Presenting symptoms and natural history of Wilson disease

The presenting symptoms of Wilson disease and its natural history as related to age are described based on 283 cases collected in Japan. The disease presented with a variety of signs and symptoms; the most frequent were in order of frequency jaundice, dysarthria, clumsiness, tremor, drooling, gait disturbance, malaise and arthralgia. The mean age at onset of the disease was 12.0 years. Hepatic and osteoarthral symptoms developed early and neurological symptoms late. Fifty-eight cases develolped neurological symptoms only, 28 cases had hepatic symptoms only, and in 26 cases hepatic mortality rate was observed in hepatic, hepato-haematological and hepato-renal cases mainly due to acute hepatic failure resulting in death only a few weeks after onset. Cases having only neurological symptoms showed a more favourable prognosis with a longer survival.     

Gd-DTPA-enhanced MR in thoracic disc herniations

Summary The Gd-DTPA-enhanced magnetic resonance findings in two patients with herniated thoracic intervertebral discs are reported. The first patient was a 56-year-old woman with a small subligamentous T6-7 disc herniation, slightly lateralized to the right. The second patient was a 51-year-old man with a central and right posterolateral disc herniation, including a large calcified fragment, at the T8-9 level. The nonenhanced MR examination revealed the presence of an extradural mass lesion in both patients, impinging upon the dural sac and compressing and displacing the spinal cord posteriorly. The lesion was slightly hypointense on both T1- and T2-weighted spin echo sequences. Following intravenous injection of Gd-DTPA in a dosage of 0.1 mmol/kg body weight, enhancement of the posterior longitudinal ligament was noted and triangular areas of contrast uptake were seen to occur in the epidural space above and below the herniated disc. At surgery, they were found to correspond to dilated and congested epidural veins.     

MRI in subacute combined degeneration

We describe a patient with clear lesions in the spinal cord on MRI due to subacute combined degeneration. T2-weighted images clearly showed abnormal high signals in the posterior columns, which disappeared on recovery from the disease.     

超声引导胶原酶治疗外侧型腰椎间盘突出症

目的探讨超声引导胶原酶治疗外侧型腰椎间盘突出症的疗效及安全性.方法在相应椎间盘突出的棘突间,旁开1.5～2.5cm处经皮穿刺,采用超声引导定位,穿刺成功后,先进行加压试验,再进行麻醉试验,无腰麻征象后,将胶原酶注入到突出的椎间盘内或周围.结果 231例病人出院时的优良率为92.21%,有效率为99.57%;随访1～5年的优良率为92.65%,有效率为99.58%.近期与远期疗效比较无显著性差异(χ2=0.129,P＞0.05).结论利用超声引导监测、定位,注射胶原酶治疗外侧型腰椎间盘突出症,安全性强、有效率高.     

经关节突入路椎间融合椎弓根固定治疗峡部裂性腰椎滑脱

目的 评价经关节突入路手术治疗峡部裂性腰椎滑脱的应用价值。方法 2000年 9月 ~2003年 5月我院手术治疗峡部裂性腰椎滑脱患者 83例,随访患者 81例,年龄(55. 25±19. 21)岁,随访时间 (1. 68±0. 93)年。按照不同的手术入路方法分为两组,Ⅰ组经关节突入路,椎间融合椎弓根固定;Ⅱ组经椎板切除入路,椎间融合椎弓根固定。按各组术中的临床出血量、手术时间、术前和术后JOA评分等数据经SPSS11. 5统计软件处理数据。结果　术后采用Stauffer Coventry下腰椎术后疗效评定标准:临床优良率分别为 87%与 75%;术后神经功能改善率分别为 87. 81%与62. 88%。术后均无螺钉松动、断裂或者滑脱加重等并发症,有 3例假关节形成。结论　在峡部裂性腰椎滑脱的患者的手术治疗中,经关节突入路椎间融合椎弓根固定技术具有良好的临床效果。     

