Alterations in short-term retention, conditioned avoidance response acquisition and motivation following aluminum induced neurofibrillary degeneration

Aluminum chloride induced neurofibrillary degeneration may provide a useful model for the study of a human dementia process. This possibility was assessed in cats trained to perform on a delayed-response task, a conditioned avoidance task, visual and temporal discrimination tasks and a motivational task involving rewarding intracranial electrical stimulation. After an initial asymptomatic period short term retention and acquisition of a conditioned avoidance response were selectively impaired. The associated ultrastructural abnormalities plausibly implicate the cytoplasmic streaming mechanism in the cellular substrate for some retention and acquisition phenomena.     

Non-functional adrenocortical adenoma with extensive degeneration

We report a case of non-functional adrenocortical adenoma of 5.5 x 5.5 x 3.2 cm in size that had an unusual histopathological appearance in two respects. First, the tumor contained small adipose foci with osteogenesis and was suspected of being a myelolipoma based on its appearance on computerized tomography (CT) and magnetic resonance imaging. However, pathologically, the fat element was seen focally and was not accompanied by hematopoietic cells, and the diagnosis of myelolipoma was abandoned. Second, the tumor was suspected of being an adrenal carcinoma based on its appearance on CT scans and showed extensive degeneration: fibrosis, hemorrhage, loss of parenchyma and moderate atypism of the tumor cells. However, as the architecture of the tumor cells was non-diffuse and there were no necrotic foci or mitoses, and vascular or capsular invasion were not present, the tumor was concluded to be an adrenocortical adenoma rather than a carcinoma. We diagnosed the tumor as a non-functional adrenocortical adenoma with extensive degeneration as the extensive areas of fibrosis were particularly remarkable. Furthermore, the extensive areas of degeneration might have been caused not only by an ischemic effect but also by low hormone levels.     

Age-dependent occurrence of an ascending axon on the omega neuron of the cricket, Teleogryllus oceanicus

The omega neurons (ON1s) are a mirror-symmetrical pair of identified prothoracic auditory interneurons of crickets which have been previously described as intraganglionic. Using intracellular techniques we stained ON1s of female Teleogryllus oceanicus and found that many ON1s have axons which project anteriorly out of the prothoracic ganglion. The ascending axon arises contralateral to the soma at the most anteriolateral bend of the bow-shaped process of an otherwise "archetypical" ON1 and travels up the neck connective in a ventral position just inside the connective tissue sheath. The occurrence of the ascending axon is age-dependent. Seventy-five percent of ON1s stained in late nymphal stages and in young adults had an ascending axon while only 30% of ON1s in older adults had an ascending axon. Evidence is presented to show that ON1s having ascending axons are developmental variants of the "archetypical" ON1 and do not represent a separate neuron type. The two morphological types of ON1s are not distinguishable on the basis of their responses to sound stimuli having carrier frequencies of 3.5-60 kHz. Although we know that the ascending axon conducts action potentials, its target and terminal morphology are not yet known.     

The topography of expanded uncrossed retinal projections following neonatal enucleation of one eye: differing effects in dorsal lateral geniculate nucleus and superior colliculus

The topographic organization of the uncrossed retinal projections to the dorsal lateral geniculate nucleus (dLGN) and superior colliculus (SC) was studied in normal adult hooded rats and in rats subjected to unilateral ocular enucleation on the day of birth. Sections were stained for anterograde degeneration products following discrete retinal lesions at various locations. The projection from the temporal crescent to the dLGN in neonatally enucleated rats had an expanded but topographically normal organization, with the nasotemporal and dorsoventral retinal axes displaying polarities identical to those in normal adults. Neonatal enucleation permits the remaining uncrossed retinogeniculate projection to extend primarily along the "lines of projection" into neuropil normally recipient of binocularly conjugate crossed projections. In the SC, the dorsoventral axis of the temporal crescent showed a normal polarity, but the nasotemporal axis failed to display any topographic organization. Retinal loci in the temporal crescent projected throughout the rostrocaudal extent of the ipsilateral SC. Retinal lesions placed outside the temporal crescent failed to produce any substantial degeneration in ipsilateral dLGN or SC. These topographically distinct effects in dLGN and SC following unilateral eye removal on the day of birth are discussed in the context of differing constraints upon axonal ingrowth and connectivity during early development, which may normally bring about the characteristically distinct features of retinogeniculate and retinocollicular organization.     

