青年上肢远端肌萎缩症的临床及电生理特点

目的 通过回顾性分析青年上肢远端肌萎缩症的临床和电生理检测结果,探讨该病的神经电生理特点.方法 分析77例青年上肢远端肌萎缩症患者正中神经和尺神经的运动和感觉神经传导、F波潜伏期及响应率,并分析针极肌电图中上肢肌受累情况.结果 在77例青年上肢远端肌萎缩症患者中,男74例,女3例;平均发病年龄(17.7±2.6)岁,平均病程(28.2±25.7)个月;57例患者以单侧上肢受累为主诉.尺神经复合肌肉动作电位(CMAP)波幅降低最多见;正中神经和尺神经F波响应率降低;感觉神经传导速度和感觉神经动作电位(SNAP)波幅均在正常范围内.针极肌电图表现为慢性神经源性改变为主,部分呈活动性改变.其中仅10例发现单侧上肢肌异常,67例均表现为双上肢肌损害.77例患者均有颈7～胸1节段的神经源性损害,部分患者伴有不同程度的颈5、6节段损害表现.无一例见颈1～颈4及其他节段支配肌的神经源性改变.结论 青年上肢远端肌萎缩症神经传导检查突出表现为尺神经CMAP波幅降低,尺神经和正中神经F波响应率降低.针极肌电图表现为双侧颈7、8与胸1节段支配肌呈慢性神经源性损害,可伴有颈5、6节段支配肌受累.     

远端型肌萎缩型颈椎病1例报告

正肌萎缩型颈椎病(CSA)又称为"Keegan型颈椎病",主要表现为单/双侧上肢肌肉萎缩(单侧为主),通常伴/不伴轻度的感觉异常及脊髓病变,是颈椎病的一种特殊类型,发生率较低~([1]),容易与运动神经元病、平山病、脊肌萎缩症等疾病混淆,造成漏诊、误诊。本院2015年8月收治1例远端型CSA患者,现将诊疗过程报告如下。     

颈椎前路植骨融合内固定术治疗平山病疗效的电生理评估

【摘要】 目的：通过电生理技术对接受颈椎前路植骨融合内固定手术治疗的平山病患者手术前后神经功能进行评估,探究手术干预对于神经功能恢复的作用。方法：回顾性分析2016年1月~2017年7月于我院骨科接受颈椎前路植骨融合内固定手术的平山病患者43例,其中男性41例,女性2例;手术时年龄14~26岁,平均18.2±2.8岁,病程6~84个月,平均22.3±20.1个月;随访时间3~18个月,平均7.2±4.4个月。所有患者均在术前及末次随访时接受上肢神经传导检查、针肌电图检查和上肢功能障碍评定（disability of arm shoulder hand,DASH）量表评估。通过比较复合肌肉动作电位（compound muscle action potential,CMAP）波幅、肌肉自发电位、运动单位电位形态及肌肉募集情况评估患者术后神经功能改变,并通过DASH量表评分变化评估患者术后上肢功能变化。分别比较术前症状严重侧和轻侧及手术前后电生理检查结果,通过配对样本t检验比较CMAP波幅,Pearson卡方检验比较肌肉自发电位、运动单位电位形态及肌肉募集情况;通过配对样本t检验比较手术前后DASH量表评分变化。结果：患者手术前神经电生理检查结果显示,症状严重侧正中神经及尺神经波幅较轻侧明显降低（P＜0.05）,超过半数患者针肌电图检查可见双侧神经源性损害。术前及术后随访结果对比显示,术前症状严重侧正中神经CMAP波幅平均为9.79±4.63mV,术后平均为11.04±4.43mV,差异有统计学意义（P＜0.05）;术前症状严重侧尺神经CMAP波幅平均为4.04±3.25mV,术后平均为6.19±4.30mV,差异有统计学意义（P<0.001）。轻侧正中神经及尺神经CMAP波幅恢复情况无统计学意义（P＞0.05）;针肌电图检查结果显示,患者严重侧上肢被检肌肉自发电位出现比例、MUAP形态宽大比例下降无统计学意义（P>0.05）;部分被检肌肉募集情况有较为显著改善,其中术前42例患者症状严重侧桡侧腕屈肌存在募集减弱（42/43,97.7%）,末次随访时32例患者同一肌肉存在募集减弱（32/38,84.2%）,差异有统计学意义（P＜0.05）。患者术前DASH量表评分为7.04±7.63分,末次随访时6.89±7.47分,差异无统计学意义（P>0.05）。结论：平山病患者接受颈椎前路植骨融合内固定手术后,上肢CMAP波幅提高,肌肉募集情况改善,该手术对控制病情进展、改善神经功能有积极作用。     