小切口椎板间开窗治疗腰椎间盘突出症

目的　探讨小切口椎板间开窗髓核摘除术的疗效及适应证。方法　从 1998年 5月～ 2 0 0 1年 9月采用小切口椎板间开窗髓核摘除术治疗腰椎间盘突出症 72例。全部患者都有腰痛和一侧或双侧下肢疼痛及麻木。结果　获得随访 65例 ,随访时间最长 2 6个月 ,最短 6个月 ,平均 13个月 ,按Nakai标准评定治疗优良率为 93 .8%。结论　小切口椎盘间开窗髓核摘除术具有适应证较广、手术时间短、出血少、创伤小、手术优良率高、后期并发症少、不损害脊柱稳定性等优点 ,是治疗腰椎间盘突出症较为有效和可靠方法之一     

腰椎间盘突出症和马尾神经综合征

本文报告３２例腰椎间盘突出症导致的马尾神经综合征。除腰痛及坐骨神经痛外，本组所有病人均存在鞍区麻木和括约肌功能障碍。根据尿便功能障碍发作程度和持续时间，本组有四种不同的临床表现类型，预后差。异较大。脊髓造影具有诊断和鉴别诊断价值，应用Ｏｍｎｉｐａａｕｅ较Ｃｏｎｒａｙ安全。早期诊断和手术治疗，对括约肌功能、肌力和鞍区浅感觉最大程度地恢复甚为重要。     

ULTRASTRUCTURAL INVESTIGATION ON THE ARTICULAR CARTILAGE FROM RABBIT KNEE JOINT AFTER IMMOBILIZATION IN CAST

The articular cartilages from the left knee of 30 rabbits immobilized in casts in functional position were studied under transmission electron microscope. The pathological changes which begin at early stage of immobilization included both degeneration and repair of cartilage. The striking morphological features observed were: (1) most chondrocytes in various layers of cartilage underwent progresive degeneration; (2) the concomitant abnormal proliferation of some chondrocytes happened in a brief period. The changes in mechanical stress caused by immobilization affected the nutrition of cartilage and finally led to its reactive structural reformation.     

An electron microscopic study of local anesthetic-induced skeletal muscle fiber degeneration and regeneration in the monkey

An electron microscopic study was done on abductor pollicis brevis muscles of 18 Rhesus monkeys after intramuscular injections of 0.75% bupivacaine, 2% mepivacaine, or 2% lidocaine + epinephrine. The muscles were examined for from 2 h to 28 days. Severe muscle fiber damage, consisting of breakdown of sarcolemma and myofibrils, was seen as early as 2 h. Phagocyte mediated fragmentation of the degenerating muscle fibers was at its peak during the third and fourth days. Myoblasts were abundant during the fourth day. Early myotubes appeared on the fifth and sixth days, and they matured during the second week. Satellite cells appeared alongside mature myotubes. Overall, the local anesthetic-induced breakdown and regeneration of skeletal muscle fibers in the monkey followed a course quite similar to that seen in the rat.     

Telencephalic projections to the goldfish hypothalamus: An anterograde degeneration study

In this study, large areas of goldfish telencephalon were ablated including rostral nucleus preopticus periventriculare (rNPP), and degenerating axons were traced by a modified Fink and Heimer procedure. The lesioning procedure ablated large regions of area dorsalis telencephali pars medialis, centralis, and dorsolateral complex; and completely removed area ventralis telencephali pars dorsalis, ventralis, and lateralis. In addition, the supracommissural nucleus and rNPP were lesioned specifically because both nuclei have been thought to be involved in courtship behavior and endocrine control of reproduction. This investigation demonstrated extensive fiber projections from telencephalic nuclei and/or rNPP to the hypothalamus. Lesioned telencephalon and/or rNPP projected bilaterally to nucleus preopticus and the suprachiasmatic nucleus and unilaterally to the following tuberal nuclei: nucleus anterior tuberis, and the lateral hypothalamic nucleus. A much larger fiber projection to the inferior lobe nuclei was also observed with a large contralateral as well as ipsilateral input.