4周）的基础上加用HBO治疗，HBO采用多人氧舱，在舱内停留120min，压力为0．25MPa（2．5ATA），戴面罩吸人纯氧60min（30min各2次，中间休息10min吸人舱内空气），10次为1个疗程，共3个疗程，每疗程间隔3～5d（排除HBO治疗禁忌证）。对照组为单纯吗丁啉，每次10mg，每天3次，连用4周。45d分别对两组患者治疗前后胃动素、空腹血糖、胃排空时间及症状的改善各项指标进行研究对比。结果HBO组和对照组总有效率分别为92．9％和76．8％，症状的改善、胃动素及胃排空时间治疗组均优于对照组，差异有显著性（P〈0．05）。结论糖尿病性胃轻瘫患者在服用吗丁啉的基础上应用HBO可降低胃动素、加速胃排空时间，对临床症状的改善具有积极作用。     

缬沙坦治疗糖尿病肾病的疗效观察

目的:观察血管紧张素Ⅱ,Ⅰ型受体拮抗剂(AT1Ra)缬沙坦(Valsartan)对伴大量白蛋白尿的临床糖尿病肾病的治疗作用。方法:32例2型糖尿病患者,24h尿白蛋白排泄率(24hUAER)>200μg/min,均伴高血压,维持原糖尿病治疗不变,分组比较应用缬沙坦(80mg/d)或贝那普利(10mg/d)治疗8周前后平均动脉压(MAP)、24hUAER、HbA1c、尿酸(UA)等指标的变化。结果:缬沙坦治疗组和贝那普利治疗组24hUAER分别由703.2±987.9μg/min降至664.2±970.6μg/min(P<0.01)和由778.6±1005.0降至734.9±996.0μg/min(P<0.01)。二者疗效相似,且均与血压变化不相关。结论:AT1Ra缬沙坦可以降低临床糖尿病肾病的蛋白尿,其肾脏保护作用除了与降血压有关,还有不依赖降压效应的其他机制。     

Inherited focal, episodic neuropathies

Hereditary neuropathy with liability to pressure palsies (HNPP; also called tomaculous neuropathy) is an autosomal-dominant disorder that produces a painless episodic, recurrent, focal demyelinating neuropathy. HNPP generally develops during adolescence, and may cause attacks of numbness, muscular weakness, and atrophy. Peroneal palsies, carpal tunnel syndrome, and other entrapment neuropathies may be frequent manifestations of HNPP. Motor and sensory nerve conduction velocities may be reduced in clinically affected patients, as well as in asymptomatic gene carriers. The histopathological changes observed in peripheral nerves of HNPP patients include segmental demyelination and tomaculous or “sausage-like” formations. Mild overlap of clinical features with Charcot-Marie-Tooth (CMT) disease type 1 (CMT1) may lead patients with HNPP to be misdiagnosed as having CMT1. HNPP and CMT1 are both demyelinating neuropathies, however, their clinical, pathological, and electrophysiological features are quite distinct. HNPP is most frequently associated with a 1.4-Mb pair deletion on chromosome 17p12. A duplication of the identical region leads to CMT1A. Both HNPP and CMT1A result from a dosage effect of the PMP22 gene, which is contained within the deleted/duplicated region. This is reflected in reduced mRNA and protein levels in sural nerve biopsy samples from HNPP patients. Treatment for HNPP consists of preventative and symptom-easing measures. Hereditary neuralgic amyotrophy (HNA; also called familial brachial plexus neuropathy) is an autosomal-dominant disorder causing episodes of paralysis and muscle weakness initiated by severe pain. Individuals with HNA may suffer repeated episodes of intense pain, paralysis, and sensory disturbances in an affected limb. The onset of HNA is at birth or later in childhood with prognosis for recovery usually favorable; however, persons with HNA may have permanent residual neurological dysfunction following attack(s). Episodes are often triggered by infections, immunizations, the puerperium, and stress. Electrophysiological studies show normal or mildly prolonged motor nerve conduction velocities distal to the affected brachial plexus. Pathological studies have found axonal degeneration in nerves examined distal to the plexus abnormality. In some HNA pedigrees there are characteristic facial features, including hypotelorism. The prognosis for recovery of normal function of affected limbs in HNA is good, although recurrent episodes may cause residual deficits. HNA is genetically linked to chromosome 17q25, where mutations in the septin-9 (SEPT9) gene have been found.     

MBP对PBMC产生IFN-γ和NO的影响

目的探讨T细胞非特异性活化在CNS脱髓鞘性疾病中的作用。方法分离实验性自身免疫性脑脊髓炎(EAE)易感性BALB/c小鼠外周血单个核细胞(PBMC),采用体外细胞培养方法在体外与碱性髓鞘蛋白(MBP)共培养,测定培养上清液中IFN-γ、NO水平。结果经MBP刺激的PBMC产生IFN-γ[(43.83±6.06)pg/mL]和NO[(180.76±20.75)μmol/L]明显增加,与对照组产生的IFN-γ[(28.52±2.18)pg/mL]和NO[(95.61±13.09)μmol/L]相比差异有统计学意义(P<0.01)。结论在CNS脱髓鞘性疾病发病过程中,活化的T细胞、单核细胞等分泌致炎细胞因子和其他有害物质增多。     

Leber’s hereditary optic neuropathy and vitamin B12 deficiency

Background Leber’s hereditary optic neuropathy (LHON) is a maternally inherited optic neuropathy caused by mutations in mitochondrial DNA (mtDNA). It is also believed that several epigenetic factors have an influence on the development of LHON.Methods A case series was observed.Results Three patients who developed bilateral optic neuropathy are presented. All patients had a primary LHON mutation in their mtDNA, but also a subnormal vitamin B12 serum level at the time of presentation.Conclusions The clinical picture of optic neuropathy associated with vitamin B12 deficiency shows similarity to that of LHON. Both involve the nerve fibres of the papillomacular bundle. The present case reports suggest that optic neuropathy in patients carrying a primary LHON mtDNA mutation may be precipitated by vitamin B12 deficiency. Therefore, known carriers should take care to have an adequate dietary intake of vitamin B12 and malabsorption syndromes like those occurring in familial pernicious anaemia or after gastric surgery should be excluded.     

老年女性2型糖尿病肾病的骨密度及相关因素分析

2型糖尿病(T2DM)和骨质疏松症的发病率越来越高,它们之间有无关系越来越引起人们的重视,本研究探讨了老年女性2型糖尿病肾病(DN)的骨矿物质密度(BMD)变化与相关因素,报道如下。1对象与方法1.1研究对象按1999年WHO诊断标准确诊为T2DM的≥60岁女性114例,年龄60～75岁,平均(66.3±