近端肌萎缩型颈椎病11例的诊治分析

目的:探讨近端肌萎缩型颈椎病的临床特点、治疗方法及临床效果。方法:回顾性分析2016年9月至2020年12月治疗的11例近端肌萎缩型颈椎病患者,其中男7例,女4例,年龄38~68岁。分析其临床症状特点、MRI及神经电生理表现,分别采用保守治疗或颈椎前路减压融合手术进行治疗,治疗前后采用徒手肌力评定方法(manual muscle test,MMT)对患者进行疗效评价,同时随访患者满意度。结果:所有患者获得随访,时间6~19个月。11例均为单侧发病,主要表现以三角肌、冈上肌、冈下肌萎缩为主,早期可以伴有同侧颈肩痛;MRI显示以C_4,5、C_5,6节段病变多见,神经电生理检查表现为受累肌肉失神经支配,患侧支配神经复合肌肉动作电位(compound muscle action potential,CMAP)波幅较健侧降低。所有手术患者获得骨性融合,1例行颈椎体次切减压融合术(anterior cervical corpectomy and fusion,ACCF)术后出现对侧C₅神经根麻痹,经对症治疗10周后完全恢复。治疗后12个月时根据MMT疗效评估:保守治疗3例,优2例,良1例;手术治疗8例,优3例,良4例,可1例。结论:近端肌萎缩型颈椎病发病率低,表现为单侧近端肌肉萎缩,早期可以伴有同侧颈肩痛,结合MRI、神经电生理检查可以减少误诊。在发病早期尤其是髓核脱出导致神经压迫的可以采取保守治疗,当保守治疗无效或疼痛不能耐受时建议行前路减压手术,整体疗效满意。     

颈椎前路减压自稳性融合器植骨融合术治疗近侧型肌萎缩型颈椎病的临床分析

目的探讨颈椎前路减压、自稳性椎间融合器植骨融合术治疗近侧型肌萎缩型颈椎病的临床疗效。方法回顾性分析2014年1月至2018年1月我科采用颈椎前路减压自稳性椎间融合器植骨融合术治疗的近侧型肌萎缩型颈椎病12例,男8例,女4例;年龄36～65岁,平均(45.2±7.6)岁;术前肌萎缩症状持续时间为6～36个月,平均(16.6±5.4)个月。术前肌电图评估神经损害情况,通过影像学观察神经受侵犯节段情况、神经减压及内固定情况,采用徒手肌力测试方法评估患者术前及术后肌力,根据肌力改善程度分成优、良、中、差4个等级,优和良评估为疗效满意。结果肌电图报告所有病例均显示受累上肢肌肉的神经源性损害,且均未显示舌肌纤颤电位及胸锁乳突肌神经源性损害;12例均获随访,随访时间为12～60个月,平均(35.5±8.8)个月。9例患者肌力较术前改善,其中优4例,良5例,肌力无明显改善3例,无术后肌力较术前下降情况,疗效满意率为75%。结论肌萎缩型颈椎病临床少见,且易与脊髓侧索硬化等运动神经元疾病相混淆,肌电图和MRI检查有助于鉴别诊断,采取颈椎前路减压自稳性融合器植骨融合术治疗近侧型肌萎缩型颈椎病有比较满意的效果。     

