痉挛性脑瘫患者感觉和运动障碍的特点及其原因

目的 :探讨痉挛性脑瘫患者感觉和运动能力障碍的特点与机制。方法 :分析 10 2例痉挛性脑瘫患者临床感觉和运动能力的观察结果 ,取 2 5例痉挛性脑瘫患者的周围神经标本 40个、骨骼肌标本 5 4个进行透射电镜观察。结果 :痉挛性脑瘫患者浅感觉 (即痛觉 )基本正常 ,深感觉 (即关节觉 )障碍明显 ;上肢远端肌肉主动随意活动能力比近端肌肉减退明显 ,而下肢远近端差异不显著 ;电镜观察周围神经呈原发性脱髓鞘表现 ,有髓纤维病变明显于无髓纤维 ;骨骼肌纤维亚显微结构Ⅰ型纤维表现突出。结论 :痉挛性脑瘫有明显的深感觉障碍 ,而浅感觉基本正常 ,周围神经有髓纤维的脱髓鞘病变可能是其原因之一 ;上肢远端运动能力障碍比近端明显与上位中枢的生理特性有关 ;骨骼肌纤维构型发生变化 ,以Ⅰ型纤维占优势 ,影响其收缩特性 ,也是影响运动能力的原因之一。     

脑瘫患者脊神经根亚显微病变及意义

目的：探讨脑瘫患儿在其后的生长发育过程中，周围神经受累改变的病理及意义。方法：应用光镜及透射电镜对２０例痉挛型瘫患者脊神经背根进行了亚星微形态学观察。结果：粗有髓神经纤维受累明显，表现为不同程度脱髓鞘病变，轴索病变相对不明显；无髓神经纤维以其周围的雪旺氏细胞病变为主。束内微血管亦有病变。神经受损后无修复与再生现象。结论：本病周围神经的继发性病变不仅广泛存在，且为不可逆性，选择性脊神经背根切断术可以     

POEMS综合征的临床与周围神经病理分析

目的：探讨POEMS综合征的临床和周围神经病理特点。方法：回顾性分析5例POEMS综合征的临床特征和4例周围神经活检结果。结果：5例患者临床上均有感觉运动性周围神经损害和肢端凹陷性水肿，腹水3例，胸腔积液2例，心包积液2例。肝脏肿大2例，脾脏肿大3例，淋巴结肿大2例。皮肤变黑5例，多毛症3例。4例腓肠神经活检中，3例神经束膜增厚，束间小血管增多，周围散在有单核细胞。4例均可见脱髓鞘改变和有髓纤维减少，3例可见到少数纤维轴索变性。单神经纤维剥离检查4例，均可见阶段性脱髓鞘改变和薄髓纤维。结论：POEMS综合征是一组以多发性神经病、脏器肿大、内分泌病变、M蛋白、皮肤损害等多系统损害的临床症候群。周围神经病理改变主要为薄髓纤维和脱髓鞘改变，晚期可见轴索变性。     

脑瘫患者脊神经根病变与临床关系探讨

对２０例脑瘫患者脊神经根光镜及秀射电镜的病理形态学观察表明，患儿在生长发育过程中，周围神经普遍受累，以粗有髓神经纤维明显，主要为各种脱髓鞘病变，且呈髓鞘轴索分离现象。无髓神经纤维以其周围的雪旺氏细胞病变为主，束内微血管亦发生病变。神经受损后无修复与再生。     

肌萎缩侧索硬化症的周围神经损害

目的 阐明肌萎缩侧索硬化症患者腓肠神经的病理改变特点。方法 选择已确诊的肌萎缩侧索硬化症患者行周围神经活检。取其腓肠神经进行光镜及电镜检查，观察其病理改变。结果 周围神经的改变以轴索损害为主，伴有髓鞘的脱失。根据病理改变可分为4组：正常；轻度轴索变性及髓鞘脱失；有髓神经纤维轻度脱失，轴索变性及脱髓鞘；有髓神经纤维严重脱失，轴索变性及脱髓鞘。结论 肌萎缩侧索硬化症患者常常早期即出现周围神经的损害，其中以轴索性的改变为主，同时可伴有不同程度的髓鞘脱失。     

