{"title":"[Alpers' disease].","authors":"J. Ulrich, A. Cunz","doi":"10.32388/661530","DOIUrl":"https://doi.org/10.32388/661530","url":null,"abstract":"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","PeriodicalId":21430,"journal":{"name":"Schweizer Archiv fur Neurologie, Neurochirurgie und Psychiatrie = Archives suisses de neurologie, neurochirurgie et de psychiatrie","volume":"54 1","pages":"297-303"},"PeriodicalIF":0.0,"publicationDate":"2019-11-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90325956","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Functional neurosurgery].","authors":"J. Siegfried","doi":"10.1227/01.neu.0000279767.51639.90","DOIUrl":"https://doi.org/10.1227/01.neu.0000279767.51639.90","url":null,"abstract":"\u0000 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.","PeriodicalId":21430,"journal":{"name":"Schweizer Archiv fur Neurologie, Neurochirurgie und Psychiatrie = Archives suisses de neurologie, neurochirurgie et de psychiatrie","volume":"92 1","pages":"435-42"},"PeriodicalIF":0.0,"publicationDate":"2019-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83428191","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Psychological aspects of different forms of puberty].","authors":"R J Corboz","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":21430,"journal":{"name":"Schweizer Archiv fur Neurologie, Neurochirurgie und Psychiatrie = Archives suisses de neurologie, neurochirurgie et de psychiatrie","volume":"134 2","pages":"219-28"},"PeriodicalIF":0.0,"publicationDate":"1984-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17520999","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Systems approach to adolescence. Refresher course of the Swiss Psychiatric Society, Zurich, May 1983].","authors":"E Fivaz","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":21430,"journal":{"name":"Schweizer Archiv fur Neurologie, Neurochirurgie und Psychiatrie = Archives suisses de neurologie, neurochirurgie et de psychiatrie","volume":"134 2","pages":"229-39"},"PeriodicalIF":0.0,"publicationDate":"1984-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17522707","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Adolescent crisis].","authors":"F Ladame","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":21430,"journal":{"name":"Schweizer Archiv fur Neurologie, Neurochirurgie und Psychiatrie = Archives suisses de neurologie, neurochirurgie et de psychiatrie","volume":"134 2","pages":"263-6"},"PeriodicalIF":0.0,"publicationDate":"1984-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17522710","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Criticism of the concept of progress in encyclopedic psychiatry].","authors":"J Glatzel","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":21430,"journal":{"name":"Schweizer Archiv fur Neurologie, Neurochirurgie und Psychiatrie = Archives suisses de neurologie, neurochirurgie et de psychiatrie","volume":"134 2","pages":"295-304"},"PeriodicalIF":0.0,"publicationDate":"1984-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17522712","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Problems of the short form of the Hamburg-Wechsler Intelligence Test for Adults].","authors":"A Wotruba, P A Truttmann","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":21430,"journal":{"name":"Schweizer Archiv fur Neurologie, Neurochirurgie und Psychiatrie = Archives suisses de neurologie, neurochirurgie et de psychiatrie","volume":"135 2","pages":"281-6"},"PeriodicalIF":0.0,"publicationDate":"1984-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17578743","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Strategic brief psychotherapy].","authors":"R Van Dyck","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":21430,"journal":{"name":"Schweizer Archiv fur Neurologie, Neurochirurgie und Psychiatrie = Archives suisses de neurologie, neurochirurgie et de psychiatrie","volume":"134 1","pages":"161-73"},"PeriodicalIF":0.0,"publicationDate":"1984-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17763910","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Hysteria: problems of definition and evolution of the symptomatology].","authors":"J Frei","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":21430,"journal":{"name":"Schweizer Archiv fur Neurologie, Neurochirurgie und Psychiatrie = Archives suisses de neurologie, neurochirurgie et de psychiatrie","volume":"134 1","pages":"93-129"},"PeriodicalIF":0.0,"publicationDate":"1984-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17763913","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Self healing attempts by schizophrenic patients].","authors":"W Böker, H Brenner","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>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\".</p>","PeriodicalId":21430,"journal":{"name":"Schweizer Archiv fur Neurologie, Neurochirurgie und Psychiatrie = Archives suisses de neurologie, neurochirurgie et de psychiatrie","volume":"135 1","pages":"123-33"},"PeriodicalIF":0.0,"publicationDate":"1984-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"17542134","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}