健侧C7神经根移位术后患肢对应皮层体感诱发电位的变化规律

目的：对照已行健侧C7神经根移位术患者与正常人的皮层体感诱发电位(somatosensory evoked potential，SEP)，了解、分析大脑重塑过程中电生理变化特点。方法：12例左侧臂丛神经撕脱伤已行健侧C7→尺神经→正中神经重建患者，其中6例患肢感觉在健侧者为A组，感觉转到患侧者为B组；同时以12位正常人作为对照组。利用SEP技术，刺激左上肢正中神经腕部、肘部，分别记录左右两侧皮层SEP的波形，测定其潜伏期、波幅和腕→肘神经传导速度，并进行统计学比较。结果：正常人两侧皮层SEP的潜伏期、波幅之间存在差异，但潜伏期差异无显著性，而波幅差异有显著性。A组患者两侧皮层间SEP潜伏期差异有显著性，波幅差异无显著性。B组患者两侧皮层间SEP潜伏期差异无显著性，而波幅差异则存在显著性。A组与B组患者对侧皮层SEP潜伏期和波幅差异均有显著性。结论：健侧C7神经根移位术后，患肢对侧皮层发生功能重塑，两侧皮层记录的SEP潜伏期差值缩短，而波幅差异增大。患侧SEP检测可为患肢对应皮层功能重塑的“再支配”现象提供相应佐证与数据，为临床采取进一步措施促进患者肢体与大脑皮层功能的恢复提供客观依据。     

肌萎缩型颈椎病1例报告

正Sobue等~([1])于1975年提出"节段性脊髓病变"理论,首次将以上肢肌肉萎缩为特征的颈椎病命名为肌萎缩型颈椎病(CSA)。CSA又称为"Keegan型颈椎病",以颈椎退变为基础,主要表现为节段性、非对称性、稳定性上肢肌肉萎缩,伴/不伴轻度的感觉异常及脊髓病变(表现为下肢症状及步态异常),使患者的生活质量严重降低~([2])。CSA发生率较低,易发生漏诊、误诊,应引起临床医生足够的重     

神经电生理检查对神经根型颈椎病与肘管综合征的鉴别诊断价值

目的：探讨神经电生理检查对神经根型颈椎病与肘管综合征的鉴别诊断价值。方法：对14例以手部内在肌萎缩为主要临床表现的患者进行双上肢体感诱发电位（somatosensory evoked potential，SEP）、双侧正中神经和尺神经传导速度（nerve conduction velocity，NCV）、双侧第一背侧骨间肌和尺侧腕屈肌肌电罔（electromyogram，EMG）检查。结果：3例以尺神经肘上-肘下段传导速度减慢大于10m／s及第一背侧骨间肌神经源性损害为主，诊断为肘管综合征；4例以SEP颈髓至外周电位（N9-N13）峰间潜伏期延长和尺侧腕屈肌、第一背侧骨间肌神经源性损害为主，诊断为神经根型颈椎病；7例为尺侧腕屈肌及第一背侧骨间肌神经源性损害、尺神经肘上-肘下段传导速度减慢大于10m／s、SEPN9-N13峰间潜伏期延长，诊断为二者合并存在。手术治疗10例，术中所见均与神经电生理检查结果相符。结论：神经电生理检查在神经根型颈椎病与肘管综合征的鉴别诊断中具有重要提示意义。     

平山病及其影像学特征研究进展

<正>平山病(Hirayama′s disease,HD),又称青少年上肢远端肌萎缩症,由日本学者平山惠造于1959年首次报道[1]。其典型的临床特点为青少年隐匿起病,男性多发,一侧或双侧不对称的上肢远端肌肉无力和萎缩,疾病进展数年后多自然静止。平山病在临床症状上与肌萎缩侧索硬化症(amyotrophic lateral sclerosis,ALS)等运动神经元疾病相似,但治疗方法和预后有很大不同。因此对平山病及时、准     

