Sensory axonopathy in hereditary distal spinal muscular atrophy

A girl of 14 year is presented with a distal spinal muscular atrophy (SMA) with autosomal recessive inheritance. The technical findings are in agreement with the diagnosis. Light microscopical examination of sural nerve biopsy, including teased fiber studies and morphometry, showed no abnormalities. Electron microscopical investigation however demonstrated axonal pathology. The question arises if distal SMA is a distal axonopathy mainly of motor nerves, but to some extent also of sensory nerves.     

膝关节镜下膝后侧腔室联合手术入路的探讨

[目的]探讨在关节镜下膝关节后侧腔室联合手术入路的重要性和可操作性。[方法]经过前内侧、前外侧和股骨髁切迹以及后内侧、后外侧和后纵隔内切口联合入路分别入镜、入器械，进行膝关节后侧腔室的探查和手术操作。[结果]216例（239膝）应用联合入路探查和治疗，其中5例膝因关节僵硬操作失败；175例膝用于治疗后侧腔室疾病，膝关节后侧腔室手术视野显著改善，探查和手术操作完善，均达到手术目的。1例膝内侧隐神经不全损伤，没有腘后神经、腓总神经、腘后血管、交叉韧带等重要组织损伤。[结论]膝关节后侧腔室病变较多，是检查和治疗的重要部位，并非“技术盲区”。这种联合手术入路，手术风险低，具备可操作性，可以提高手术效率和质量，可作为膝关节镜下常规手术入路。     

青少年特发性胸椎侧凸患者的脊髓偏移及临床意义

[目的]揭示青少年胸椎特发性侧凸患者椎管内脊髓的偏移，明确其变化趋势，并探讨其可能的发生机制和临床意义。[方法]本研究包括39名以右胸弯为主弯的青少年特发性脊柱侧凸（adolescent idiopathic scoliosis，AIS）患者。测量主胸弯节段内（T5-12）椎管内脊髓与凸凹侧椎弓根之间的距离，计算脊髓的偏移，揭示其变化趋势，并分析顶椎区脊髓偏移与主胸弯Cobb＇s角和顶椎相对偏移之间的相关性。[结果]在T5-12节段椎管内，脊髓与凸侧椎弓根之间的距离显著大于与凹侧椎弓根之间的距离（P〈0．05），即脊髓向凹侧椎弓根偏移，且以顶椎区最为显著，而逐渐向两侧端椎区递减。顶椎区脊髓偏移与主胸弯Cobb’s角和顶椎相对偏移存在显著的正相关（相关系数分别为0．631和0．546）。[结论]胸椎特发性脊柱侧凸患者存在侧凸节段椎管内脊髓偏移的现象，且以顶椎区最显著。研究结果提示脊髓偏移可能与脊椎偏移后凹侧脊神经的牵拉有关，而且凹侧置钉的风险高于凸侧。     

神经干细胞静脉移植治疗脊髓损伤的实验研究

[目的]观察神经干细胞静脉移植对损伤大鼠脊髓功能的治疗作用。[方法]取孕14—16dSD胎鼠的脑室下区组织，体外培养后鉴定细胞。制作脊髓全切模型，伤后1周将Brdu标记好的神经干细胞通过尾静脉注射移植到大鼠体内，移植后及8周行皮层体感诱发电位（CSEP）检测和BBB功能评分，并留损伤脊髓处作病理切片及免疫组化染色。[结果]（1）移植后8周BBB评分损伤组、移植组都有所恢复，但都未达到正常水平，移植组恢复较好；（2）模型制作后，CSEP波均消失，细胞移植后8周移植组的波形有不同程度的恢复，但潜伏期延长；（3）移植组大鼠脊髓损伤处存在大量Brdu染色阳性细胞，表明移植的细胞在体内可到达损伤脊髓处并能存活；脊髓损伤部位NF-200及GFAP染色阳性的细胞表明移植的细胞可以分化为具有神经元和胶质细胞特性的细胞。[结论]静脉移植的神经干细胞能到达损伤区代替受损的神经元及神经胶质细胞，使损伤的脊髓功能得到一定程度的恢复。     

Specific selection of osteosynthetic material in the treatment of thoracic or lumbar spinal injuries by the posterior approach

Summary The authors report 165 cases of thoraco-lumbar lesions with neurological dysfunction. All the patient were operated. They analyze the neurological and mechanical results and indicate the use of different osteosynthesis apparatus according to the type and level of lesions.Harrington's rods seem to give more precise repositioning while Roy Camille's plates give more stability. When the posterior wall of the spinal canal is intact, Kempf's compression rods can be used.Thoraxic spine injuries seem to be an indication for Harrington's rods, while lumbar injuries seem to call for Camille's plates.     