腓肠神经形态学观察及半定量分析在多发性周围神经病中的作用

目的 :评价腓肠神经的形态学观察及半定量分析在多发性周围神经病诊断中的作用。方法 :回顾性分析 3例急性炎性脱髓鞘性周围神经病、12例慢性炎性脱髓鞘性周围神经病和 3例遗传性感觉运动神经病患者的病理资料 ,并通过PharmaciaBiotech图象分析仪在患者腓肠神经的半薄切片上随机选取 30条有髓纤维 ,计算平均纤维直径、平均轴索直径、g比率、髓鞘面积与轴索面积的比值。结果 :各组患者腓肠神经的纤维平均直径和轴索平均直径均大于正常对照 ,但仅遗传性感觉运动神经病组有显著差异 (P <0 .0 1) ,而 g比率、髓鞘与轴索面积之比在各组间未显示明显差异 (P >0 .0 5 )。患者组腓肠神经的形态学改变为非特异性 ,炎性细胞浸润较少见。结论 :腓肠神经半定量分析对于诊断急性及慢性炎性脱髓鞘性周围神经病有一定价值。     

丙烯酰胺中毒对周围神经和血管的损害

目的 探讨急性丙烯酰胺中毒患者的周围神经和血管的病理改变。方法 报道2例青年男性工作中接触丙烯酰胺的患者。结果 2例患者的神经传导速度和诱发电位均明显异常。神经活检显示有髓神经纤维轻度减少，在例1可以看到有髓神经纤维和无髓神经纤维的轴索变性和出现个别巨大轴索。例2的有髓神经纤维和无髓神经纤维的轴索出现Wallerian变性和轴索内微丝聚集现象。两例患者均存在个别有髓神经纤维的髓鞘变薄。小动脉内膜显著肥厚，毛细血管周细胞存在凋亡改变，毛细血管的基底膜显著肥厚。结论 丙烯酰胺中毒的周围神经损害为轴索性周围神经病伴随有髓神经纤维的髓鞘损害。血管损害在周围神经病变的发生中也可能发挥重要作用。     

曲尼司特对单纯周围神经慢性瘢痕增生的抑制作用

目的：研究曲尼司特（Tranilast)对单纯慢性卡压性周围神经病变的作用。方法：按照Bennett方法，将36只大鼠分为两组，比较两组动物患肢热板试验，神经传导速度，组织病理学和微细结构的改变。结果：实验组右下肢热逃避反射潜伏期和神经传导速度较对照组明显变短，对照组坐骨神经为神经周围或神经束间炎性反应，轴索变性，大量的轴索发芽，再生的有髓纤维及神经束再生。实验组有效地预防了早期神经的炎性反应和轴索的变性。结论：Tranilast在大鼠单纯慢性卡压性神经病变模型中有对瘢痕增生抑制和神经功能的保护作用。     

痉挛型脑性瘫痪患儿脊神经背根病理改变与临床意义

目的：研究痉挛型脑性瘫痪（脑瘫）患儿脊神经后根病理改变与临床的关系。方法：选择经临床确诊的痉挛型脑瘫患儿27例，采用选择性脊神经根切断术（SPR）切断L5－S1两侧脊神经后根，对标本进行计算机辅助图象分析处理，测定各种指标与患儿Ashworth肌张力分级的关系。结果：所有脑瘫患儿手术切除的脊神经后根均有不同程度的病理改变，以粗有髓神经纤维病变明显，主要为脱髓鞘病变。无髓神经纤维以其周围的雪旺氏细胞病变为主，神经受损无修复与再生。随鞘厚度、病理改变程度与Ashworth肌张力分级正相关。结论：脊神经后根的病理变化是继发性退行性改变。这种改变与Ashworth肌张力分级有关。     

