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[Alpers' disease]. Alpers”病。
J. Ulrich, A. Cunz
Alpers' disease is a progressive, neurodevelopmental, mitochondrial DNA depletion syndrome characterized by three co-occurring clinical symptoms: psychomotor regression (dementia); seizures; and liver disease. It is an autosomal recessive disease caused by mutation in the gene for the mitochondrial DNA polymerase POLG. T he disease occurs in about one in 100,000 persons. Most individuals with Alpers' disease do not show symptoms at birth and develop normally for weeks to years before the onset of symptoms. Diagnosis is established by testing for the POLG gene. Symptoms typically occur months before tissue samples show the mitochondrial DNA depletion, so that these depletion studies cannot be used for early diagnosis. About 80 percent of individuals with Alpers' disease develop symptoms in the first two years of life, and 20 percent develop symptoms between ages 2 and 25. T he first symptoms of the disorder are usually nonspecific and may include hypoglycemia secondary to underlying liver disease, failure to thrive, infection-associated encephalopathy, spasticity, myoclonus (involuntary jerking of a muscle or group of muscles), seizures, or liver failure. An increased protein level is seen in cerebrospinal fluid analysis. Cortical blindness (loss of vision due to damage to the area of the cortex that controls vision) develops in about 25 percent of cases. Gastrointestinal dysfunction and cardiomyopathy may occur. Dementia is typically episodic and often associated with an infection that occurs while another disease is in process. Seizures may be difficult to control and unrelenting seizures can cause developmental regression as well. "Alpers-like" disorders without liver disease are genetically different and have a different clinical course. Fewer than one-third of individuals with the "Alpers-like" phenotype without liver disease have POLG mutations. Qeios · Definition, November 11, 2019
阿尔帕斯病是一种进行性、神经发育性、线粒体DNA缺失综合征,以三种共同发生的临床症状为特征:精神运动性衰退(痴呆);癫痫发作;还有肝脏疾病。它是由线粒体DNA聚合酶POLG基因突变引起的常染色体隐性遗传病。这种疾病的发病率约为十万分之一。大多数阿尔伯斯病患者在出生时没有症状,在出现症状前正常发育数周至数年。诊断是通过检测POLG基因来确定的。症状通常在组织样本显示线粒体DNA缺失前几个月出现,因此这些缺失研究不能用于早期诊断。大约80%的阿尔伯斯病患者在生命的头两年出现症状,20%的人在2岁到25岁之间出现症状。这种疾病的最初症状通常是非特异性的,可能包括继发于潜在肝病的低血糖、发育不良、感染相关脑病、痉挛、肌阵挛(肌肉或肌肉群的不自主抽搐)、癫痫发作或肝功能衰竭。脑脊液分析显示蛋白水平升高。大约25%的病例会发展为皮质性失明(由于控制视力的皮质区域受损而导致的视力丧失)。可能发生胃肠功能障碍和心肌病。痴呆症通常是发作性的,通常与另一种疾病正在发生时发生的感染有关。癫痫发作可能难以控制,持续发作也会导致发育倒退。没有肝脏疾病的“阿尔卑斯样”疾病在基因上是不同的,有不同的临床病程。只有不到三分之一的无肝脏疾病的“阿尔卑斯样”表型患者有POLG突变。Qeios·Definition, 2019年11月11日
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引用次数: 2
[Functional neurosurgery]. 功能神经外科。
J. Siegfried
Functional neurosurgery by example is a concise text designed to provide neurosurgeons in training and early career neurosurgeons a deeper dive into the nuances of functional neurosurgery through carefully selected case examples. The overall structure and format of this book follows the structure and format of the series, which includes bulleted questions, pearls, pivot points and complications. The discussions reflect the thought process behind the management choice(s) presented for each case as well as the alternative choices with their pros and cons. The cases chosen encompass the breadth of functional neurosurgery and constitute the vast majority of likely cases to be encountered during practice of functional neurosurgery.
功能神经外科的例子是一个简洁的文本,旨在提供神经外科医生在培训和早期的职业生涯神经外科医生通过精心挑选的案例例子深入到功能神经外科的细微差别。本书的整体结构和格式遵循系列的结构和格式,包括项目问题、珍珠、支点和复杂问题。讨论反映了每个病例的管理选择背后的思想过程,以及其优缺点的替代选择。所选择的病例涵盖了功能神经外科的广度,构成了功能神经外科实践中可能遇到的绝大多数病例。
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引用次数: 24
[Psychological aspects of different forms of puberty]. [青春期不同形式的心理方面]。
R J Corboz

The somato-psychic development in puberty should not be described isolated. It has to be brought in connexion with childhood on the one side and the age of the young adults on the other side. Puberty and adolescence generally are not used as synonyms. They accord with two different stages of development, succeeding one another between childhood and adulthood. They take a different, either harmonious or synchronous course. An asynchronism is often partial cause for a puberal crisis. Besides, one has to consider phenomenons like retardation or acceleration of development. These special kinds of development are illustrated by examples with two retarded adolescents. On the end, the author takes briefly up some therapeutical possibilities.