Time for sport and health to be formally linked in a positive way

Preventing sports injury has rarely been cited as an appropriate action to respond to the obesity epidemic, and in fact a recent letter has suggested that those playing sport are as responsible for their predicament as those who are obese. This opinion piece argues that it is time for better prevention and management of sports injury to be seen as part of the complex solution to preventing obesity, rather than being a self-inflicted problem that governments should continue to ignore.     

Effect of the free radical scavenger MCI-186 on spinal cord reperfusion after transient ischemia in the rabbit

Objective: Paraplegia remains a serious complication of aortic operations. The production of free radicals during reperfusion after transient ischemia is believed to induce secondary spinal neuronal injury, resulting in paraplegia. The aim of the present study was to clarify the protective effect and method of administration of antioxidants on the neurological and histological outcome in the animal model for reperfusion injury after transient spinal cord ischemia. Methods: New Zealand white rabbits underwent surgical exposure of the abdominal aorta that was clamped for 15 minutes to achieve spinal cord ischemia. Group A animals received two 10 mg/kg doses of 3-methyl-l-phenyl-2-pyrazolin-5-one (MCI-186) at the time of release of the aortic clamp and 30 minutes later. In group B, MCI-186, 5 mg/kg, was given three times, at the time of aorta clamp release, 30 minutes and 12 hours later. In group C (control group), one dose of vehicle was administered. Neurological status was assessed using modified Tarlov’s score until 168 hours after operation. Spinal cord sections were examined microscopically to determine the extent of ischemic neuronal damage. Results: Groups A and B animals had better neurological function than group C (p(0.001). In contrast, group C animals exhibited paraplegia or paraparesis with marked neuronal necrosis. The number of surviving neurons within examined sections of the spinal cord was significantly greater in group B than in group C (p(0.001). Conclusion: In a 15-minute ischemia-reperfusion model using rabbits, systemic repetitious administration of MCI-186, a free radical scavenger, was found to have a protective effect on the spinal cord neurons both neurologically and histologically. We postulate that the drug minimizes the delayed neuronal cell death for reperfusion injury after transient ischemia by reducing the free radical molecules. Moreover, it was thought that we could protect delayed neuronal cell death more effectively by administering MCI-18612 hours later.     

腰椎后路非融合固定系统的临床应用

复习腰椎后路非融合固定系统的设计原理、临床应用及治疗效果等相关文献,与传统的脊柱融合术相比,应用非融合系统可获得很好的疗效,并可以减少邻近节段退变的发生率。     

Neuroimaging and Neurologic Complications after Organ Transplantation

Neurologic complications are common after transplantation and affect 30-60% of transplant recipients. The etiology of most of the posttransplant neurologic disorders is related to the opportunistic infections, both systemic and involving central nervous system (CNS), toxicity of immunosuppressive medications, and the metabolic insult created by the underlying primary disease and the transplant procedure. Neuroimaging studies are one of the key tools in the evaluation and enable early diagnosis of neurologic complications in transplant patients, especially posterior reversible leukoencephalopathy syndrome, central pontine myelinolysis, intracerebral hemorrhage, and fungal and bacterial abscesses. Magnetic resonance imaging (MRI) is the preferred technique, but each of the available neuroimaging techniques offers a unique insight into the pathophysiologic mechanisms underlying neurologic complications of transplantation. The role of neuroimaging in this population includes early detection of calcineurin inhibitor neurotoxicity, opportunistic infections, neoplasia, metabolic disorders, or cerebrovascular diseases. In addition, we can monitor longitudinal progression of disease and treatment response.     

Prenatal diagnosis of Werdnig Hoffmann disease in China

Objective To establish a means for prenatal prediction of spinal muscular atrophy (SMA) through survival motor neuron (SMN) gene deletion analysis and genetic counseling in families with a child affected with SMA.
Methods Genetic analysis for prenatal prediction of Werdnig-Hoffmann disease was performed in a at risk Chinese family by polymerase chain reaction (PCR)-single-strand conformation polymorphism (SSCP) in SMN gene exons 7 and 8.
Results The pregnancy was positive for the homozygous deletion of the SMN gene, thus the fetus was diagnosed as being affected and the pregnancy was terminated.
Conclusion This approach is fast and reliable for DNA-based prenatal diagnosis of Werdnig-Hoffmann disease.