遗传性痉挛性截瘫的临床表现与基因诊断策略

遗传性痉挛性截瘫(hereditary spastic paraplegia,HSP),又称Strümpell-Lorrain病,是一种具有临床及遗传异质性的神经系统遗传病。HSP的病理改变主要表现为脊髓中双侧皮质脊髓束的轴索变性和(或)脱髓鞘,且以胸段病变明显;临床表现为缓慢进展的双下肢无力及痉挛性截瘫。根据     

痉挛性脑瘫肌肉的超微结构研究

目的　探讨痉挛性脑瘫骨骼肌纤维的病理改变特点。方法　取材于 2 5例痉挛性脑瘫的骨骼肌标本 5 4个 ,通过透射电镜观察其亚显微结构改变。结果　大体观察基本正常。光镜下有集簇性肌纤维变性、结缔组织增生 ,肌梭结构尚好。电镜下肌纤维线粒体多 ,三联管结构少、发育差 ;病变肌纤维呈细胞水肿和肌原纤维破坏的表现 ;部分肌纤维中可见肌原纤维旋涡样变结构。结论　痉挛性脑瘫的骨骼肌存在着继发性病变 ,Ⅰ型肌纤维的亚显微结构表现明显。     

周围神经侧侧缝合治疗痉挛性脑性瘫痪的初步观察

Spasmiccerebralpalsyisoneoftheseriousdiseasesofthenervessystemthatseverelyaffectsgrowingdevelopmentandlimbfunctions．Softtissuerelaxion，selectivespinalnerverootperiph－eralnervebranchamputationorandtendontranspositionarecom－montherapiesatpresent．Postoperativerecurrenceistheshort－comingofsofttissueoperation．Spinalnerverootaputatincouldeffectivelyrelievelimbspasmwhilecontroversyexistsinitslong－termtherapeuticeffect，andtoomuchamputationresultsinweakmuscularforcewhichmightcauseinjuresthatco…     

The treatment of spastic cerebral palsy by side—to—side neurorrhaphy of perpheral nerves

Objective :To discuss a new method and its mechanism for the treatment of spastic cerebral palsy.Methods:6 case were treated.The injurious nerve trunk was kept abreast of neithbor donor nerve at suitable segement,the epineurium and fascicu of two neighboring area were incised to appear nerve fibers,then side-to-side anastomosed each other through the epineurium and fascicu.Result All cases were followed-up for 4-15 months.The spastic limb and deformity of all cases have obvious relieve,5 cases had no spasm without extra stimulation and have the main function recovered,Conclusion Side-to-side neruorrhaphy is a new method to treat spastic cerebral palsy ,After operation,the spatstic muscle could obtain normal never control,thereby,the cervical orientation area was changed.     

The effect of rhizotomy on movement in patients with cerebral palsy

Selective posterior lumbar rhizotomy has recently become an alternative method for relieving spasticity in patients with spastic cerebral palsy. This procedure involves the selective sectioning of the lumbosacral posterior nerve rootlets. Because the whole nerve root is not cut, tactile and proprioceptive sensation remain intact. The present study measured the changes that occurred in 29 patients with spastic cerebral palsy 2 days before surgery and from 4 to 14 months after surgery. Each patient was used as his or her own control. In addition to the clinical evaluation of function, gait was analyzed on appropriate patients with a simple digital camera and microcomputer. The results indicated positive gains after rhizotomy. These gains were, however, dependent on the patients' abilities before surgery. The most significant improvement occurred in thigh and knee ranges of movement. This somewhat controversial new method of treating spasticity shows promise in improving the quality of life of patients with spastic cerebral palsy and in facilitating their treatment.     