青春期的身心发展不应该被孤立地描述。一方面,它必须与童年联系起来,另一方面,与年轻人的年龄联系起来。青春期和青春期通常不作为同义词使用。它们符合两个不同的发展阶段,在童年和成年之间一个接一个。它们采取不同的、和谐的或同步的路线。不同步常常是青春期危机的部分原因。此外,人们还必须考虑发展迟缓或加速等现象。以两个智障青少年为例说明了这些特殊的发展类型。最后,作者简要介绍了一些治疗的可能性。
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引用次数: 0
[Systems approach to adolescence. Refresher course of the Swiss Psychiatric Society, Zurich, May 1983]. 青春期的系统方法。瑞士精神病学会进修课程,苏黎世,1983年5月]。
E Fivaz

The author summarizes contributions of General Systems Theory to the understanding of adolescence. GST, a metatheory that does not replace intermediary theories which are directly comparable to experience, has firstly attempted to describe hierarchical organizations. More recently, new models have focused on processes. One of these models, the evolutive paradigm, has specified essential conditions of interaction between environment and evolutive systems, called constancy and adjustment. It is applied here to data of a crisis within a non-clinical family with adolescents.

作者总结了一般系统理论对理解青少年的贡献。GST是一种不能取代直接与经验相比较的中介理论的元理论,它首先试图描述分层组织。最近,新的模型关注于过程。其中一种模式是进化范式,它规定了环境和进化系统之间相互作用的基本条件,称为恒常性和调适性。它是适用于这里的数据危机在一个非临床家庭与青少年。
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引用次数: 0
[Adolescent crisis]. (青少年危机)。
F Ladame
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引用次数: 0
[Criticism of the concept of progress in encyclopedic psychiatry]. [对百科精神病学进步概念的批评]。
J Glatzel

The concept of progress in biological psychiatry its being opposed in this study to another one which makes it necessary to adopt a different mode of thinking and to be ready for a change of paradigms. Both concepts are not exclusive of each other, yet, one cannot confer either of them an absolute value without limiting the subject and the methological tools necessary to psychiatric-psychopathological research. This will be explained, using everyday clinical cases.

在这项研究中,生物精神病学的进步概念与另一个概念是对立的,这使得有必要采用不同的思维模式,并为范式的变化做好准备。这两个概念并不是相互排斥的,然而,如果不限制精神病学-精神病理学研究所必需的主题和方法工具,就不能赋予它们任何一个绝对的价值。我们将使用日常临床病例来解释这一点。
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引用次数: 0
[Problems of the short form of the Hamburg-Wechsler Intelligence Test for Adults]. [成人汉堡-韦氏智力测验简写的问题]。
A Wotruba, P A Truttmann

A comparative study was performed using HAWIE-short form (AW + ZN + GF + MT) and the entire procedure, in which 153 psychiatric in-patients (legal-medical opinion cases), and a control group of 38 other patients were tested. Despite the proven high correlation between both HAWIE forms, it can be seen from the ascertained trend of the regression line, that the tested short form has a higher average mean value. The significant distortions, especially those in the lower I.Q. field, seem to contradict the probability of any imprudent reduction in intelligence scoring.

采用hawie -简写形式(AW + ZN + GF + MT)和整个程序进行了一项比较研究,其中对153名精神病住院患者(法律医学意见案例)和38名其他患者的对照组进行了测试。尽管证实了两种HAWIE形式之间的高度相关性,但从回归线的确定趋势可以看出,测试的短形式具有更高的平均平均值。显著的扭曲,尤其是那些低智商领域的扭曲,似乎与任何轻率降低智力得分的可能性相矛盾。
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引用次数: 0
[Strategic brief psychotherapy]. [战略简短心理治疗]。
R Van Dyck

The brief strategic therapy (directive therapy)--an active psychotherapy--is derived from the hypnotic technique of Erickson as well as from the communications and systems theories. The author describes five characteristical points of this therapy. Interactions and task prescriptions are the most important instrument of the directive therapy. The number of session is generally limited. Therapist and patient determine concrete and accurate goals for the therapy. Patient's problems should be set in a positive context. About theory and practice, the therapist adopts an eclectic and pragmatic attitude. The author develops these points and illustrates them with clinical vignettes.

