Sub‐retinal neovascular membrane in exudative macular degeneration

Age‐related macular degeneration is a major cause of serious vision loss. The earliest stages of age‐related maculopathy may be defined by the size of the drusen present in the macula and the effects on vision. Further manifestations may include soft drusen, choroidal neovascularisation, macular haemorrhage and cicatricial or disciform degeneration of the macula. This report describes a patient with a macular haemorrhage, a choroidal neovascular membrane and serious loss of vision. In addition, the pathogenesis, diagnosis and treat‐ment options of macular degeneration are reviewed.     

MR Findings of Intrathecal Chemotherapy-Related Myelopathy in Two Cases: Mimicker of Subacute Combined Degeneration

We report the findings of spinal magnetic resonance imaging (MRI) in 2 patients who had undergone intrathecal chemotherapy and presented with the subacute onset of ascending numbness and weakness. MRI revealed a symmetric hyperintensity at the posterior columns of the spinal cord from the lower cervical region down to the conus medullaris level on T2-weighted images, and no abnormal enhancement. The imaging findings are similar to those seen in subacute combined degeneration (SCD), but the serum vitamin B(12) levels were normal in these 2 cases.     

THE CLINICAL AND NEURO-ELECTROPHYSIOLOGIC STUDY ON CORTICOBASAL DEGENERATION

目的：探讨皮质基底节变性(CBD)患者的临床和神经电生理特征。方法：对29例临床诊断为CBD患者，系统分析其临床症状、体征、常规化验检查、脑CT／MRI、SPECT以及神经电生理改变，并与其它疾病相对比。结果：结果显示，29例患者均出现不对称性帕金森样表现，皮层功能受损占79．3％，其它功能障碍占37．9％。21例患者脑CT或MRI显示不对称性脑萎缩，SPECT显示额顶区低灌注表现，以受损肢体的功能区较明显。EEG显示19例患者出现不对称性慢波，伴有肌阵挛的15例病人，表面肌电图显示患肢随意运动时可诱发巨大电位，皮层反应增强，SEP无特殊变化，运动诱发电位检查，26例患者中枢运动传导时间正常。结论：CBD是一种累及皮层和基底节、病程以缓慢进展为特征的变性性疾病。大部分患者可出现帕金森综合征样表现，皮层及其他运动功能障碍。脑影象学显示额顶叶萎缩。神经电生理检查表现为皮层兴奋性增强，而锥体束传导功能正常。     

Tibiotalocalcaneal arthrodesis with retrograde intramedullary nailing

Nineteen patients (20 feet) with severe hindfoot and ankle deformity underwent tibiotalocalcaneal fusion with a retrograde locked intramedullary nail as a limb-salvage procedure. The purpose of this study was to compare the complication rates of this procedure in diabetic versus nondiabetic patients. There were 8 men and 11 women with preoperative diagnoses including Charcot neuroarthropathy, primary osteoarthritis, rheumatoid arthritis, equinocavovarus, posttraumatic osteoarthritis, gouty arthritis, and ankle malunion. Ten of 20 procedures were performed in patients with diabetes. The average patient age was 56 years, and the average postoperative follow-up was 19.8 months. Nineteen of 20 ankles (95%) achieved successful fusion with an average time of 4.1 months. Four patients (21%) required either a fracture brace or an ankle foot orthosis at final follow-up. Five patients (25%) had major complications and 11 patients had minor complications. Major complications included osteomyelitis (n = 2), Charcot arthropathy (n = 2), failure of fixation (n =1), soft-tissue necrosis (n = 1), cardiac arrest (n = 1), cerebral vascular accident (n = 1), and fatal pulmonary embolus (n = 1). All patients with major complications were diabetic, and 14 of 20 combined major and minor complications occurred in patients with diabetes. The complication rate was found to be high in diabetic patients with end-stage deformity undergoing a limb salvage     

Glial localization of four‐repeat tau in atypical progressive supranuclear palsy

In the present case, a patient in whom limb apraxia and asymmetrical parkinsonism developed suggesting corticobasal degeneration, is reported. Neuropathologic examination revealed numerous tufted astrocytes in the precentral cortex in addition to the characteristic pathologic findings of PSP. Therefore, on the basis of clinicopathologic features, atypical progressive supranuclear palsy was diagnosed. In addition, the brain tissue of the present patient was investigated with an antibody specific for four‐repeat tau (4R‐tau). In the precentral cortex, numerous tau‐positive tufted astrocytes, pretangles, and threads were positive for 4R‐tau. Using a confocal microscopy we demonstrated that tufted astrocytes positive for 4R‐tau were adjacent to astrocytes positive for GFAP. The present findings suggest that accumulation of four‐repeat tau in astrocytes is a degenerative process rather than a reactive process.     

