人体系统非优性的进化论诠释

从进化论的角度论述了人体系统非优性的成因,说明了生物竿化在人体系统中保留下大量的时空沉迹,其形式与特征表现为层次套叠,多元集合,转化退化。从而成为新与旧,高级与低级,复杂与简单,一句话就是优 与非优相互缠绕,相互作用,互补关联的协同统一性,而疾病的发生则是这种辩证统一的失调或破坏。     

Experiences of patients undergoing repeated intravitreal anti-vascular endothelial growth factor injections for neovascular age-related macular degeneration

Current therapy to slow disease progression in patients with neovascular age-related macular degeneration (AMD) entails regular intravitreal anti-vascular endothelial growth factor (VEGF) injections, often indefinitely. Little is known about the burden imposed on patients by this repetitive treatment schedule and how this can be best managed. The aim of this study was to explore the psychosocial impact of repeated intravitreal injections on patients with neovascular AMD. Forty patients (16 males, 24 females) with neovascular AMD undergoing anti-VEGF treatment were recruited using purposive sampling from a private ophthalmology practice and public hospital in Melbourne. Patients were surveyed using the Macular Disease Treatment Satisfaction Questionnaire (MacTSQ; Bradley, Health Psychology Research Unit, Surrey, England) and underwent semi-structured, one-on-one interviews. Interview topics were: treatment burden and satisfaction; tolerability; barriers to adherence; treatment motivation; and patient education. Interviews were audio recorded and thematic analysis performed using NVivo 10 (QSR International, Doncaster, Australia). Patients recognised the importance of treatment to preserve eyesight, yet experienced significant psychosocial and practical burden from the treatment schedule. Important issues included treatment-related anxiety, financial considerations and transport burden placed on relatives or carers. Many patients were restricted to sedentary activities post-injection owing to treatment side effects. Patients prioritised treatment, often sacrificing family, travel and social commitments owing to a fear of losing eyesight if treatment was not received. Whilst anti-VEGF injections represent the current mainstay of treatment for neovascular AMD, the ongoing treatment protocol imposes significant burden on patients. An understanding of the factors that contribute to the burden of treatment may help inform strategies to lessen its impact and assist patients to better manage the challenges of treatment.     

Quantifier comprehension in corticobasal degeneration

In this study, we investigated patients with focal neurodegenerative diseases to examine a formal linguistic distinction between classes of generalized quantifiers, like "some X" and "less than half of X." Our model of quantifier comprehension proposes that number knowledge is required to understand both first-order and higher-order quantifiers. The present results demonstrate that corticobasal degeneration (CBD) patients, who have number knowledge impairments but little evidence for a deficit understanding other aspects of language, are impaired in their comprehension of quantifiers relative to healthy seniors, Alzheimer's disease (AD) and frontotemporal dementia (FTD) patients [F(3,77)=4.98; p<.005]. Moreover, our model attempts to honor a distinction in complexity between classes of quantifiers such that working memory is required to comprehend higher-order quantifiers. Our results support this distinction by demonstrating that FTD and AD patients, who have working memory limitations, have greater difficulty understanding higher-order quantifiers relative to first-order quantifiers [F(1,77)=124.29; p<.001]. An important implication of these findings is that the meaning of generalized quantifiers appears to involve two dissociable components, number knowledge and working memory, which are supported by distinct brain regions.     

肝豆状核变性（Wilson病）治疗策略的变革及思考

评析肝豆状核变性(WD)当前的治疗方法,主要有低铜饮食,铜的螯合剂青霉胺及曲恩汀,锌制剂和四硫钼酸胺,肝移植。介绍WD治疗策略的变革:不再提倡驱铜疗法以去除堆积的铜,而是使血液中的游离铜离子浓度常态化以逆转铜中毒,并结合2007年《柳叶刀》WD治疗专家共识进行思考。最后,展望未来对WD可能的根治疗法,即基因治疗、肝细胞移植及现在可施行的肝移植。     

A Model-Based Approach to Understanding Apraxia in Corticobasal Syndrome

Limb apraxia is a neurological disorder of higher cognitive function characterized by an inability to perform purposeful skilled movements and not attributable to an elementary sensorimotor dysfunction or comprehension difficulty. Corticobasal Syndrome (CBS) is an akinetic rigid syndrome with asymmetric onset and progression with at least one basal ganglia feature (rigidity, limb dystonia or myoclonus) and one cortical feature (limb apraxia, alien hand syndrome or cortical sensory loss). Even though limb apraxia is highly prevalent in CBS (70–80%), very few studies have examined the performance of CBS patients on praxis measures in detail. This review aims to (1) briefly summarize the clinical, neuroanatomical and pathological findings in CBS, (2) briefly outline what limb apraxia is and how it is assessed, (3) to comprehensively review the literature on limb apraxia in CBS to date and (4) to briefly summarize the literature on other forms of apraxia, such as limb-kinetic apraxia and buccofacial apraxia. Overall, the goal of the review is to bring a model-based perspective to the findings available in the literature to date on limb apraxia in CBS.     

