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.     

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.     

Significance of various systemic and ocular parameters in the long-term prognosis after diabetic vitrectomy

The presence or absence of retinal detachment, especially detachment of the macula, was the most critical parameter predicting visual outcome, and long duration of detachment worsened the prognosis (P=0.0084). In contrast, the time interval from vitreous haemorrhage to surgery did not associate with the long-term visual outcome. Pre-operative visual acuity of counting fingers 1 m or better was associated with favourable visual outcome (P=0.0032). Other pre- or per-operative parameters (e.g. aphakia, iris rubeosis, extent of photocoagulation, retinal breaks) did not associate with visual prognosis significantly.Significant association between general parameters and visual outcome was found only in the group of traction retinal detachment where loss of vision was related to shorter duration of diabetes (P=0.0213), and shorter duration of background retinopathy (P=0.0300), and proliferative retinopathy (P=0.0072).     

准分子激光治疗近视亚临床视网膜脱离11例

目的 探讨亚临床视网膜脱离(subclinical retinal detachment,SCRD)的诊治及临床意义。方法 对预施PRK及LASICK术之前扩瞳三面镜查出有SCRD的患者随访6～15月,详细记录其诊治过程及预后。结果 共查1088眼,11眼伴有SCRD,占1.01％,随访8例患者均为单眼,其中正视眼1例,中低度近视2例,高度近视5例;采用保守治疗6例,其中2例网膜复位,4例视网膜脱离仍存;采用手术治疗2例,网膜复位;选择PRK 2例,1例行LASIK。结论 对于预施PRK及LASIK术的患者应详细检查眼底。一旦发现伴有SCRD,应及时采用保守治疗,定期随访,尽量避免网膜脱离复位手术。如需行近视手术应考虑手术的方法与时机。     

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.     

PARS INTERARTICULARIS STRESS AND DISC DEGENERATION IN CRICKET'S POTENT STRIKE FORCE: THE FAST BOWLER

Cricket fast bowlers are the potent strike force in a multidiscipline team. They subject their spines to repetitive sagittal plane and rotatory movements over many years. The effect of this repetitive stress has not previously been analysed. This study examined 20 former fast bowlers to determine the incidence of spondylolysis, spondylolisthesis and degenerative change. Fast bowlers are noted to have an increased incidence of spondylolysis. A mixed front/side bowling style involving more lumbar hyperextension or rotation has significant association with spondylolysis when compared with side-on bowling styles. There was a high incidence of radiological thoracolumbar degenerative facet joint and disc disease in former fast bowlers.     

Dopamine transporters, D2 receptors, and glucose metabolism in corticobasal degeneration.

Alterations in presynaptic and postsynaptic dopaminergic system and cerebral glucose metabolism in corticobasal degeneration (CBD) were assessed to evaluate the potential usefulness of different imaging methods for CBD. (123)I-FP-CIT/(123)I-beta-CIT SPECT and (123)I-IBZM SPECT as well as (18)F-FDG PET were performed in eight CBD patients. Decreased presynaptic dopamine transporter binding was found in all CBD patients while D2 receptor binding was reduced in only one patient. (18)F-FDG PET displayed a contralateral hypometabolism in cortical and subcortical areas in seven out of eight patients. Our results demonstrate that glucose metabolism and DAT are reduced, while D2 receptors may be frequently preserved in CBD.     

Macrophage-like cells from explant cultures of rat sciatic nerve produce apolipoprotein E

Apolipoprotein E is synthesized and secreted by degenerating peripheral nerve, but the role of resident endoneurial cells in this process is not clear. To exclude the involvement of nonresident cells, we examined the cellular source of endoneurial apolipoprotein E in explant cultures of rat sciatic nerve. The cellular outgrowth from these explant cultures released apolipoprotein E into the culture medium. The cellular outgrowth contained fibroblasts, Schwann cells, and a population of cells with many phenotypic characteristics of macrophages, including the production of apolipoprotein E. No other cell type in the cultures appeared to contribute to this production. These data suggest that apolipoprotein E is produced by resident endoneurial cells in explant cultures and that these cells are macrophages.