Treatment of pigment epithelial detachments due to age-related macular degeneration with intra-ocular C3F8 injection*

Purpose: To flatten pigment epithelial detachments (PED) cue to age-related macular degeneration in an attempt to visualize the underlying choroidal neovascularization by fluorescein angiography (FA) and reveal a treatment target. Methods: Nine patients with PED received intravitreal gas injections via the pars plana and postured face down. Fluorescein angiograms were obtained before and after gas injection. In two patients, retinal scotopic sensitivity was also measured. Results: Eight patients demonstrated change in the shape and size of the PED following gas injection. Four patients showed a better delineation of underlying structures on FA. Three patients had focal laser treatment to the newly visible choroidal neovascular complex, but this was successful in only one patient with flattening of the PED. Conclusion: Pigment epithelial detachments can be modified by intravitreal gas injection in some patients, but this treatment did not have a major impact on overall outcome or management.     

Deficient activation of microglia during optic nerve degeneration

Transection of an optic nerve (ON) is followed by slow removal of myelin. We studied microglia for the expression of molecules that characterize activated myelin phagocytosing macrophages: MAC-1, FcγII/III receptor (FcR), MAC-2, and F4/80. In-vitro, microglia expressed all molecules and phagocytosed myelin. In-vivo, intact ON displayed high levels of MAC-1, little FcR and F4/80, and no MAC-2. The expression of these molecules was upregulated differentially in in-vivo degenerating ON: MAC-1 uniformly, FcR and F4/80 variably, and MAC-2 sporadically. The distribution of MAC-2 expression correlated best with a pattern of sporadic structural degeneration. Thus in-vivo, ON injury is followed by deficient microglia activation, which we suggest contributes significantly to the slow clearance of myelin.     

Type classification of anomalous pancreaticobiliary junction by ERCP

The role of endoscopic retrograde cholangiopancreatography (ERCP) in the preoperative assessment of anomalous pancreaticobiliary junction was retrospectively evaluated in 74 consecutive patients (19 males and 55 females; aged 0–80 years). Sixty-three patients had congenital biliary dilatation and 11 did not. Type classification of congenital biliary dilatation was possible by ERCP alone in 45 patients (71%). The main causes of classification failure were previous bilio-enteric anastomosis and restriction of postural changes during ERCP due to general anesthesia in pediatric patients. Classification of anomalous junction was possible in 69 patients (93%). Technical difficulty in ERCP caused classification failure in 5 patients. Neoplastic lesions were found in 12 patients (16%) and all but 1 were correctly diagnosed by ERCP. We conclude that ERCP plays an important role in the preoperative diagnosis and type classification of anomalous pancreaticobiliary junction and congenital biliary dilatation.     

老年黄斑变性的视网膜色素上皮及其视功能的改变

目的：应用黄斑区密集点矩阵视野并结合眼底荧光血管造影电脑眼底图像分析老年黄斑变性视网膜色素上皮损害与ＤＭＭＭ的视野光敏感度的改变，找出改变的规律有评价其应用价值。方法：用Ｈｕｍｐｈｒｅｙ－６４０视野计自行设计ＤＭＭＭ检测ＡＭＤ干性组５１人６４只眼湿性且２３人２７只眼，用Ｚｅｉｓｓ公司生产的ＫＯＮＴＲＯＮＥＬＥＫＴＲＯＮＩＫ Ｉｎａｇｅ Ａｎａｌｙｓｉｓ Ｄｉｖｉｓｉｏｎ－计算机图像分析仪总丢失量显     

Proteinuria and other renal functions in Wilson’s disease

Renal lesions have repeatedly been described in Wilson’s disease (WD). We investigated the excretion of total protein, albumin, low (LMW) and high molecular weight (HMW) proteins, N-acetyl-β-D-glucosaminidase (NAG), and calcium, as well as creatinine clearance, in 24-h urine samples of 41 patients with WD aged 6 – 37 (mean 17) years who had been treated for a period of 0 – 15 (mean 4.5) years with D-penicillamine (900 mg/day). The amount of all protein excreted was significantly increased compared with controls, 39% of patients presenting with total proteinuria more than two standard deviations from the mean of controls. The changes in protein excretion depended on the duration of treatment. LMW proteinuria was elevated almost exclusively in the first 2 years after the start of treatment, indicating early tubular damage. This is supported by an initially high excretion of β₂-microglobulin, NAG, and calcium. Increased excretion of HMW proteins, including albumin, persisted over longer periods, which suggests glomerular injury in some patients, possibly related to the use of D-penicillamine. Creatinine clearance remained roughly within normal limits. We propose that renal function should regularly be checked in patients with WD. Received October 26, 1995; received in revised form August 27, 1996; accepted September 20, 1996