年龄相关性黄斑变性治疗的比较分析

从治疗原理、费用、疗效、不良反应等方面对年龄相关性黄斑变性的不同治疗方法进行比较和分析,为患者进行治疗上的选择提供帮助,同时指导和规范该类疾病治疗的临床决策,以期能在目前条件下不仅提高病人的治疗效果和生活质量同时又达到降低医疗费用的目的。     

Sound naming in neurodegenerative disease

Modern cognitive neuroscientific theories and empirical evidence suggest that brain structures involved in movement may be related to action-related semantic knowledge. To test this hypothesis, we examined the naming of environmental sounds in patients with corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP), two neurodegenerative diseases associated with cognitive and motor deficits. Subjects were presented with 56 environmental sounds: 28 sounds were of objects that required manipulation when producing the sound, and 28 sounds were of objects that required no manipulation. Subjects were asked to provide the name of the object that produced the sound and also complete a sound-picture matching condition. Subjects included 33 individuals from four groups: CBD/PSP, Alzheimer disease, frontotemporal dementia, and normal controls. We hypothesized that CBD/PSP patients would exhibit impaired naming performance compared with controls, but the impairment would be most apparent when naming sounds associated with actions. We also explored neural correlates of naming environmental sounds using voxel-based morphometry (VBM) of brain MRI. As expected, CBD/PSP patients scored lower on environmental sounds naming (p < 0.007) compared with the controls. In particular, the CBD/PSP patients scored the lowest when naming sounds of manipulable objects (p < 0.05), but did not show deficits in naming sounds of non-manipulable objects. VBM analysis across all groups showed that performance in naming sounds of manipulable objects correlated with atrophy in the left pre-motor region, extending from area six to the middle and superior frontal gyrus. These results indicate an association between impairment in the retrieval of action-related names and the motor system, and suggest that difficulty in naming manipulable sounds may be related to atrophy in the pre-motor cortex. Our results support the hypothesis that retrieval of action-related semantic knowledge involves motor regions in the brain.     

颈椎单间隙融合后椎间高度的改变对邻近节段退变的影响

本文探讨颈前路单间隙融合术后该节段的椎间高度改变对相邻节段椎间盘退变所产生的不同影响。将颈前路单节段融合术后的患者78例按融合高度的不同分为三组,通过测量上位节段的椎间高度、活动度及椎体间滑移进行比较,结果显示融合后的椎间高度达到正常水平时的退变较慢,增大或减小均会加速邻近椎间盘的退变。     

耳蜗退行性病变疗效评价

耳蜗退行性病变所致疾病已成为影响人类生活质量的主要问题之一。本文从发病机制、影响因素、临床决策方面阐述耳蜗退行性病变的研究进展,为耳蜗退行性病变所致的耳聋、耳鸣等疾病的科学研究及临床对策制定提供参考。旨在干预耳蜗退行病变,降低其发生率,提高人们生活质量。     

Neuropsychological differentiation of progressive aphasic disorders

The differentiation of subtypes of primary progressive aphasia (PPA) remains challenging. We aimed to identify optimum neuropsychological measures for characterizing PPA, to examine the relationship between behavioural change and subtypes of PPA and to determine whether characteristic profiles of language, working memory, and behavioural changes occur in PPA. Forty-seven patients with PPA and multi-domain Alzheimer's disease (AD) together with 19 age-matched controls underwent a large battery of working memory and language tests. We found that simple tasks of sentence ordering, narrative production, and buccofacial praxis were particularly useful in differentiating non-fluent/agrammatic variant PPA (nfvPPA) from other PPA subtypes, whereas a test of single word comprehension was useful in detecting semantic dementia (SD). No individual tests were discriminating for logopenic variant PPA (lvPPA) relative to nfvPPA. LvPPA and multidomain AD exhibited similar language profiles. A principal components analysis revealed that characteristic PPA profiles extended beyond the realms of language, in particular, the presence of apraxia in nfvPPA, behavioural changes in SD, and working memory deficits in lvPPA. These findings suggest that not all tests are equally discriminatory for PPA and highlight the importance of a test profile in differentiating PPA. These results also support the view that lvPPA is a focal form of AD and emphasize the difficulties classifying lvPPA.