肌萎缩型颈椎病的诊断与治疗

肌萎缩型颈椎病是一种特殊型颈椎病 ,它没有明显的感觉障碍 ,也不伴疼痛 ,主要症状是双侧或单侧上肢、近侧肌或远侧肌无力及萎缩。此型颈椎病由Ａｌｌｅｎ和Ｂｒａｉｎ于 195 2年首先报告 ,由Ｋｅｅｇａｎ[1] 于 1965年根据解剖所见 ,提出发病机制。由于该型颈椎病是一种少见的非典型颈椎病 ,对其发病及诊断报告的较少。我们根据 2 0 0 0年 2月～ 2 0 0 0年 8月临床所见的 4例典型病例及有关文献资料对该型颈椎病的诊断及治疗加以探讨。1　临床资料1.1　一般资料　本组 4例 ,均为女性 ,年龄 73、66、5 6、5 7岁 ,病程 1个月～ 2年。1.2　临…     

Reduction of Plasma Fibrinogen, Immunoglobulin G, and Immunoglobulin M Concentrations by Immunoadsorption Therapy with Tryptophan and Phenylalanine Adsorbents

Abstract Immunoadsorption (1A) therapy with tryptophan (TR-350) or phenylalanine (PH-350) adsorbents has been used to reduce the concentration of serum antibodies in human lymphocyte antigen (HLA)-immunized patients. Other forms of plasma purification have been reported to reduce the level of fibrinogen, which affects the blood properties. In this study we investigated the effects of IA therapy using both adsorbents on plasma fibrinogen and immunoglobulins G and M in 13 patients (8 patients were treated with TR-350, and 5 patients were treated with PH-350). During each session 1 plasma volume (2.8 ± 0.4 L of plasma) was processed through the immunocolumn and then returned to the patient together with the blood cells. Compared with the pretreatment values, the plasma fibrinogen, IgG, and IgM concentrations were significantly reduced after IA therapy (p < 0.01 for TR-350; p < 0.04 for PH-350). There was a positive correlation between the degree of reduction of plasma proteins and the number of IA treatments given. A nonpara-metric test (Wilcoxon's signed-rank test or the Mann-Whitney test) was used for statistical analysis. We conclude from our study that IA therapy effectively lowers the plasma levels of fibrinogen, IgG, and IgM and thus can be considered a valuable alternative to other blood purification methods.     

Is the recovery profile of mivacurium independent of the rate of decay of its plasma concentration in patients with normal plasma cholinesterase activity?

Background: The duration of action of muscle relaxants is poorly correlated to the rate of decay of their plasma concentration. The plasma concentration of mivacurium may rapidly decrease below its active concentration because of the extensive hydrolysis of mivacurium. By inflating a tourniquet on one upper limb for 3 min after the administration of atracurium, mivacurium or vecuronium, we studied the influence of the initial decline of their plasma concentration on their effect. Methods: In 50 patients anaesthetised with thiopental, isoflurane and fentanyl, the effect of bolus doses of 0.15 or 0.25 mg . kg^?1 mivacurium (MIV 15, MIV 25), 0.3 or 0.5 mg . kg^?1 atracurium (ATR 30, ATR 50) and 0.06 or 0.1 mg . kg^?1 vecuronium (VEC 06, VEC 10) were measured on both arms (evoked response of the adductor pollicis to train-of-four stimulation every 12 s), a tourniquet being applied on one arm just before and during 3 min after the muscle relaxant bolus. Results: Tourniquet inflation of 3 min almost abolished the neuromuscular effect of mivacurium. In the vecuronium groups and in the ATR 50 group, tourniquet inflation did not modify the maximum degree of depression of the twitch response. Also, the duration of action of vecuronium was unaffected by the tourniquet. In the ATR 30 group, times to return of the twitch response to 25% (duration 25%) and 75% (duration 75%) of control response were significantly shorter in the cuffed arm, 23 min vs 27 min, and 41 min vs 45 min, respectively. In the ATR 50 group, only duration 25% was significantly shorter in the cuffed arm (41 min vs 45 min). Conclusion: The results suggest that the rate of decline of the plasma concentration of mivacurium is so rapid, that a very low and almost clinically ineffective concentration is present as soon as 3 min after its administration. The results also indicate that the recovery from a mivacurium-induced neuromuscular blockade is not influenced by the rate of decay of its plasma concentration in patients with genotypically normal plasma cholinesterase.     