Electromyography and nerve conduction study in autosomal dominant olivopontocerebellar atrophy

Summary Electromyographic examination and studies of motor and sensory conduction velocities were performed in 11 patients with a presumptive diagnosis of olivopontocerebellar atrophy with autosomal dominant transmission. Peripheral nervous system involvement was shown in eight. In two patients with early onset of disease, electrophysiological alterations clearly pointed to severe axonal degeneration, whereas in six they were compatible with slight demyelination.

---

Zusammenfassung Bei elf Patienten, bei welchen eine olivopontocerebelläre Atrophie mit autosomal dominanter Übertragung angenommen wurde, führten wir eine elektromyographische Untersuchung und eine Bestimmung der motorischen und sensiblen Erregungsleitungsgeschwindigkeit durch. Bei acht dieser Patienten wurde eine Mitbeteiligung des peripheren Nervensystems nachgewiesen. In zwei Fällen mit frühem Krankheitsbeginn wiesen die elektrophysiologischen Veränderungen eindeutig auf eine schwere axonale Degeneration hin, während bei sechs die Befunde mit einer leichten Demyelinisation vereinbar waren.

     

A Randomized Study of Nutritional Supplementation in Patients with Unilateral Wet Age-Related Macular Degeneration

The purpose of this study is evaluate the efficacy and safety of medicinal products containing the original Age-Related Eye Disease group (AREDS) formulation at doses approved in Europe (EU, control group; n = 59) with a product that adds DHA, lutein, zeaxanthin, resveratrol and hydroxytyrosol to the formula (intervention group; n = 50). This was a multicenter, randomized, observer-blinded trial conducted in patients aged 50 years or older diagnosed with unilateral exudative Age related Macular Degeneration AMD. At month 12, the intervention did not have a significant differential effect on visual acuity compared with the control group, with an estimated treatment difference in Early Treatment Diabetic Retinopathy Study (ETDRS) of −1.63 (95% CI −0.83 to 4.09; p = 0.192). The intervention exhibited a significant and, in most cases, relevant effect in terms of a reduction in some inflammatory cytokines and a greater improvement in the fatty acid profile and serum lutein and zeaxantin concentration. In patients with unilateral wet AMD, the addition of lutein, zeaxanthin, resveratrol, hydroxytyrosol and DHA to the AREDS EU recommended doses in the short-term did not have a differential effect on visual acuity compared to a standard AREDS EU formula but, in addition to improving the fatty acid profile and increasing carotenoid serum levels, may provide a beneficial effect in improving the proinflammatory and proangiogenic profile of patients with AMD.     

亚急性联合变性的临床分析

目的探讨亚急性联合变性(SCD)的临床特点、发病机制及电生理和磁共振成像的诊断价值.方法对14例SCD患者的临床资料进行回顾性分析.结果发现所有SCD患者发病均由维生素B12缺乏所引起.肢体感觉异常,深感觉减退,共济失调及痉挛性轻瘫是SCD常见的症状和体征.该病早期易误诊,电生理检查有极高的敏感性,磁共振成像可以确定脱髓鞘的部位.结论血清维生素B12浓度测定,体感诱发电位及磁共振对诊断和治疗有重要作用.     