Ultrastructural pathology of peripheral nerves in patients with diabetic neuropathy

Sural nerve lesions in patients with clinically manifest diabetic neuropathy were investigated electron microscopically. Myelinated nerve fibers were reduced in all the diabetic patients. Axonal degeneration of both myelinated and unmyelinated nerve fibers was most conspicuous finding in the diabetic sural nerves. Structural changes of the axons were represented by axonal dwindling, depletion of axoplasmic organelles, vacuolarization and an increase in neurofilaments. Accumulation of glycogen-like particles and deposition of electron homogeneous amorphous materials were noted within a few axons. On the other hand, there could also be found degenerative changes of myelin sheaths, various kinds of cytoplasmic inclusion bodies (crystalloid, lamellar inclusion bodies and lipids-like droplets), aggregates of glycogen particles in the Schwann cell cytoplasm and basement membrane hyperplasia of Schwann cells in all the subjects. Furthermore, multiplication and thickening of the basement membrane of vasa nervorum were constant findings of the diabetic sural nerves. The vascular changes, demyelination and axonal degeneration of the cases were not apparently correlated with each other. There was no special relationship between nerve tissue changes and clinical symptoms or laboratory findings. These results indicated that the peripheral nerve lesions in human diabetics were mainly due to metabolic impairment of nerve fibers, accompanying dysmetabolism of Schwann cells and diabetic microangiopathy, and that these changes proceeded independently.     

脑瘫患儿周围神经显微缩小术后肌力恢复研究

目的观察肌兴奋治疗仪在脑瘫患儿下肢周围神经显微缩小术后康复治疗中的作用。方法将 77例接受周围神经显微缩小术治疗的下肢痉挛脑瘫患儿术后分为治疗组 (3 9例 )和对照组 (3 8例 ) ,前者在常规康复训练的基础上加用肌兴奋治疗仪治疗 ,后者仅进行常规康复训练。于肌兴奋治疗仪治疗前、治疗 1个疗程及术后 3个月、6个月时 ,分别对 2组患儿相应肌肉平均肌力进行评定 ,同时评定 2组患儿术后 3个月、6个月的运动功能改善情况 ,并进行组间比较。结果治疗组患者肌力提高和运动功能改善情况均明显好于对照组 (P <0 .0 1)。结论脑瘫患儿下肢周围神经显微缩小术术后 ,在康复训练的同时应用肌兴奋治疗仪可加快肌力恢复和运动功能改善。     

Noninvasive and Painless Magnetic Stimulation of Nerves Improved Brain Motor Function and Mobility in a Cerebral Palsy Case

Motor deficits in cerebral palsy disturb functional independence. This study tested whether noninvasive and painless repetitive peripheral magnetic stimulation could improve motor function in a 7-year-old boy with spastic hemiparetic cerebral palsy. Stimulation was applied over different nerves of the lower limbs for 5 sessions. We measured the concurrent aftereffects of this intervention on ankle motor control, gait (walking velocity, stride length, cadence, cycle duration), and function of brain motor pathways. We observed a decrease of ankle plantar flexors resistance to stretch, an increase of active dorsiflexion range of movement, and improvements of corticospinal control of ankle dorsiflexors. Joint mobility changes were still present 15 days after the end of stimulation, when all gait parameters were also improved. Resistance to stretch was still lower than prestimulation values 45 days after the end of stimulation. This case illustrates the sustained effects of repetitive peripheral magnetic stimulation on brain plasticity, motor function, and gait. It suggests a potential impact for physical rehabilitation in cerebral palsy.     

酚阻滞术治疗痉挛型脑瘫的长期疗效分析

目的 探讨酚阻滞术缓解脑瘫患儿痉挛的长期疗效。方法 42例年龄为2一17岁的痉挛型脑瘫患儿应用5％的酚溶液进行74次阻滞，酚用量0.5-4.6ml/次，平均2.2ml。采用修改的Ashworth评分法评定疗效。结果 66次酚阻滞术取得成功，患儿肌张力有所下降，运动功能有所提高，有效率为89.2%，显效率为59.5%，且副作用少。结论 酚阻滞术有缓解痉挛见效快，选择性强、操作简单、安全、费用低廉等优点。