Membranes in Artificial Organs

Abstract: Membrane processes play a pivotal and enabling role in modern replacement therapy for acute and chronic organ failure and in the management of immunologic diseases. In fact, virtually all contemporary extracorporeal blood purification methods employ membrane devices, and the next generation of artificial organs and tissue engineering therapies are almost certain to be similarly grounded in membrane technology. In this short essay, we comment on the similarities and differences among synthetic membranes and their natural counterparts and also provide a critical overview of the demographics and technology of hemodialysis, hemofiltration, apheresis, oxygenation, and emerging membrane technologies and applications.     

The multiply injured child

Blunt trauma is the principal cause of childhood death in many developed countries. This review outlines the differences between adults and children with respect to resuscitation and treatment of orthopaedic injuries in a child with polytrauma. Recent advances in techniques of fracture stabilization are reported.     

Dilemma of Membrane Biocompatibility and Reuse

Abstract: Numerous articles have been published on the multiple use of dialyzers and on the effect of different reprocessing chemicals and techniques on the dialyzer biocompatibility and performance. The results often appear contradictory, especially those comparing standard biocompatibility parameters. Despite this confusion, a discerning review of the published works allows certain limited conclusions to be drawn. Reprocessing of used hemodialyzers changes the biocompatibility profile of a dialyzer as defined by the parameters complement activation. leukopenia, and cytokine release. The effect of reprocessing depends on the chemicals and reprocessing technique applied and also on the type of membrane polymer being subjected to the reprocessing procedure. Reports of pyrogenic reactions indicate that the flux of the membrane also influences how suitable it is for safe reuse. An increased risk of allergic and pyrogenic reactions appears to be associated with dialyzer reuse. Furthermore, there has been a lack of investigations into the immunologic effect of the layer of adsorbed and chemically altered proteins that remains on the inner surface of reprocessed dialyzers. We conclude that the clinical benefit of dialyzer reuse cannot be generally accepted from a biocompatibility point of view.     

The analgesic effect of racemic ketamine in patients with chronic ischemic pain due to lower extremity arteriosclerosis obliterans

Background : Ketamine in sub-dissociative doses has been shown to have analgesic and phantom-Limb pain, where conventional treatment has often failed. Chronic ischemic pain due to lower extremity arteriosclerosis obliterans often responds poorly to analgesics, and the pain-generating mechanisms are not well understood.
Methods : Eight patients with rest pain in the lower extremity due to arteriosclerosis obliterans were given sub-dissociative doses of 0.15, 0.30, or 0.45 mg/kg racemic ketamine and morphine 10 mg as a 5-min infusion on four separate days in a cross-over, double-blind, randomised protocol. Plasma levels of (S)- and (R)-ketamine and their nor-metabolites were analysed with an enantioselective high-performance liquid chromatography (HPLC) method. Pain levels were evaluated with a visual analogue scale (VAS).
Results : Individual pain levels were highly variable during and after all the infusions but the pooled pain levels showed a dose-dependent analgesic effect of ketamine with a transient but complete pain relief in all patients at the highest dose (0.45 mg/ kg). Side-effects, mainly disturbed cognition and perception, were pronounced and dose-dependent. Morphine 10 mg had an analgesic peak at 20 min and 5/8 patients had complete pain relief. The remaining 3 patients also had high baseline pain scores, indicating a higher analgesic potency for the 0.30 and 0.45 mg/ kg ketamine doses than for morphine 10 mg.
Conclusion : We have demonstrated a potent dose-dependent analgesic effect of racemic ketamine in clinical ischemic pain. Due to a narrow therapeutic window, this analgesic effect is probably best utilised in combination with other analgesics.     