肝豆状核变性分子生物学研究

【目的】探讨中国人肝豆状核变性(WD)的分子发病机制和基因诊断的方法。【方法】应用基因工程技术对WD进行了10年的分子生物学研究。【结果】①WD的基因定位研究通过RFLP及微卫星多态性分析,应用两位点及多位点连锁软件,建立了中国人WD基因在D     

长链非编码RNA KLHL7-DT对人退变髓核细胞增殖和凋亡的影响及其相关机制

目的 探讨长链非编码RNA KLHL7-DT对人退变髓核细胞增殖和凋亡的影响及其相关机制。方法 选取2018年1月—2019年10月南京江北医院骨科脊柱骨折患者手术切除的正常椎间盘标本18例,其中男11例、女7例,年龄为22~46 （38.3±4.3）岁,PfirrmannⅠ级6例、Ⅱ级12例。取椎间盘标本常规分离、培养髓核细胞,使用10 ng/mL IL-1β处理髓核细胞获得退变髓核细胞。将退变髓核细胞分为沉默对照组、KLHL7-DT沉默组、过表达对照组、KLHL7-DT过表达组。4组细胞分别对应转染沉默对照序列、siRNA-KLHL7-DT沉默序列、过表达对照序列、KLHL7-DT过表达序列。取转染后4组退变髓核细胞采用5-乙炔基-2’脱氧尿嘧啶核苷（EdU）法检测细胞增殖情况,流式细胞术检测细胞凋亡情况,Western blot检测聚集蛋白聚糖（Aggrecan）、Ⅱ型胶原（Col Ⅱ）蛋白的表达情况。结果 （1）KLHL7-DT过表达组EdU染色阳性细胞/DAPI染色阳性比值（0.147±0.002）低于过表达对照组（0.203±0.007）,而KLHL7-DT沉默组比值（0.428±0.050）高于沉默对照组（0.240±0.032）,差异均有统计学意义（t=14.25、-5.44,P值均<0.05）。（2）KLHL7-DT过表达组细胞凋亡率（19.01%±0.41%）高于过表达对照组（14.38%±0.31%）,KLHL7-DT沉默组细胞凋亡率（16.08%±0.59%）低于沉默对照组（17.42%±0.36%）,差异均有统计学意义（t=15.69、3.36,P值均<0.05）。（3）Western blot结果显示,KLHL7-DT过表达组细胞Aggrecan和Col Ⅱ蛋白的相对表达量（分别为0.34±0.29、0.57±0.11）均低于过表达对照组（1.00±0.22、1.05±0.10）,KLHL7-DT沉默组Aggrecan和Col Ⅱ蛋白的相对表达量（分别为1.77±0.14、1.63±0.12）均高于沉默对照组（1.10±0.18、0.98±0.08）,差异均有统计学意义（t=3.10、5.54、-5.05、-7.66,P值均<0.05）。结论 上调KLHL7-DT的表达可抑制退变髓核细胞的增殖,促进退变髓核细胞的凋亡,其机制可能是通过调节细胞外基质Aggrecan、Col Ⅱ蛋白的合成,进而参与椎间盘退变的发展。     

化痰祛瘀法治疗肝豆状核变性研究进展

肝豆状核变性(WD)在西医学上是铜代谢障碍导致全身多器官损害等为主要临床特征的常染色体隐性单基因遗传疾病,病变影响肝、脑、肾、角膜等重要脏器。中医通过对WD患者临床症状及病理改变的认识,认为WD的基本病理因素为痰浊、瘀血,二者相互作用而发病,以痰瘀互结为主要的病机,贯穿疾病发展始终,故而运用化痰祛瘀法治疗WD在临床中常见。众多研究表明运用中药汤剂、中医外治法化痰祛瘀治疗可以有效缓解WD患者临床症状,发挥排铜优势、改善肝纤维化、改善脑流动力学、抑制炎症和抗氧化应激作用,进而延缓疾病进程,控制疾病发展。现结合新近研究成果,对化痰祛瘀法在WD的临床应用予以综述,以期为临床治疗WD提供中西医结合治疗有效的方案及依据。