简要策略治疗(指导性治疗)是一种积极的心理治疗,它来源于埃里克森的催眠技术以及传播学和系统学理论。作者描述了该疗法的五个特征点。互动和任务处方是指导治疗中最重要的工具。会话的数量通常是有限的。治疗师和患者确定具体和准确的治疗目标。病人的问题应该放在一个积极的背景下。在理论和实践上,治疗师采取折衷和务实的态度。作者发展了这些观点,并用临床实例加以说明。
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引用次数: 0
[Hysteria: problems of definition and evolution of the symptomatology]. 癔症:定义与症状演变问题。
J Frei

What is the evolution of hysteria, now that the spectacular crises of previous times tend to occur less frequently? Are hysterical manifestations disappearing or do they take other forms? This question raises semantic and phenomenological problems concerning hysteria. A review of the literature shows the complexity and precariousness of some definitions, the question raised by associated organic disorders and the compounding influences of other nosological entities, such as depression and psychosomatic disorders. Clinical histories from the records of a psychiatric hospital for the period 1910-1929 were compared with those for the period 1970-1980. The results of the study were surprising with respect to the similarity of epidemiological profiles. If it can be concluded that hysterical manifestations are not disappearing, then the study confirms the evolution of symptomatology which, especially in women, takes atypical forms of depressive, neurovegetative or psychosomatic disorders. In males, hysterical symptoms are of a more acute nature and frequently occur in dependent, immature or infantile personalities, whilst in the past they were more chronic and frequently associated with more serious personality disorders.

既然以往那些引人注目的危机发生的频率越来越低,歇斯底里症的演变是什么?歇斯底里的表现正在消失还是以其他形式出现?这个问题提出了关于歇斯底里的语义学和现象学问题。对文献的回顾显示了一些定义的复杂性和不稳定性,相关器质性疾病提出的问题以及其他疾病实体(如抑郁症和心身疾病)的复合影响。将1910-1929年精神病院记录的临床病史与1970-1980年的临床病史进行比较。就流行病学概况的相似性而言,研究结果令人惊讶。如果可以得出结论,歇斯底里的表现并没有消失,那么这项研究证实了症状学的演变,特别是在女性身上,表现为非典型的抑郁、神经植物性或心身疾病。在男性中,歇斯底里症状是一种更急性的症状,经常发生在依赖型、不成熟型或婴儿型人格中,而在过去,它们是更慢性的,经常与更严重的人格障碍有关。
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引用次数: 0
[Self healing attempts by schizophrenic patients]. 精神分裂症患者的自我治愈尝试。
W Böker, H Brenner

There has effectively been no investigation, at a systematic empirical level, whether schizophrenics possess self-help capability. Clinical observation and self description would seem to make its existence likely. This will first be shown on hand of the pertinent clinical literature. In an own investigation, which was based on a "Model of Vulnerability" (Zubin and Spring, 1977), 40 schizophrenics were questioned in accordance to the formulation provided in the Frankfurt Complaint Questionnaire (Süllwold, 1977) about their compensatory efforts concerning subjectively experienced basic disorders Reaction time measurements served as indicators of schizophrenic vulnerability. All 40 patients reported awareness about compensatory efforts in regard to experienced disorders which they conceived to be danger signals. A clearly greater number of problem solving oriented (active) than non problem solving (passive avoidance) attempts were reported. They can provisionally be classified as "reinterpretation", "restructuring", "reality testing", "action displacement" or "search for action stereotypes".

在系统的经验层面上,精神分裂症患者是否具有自助能力,实际上没有任何调查。临床观察和自我描述似乎使其存在成为可能。这将首先显示在手的相关临床文献。在一项基于“脆弱性模型”(Zubin and Spring, 1977)的调查中,根据法兰克福投诉问卷(s llwold, 1977)中提供的提法,对40名精神分裂症患者进行了询问,询问他们对主观经历的基本障碍的补偿努力。反应时间测量是精神分裂症脆弱性的指标。所有40名患者都报告了对他们认为是危险信号的经历障碍的代偿努力的认识。问题解决导向(主动)的尝试明显多于非问题解决导向(被动回避)的尝试。它们可以暂时归类为“重新诠释”、“重组”、“现实检验”、“行动置换”或“寻找行动刻板印象”。
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Schweizer Archiv fur Neurologie, Neurochirurgie und Psychiatrie = Archives suisses de neurologie, neurochirurgie et de psychiatrie
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