Intravenous endotoxin does not increase tissue extravasation of albumin in rats

Background : It is unclear whether activation of the inducible nitric oxide synthase (iNOS) increases or decreases the extravasation of plasma.
Methods : Chloralose anaesthetised male Wistar rats received E. coli lipopolysacharide (LPS), 3 mg kg^-1 i.v., or the corresponding volume of saline, 3 or 5 h before the end of the experiment. Mean arterial pressure (MAP) and heart rate (HR) were recorded. Tissue clearance of radio-labelled albumin, during the last 2 h of each experiment, was determined by a double-isotope method. In separate animals, the serum concentration of nitrite and nitrate was determined, 5 h after LPS or the solvent.
Main Results : LPS initially decreased MAP and lastingly increased HR. In the 3-h LPS animals (n=8), tissue plasma clearance was lower in the heart and calf muscle and increased only in diaphragm, compared to corresponding control animals (n=8). In the 5-h LPS rats, clearance was lowered (n=8) in the entire gastrointestinal tract and in testes, compared to controls (n=8). The serum nitrite/nitrate concentration was higher in animals given LPS (n=6) than in controls (n=6).
Conclusion : After LPS, tissue clearance of albumin was not increased in any major tissue, in spite of increased serum levels of NO end products. Apparently, after activation of iNOS, the augmented release of NO is not necessarily associated with increased albumin extravasation.     

Adrenaline markedly improves thoracic epidural analgesia produced by a low-dose infusion of bupivacaine, fentanyl and adrenaline after major surgery: A randomised, double-blind, cross-over study with and without adrenaline

Background: Basic pharmacological research indicates that there are synergistic antinociceptive effects at the spinal cord level between adrenaline, fentanyl and bupivacaine. Our clinical experience with such a mixture in a thoracic epidural infusion after major surgery confirms this. The objectives of the present study were to evaluate the effects on postoperative pain intensity, pain relief and side effects when removing adrenaline from this triple epidural mixture. Methods: A prospective, randomised, double-blind, cross-over study was carried out in 24 patients after major thoracic or abdominal surgery. Patients with only mild pain when coughing during a titrated thoracic epidural infusion of about 10 ml · h^?1 of bupivacaine 1 mg · ml^?1, fentanyl 2 μg · ml^?1, and adrenaline 2 μg · ml^?1 were included. On the 1^st and 2^nd postoperative days each patient was given a double-blind epidural infusion, at the same rate, with or without adrenaline. The effect was observed for 4 h or until pain when coughing became unacceptable in spite of a rescue analgesic procedure. Rescue analgesia consisted of up to two epidural bolus injections per hour and i.v. morphine if necessary. All patients received rectal paracetamol 1 g, every 8 h. Fentanyl serum concentrations were measured with a radioimmunoassay technique at the start and end of each study period. Main outcome measures were extent of sensory blockade and pain intensity at rest and when coughing, evaluated by a visual analogue scale, a verbal categorical rating scale, the Prince Henry Hospital pain score, and an overall quality of pain relief score. Results: The number of hypaesthetic dermatomal segments decreased (P <0.001) and pain intensity at rest and when coughing increased (P <0.001) when adrenaline was omitted from the triple epidural mixture. This change started within the first hour after removing adrenaline. After 3 h pain intensity when coughing had increased to unacceptable levels in spite of rescue analgesia (epidural bolus injections and i.v. morphine). Within 15–20 min after restarting the triple epidural mixture with adrenaline, pain intensity was again reduced to mild pain when coughing. Serum concentration of fentanyl doubled from 0.22 to 0.45 ng · ml^?1 (P <0.01), and there was more sedation during the period without adrenaline. Conclusions: Adrenaline increases sensory block and improves the pain-relieving effect of a mixture of bupivacaine and fentanyl infused epidurally at a thoracic level after major thoracic or abdominal surgery. Serum fentanyl concentrations doubled and sedation increased when adrenaline was removed from the epidural infusion, indicating more rapid vascular absorption and systemic effects of fentanyl.