Corrélats anatomocliniques au cours de la démence de la maladie de Parkinson

IntroductionCognitive disorders such as deficit of attention and executive and visuoconstructive dysfunctions occur in Parkinson's disease dementia (PDD). Memory impairment is not an early feature and statement not well delimited.Case reportA 78-year-old man with PDD underwent neuropsychological assessment and moreover demonstrated memory decline. After death, pathology examination of the brain and immunohistochemy analysis confirmed PD and showed Lewy body pathology (LBP) in the insula, limbic and especially in CA3 hippocampus areas. Hippocampus and gyrus parahippocampic also exhibited neurofibrillary tangles. Lack of senile plaque and lack of beta A4 amyloid deposition were noticeable in the whole brain examination.ConclusionSevere executive dysfunctions are probably related to LBP and dysfunction in memory process may be related to DNF lesions in medial temporal area.     

Sur la pathogénie de la Sclérose Tubéreuse

Résumé Les auteurs ont étudié cinq cas de sclérose tubéreuse, dont trois autopsiés. À partir des lésions cutanées, ils ont soupçonné que le processus était essentiellement une perturbation métabolique des organes atteints, due à des malformations vasculaires. L'étude des lésions encéphaliques confirma l'hypothèse. En effet, l'examen microscopique de coupes colorées par les méthodes classiques pour l'étude des cellules nerveuses, de la glie et des vaisseaux révélèrent que les cellules monstrueuses de la sclérose tubéreuse sont des éléments dégénérés des trois lignages qui donnent le tissue nerveux, et, d'un autre côté, montrèrent que, semblablement à ce qui arrive pour le peau, les vaisseaux de ce tissu sont mal formés dans cette maladie. À partir de ces données, ils formulèrent une nouvelle conception pathogénique pour expliquer la maladie.

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Zusammenfassung Die Verfasser untersuchten fünf Fälle von tuberöser Sklerose, darunter drei autoptisch verifizierte. Auf Grund der Hautveränderungen vermuten sie, daß der Prozeß im wesentlichen eine Stoffwechselstörung der betroffenen Organe infolge von Gefäßmißbildungen darstellen könnte. Die Untersuchung der Hirnveränderungen bekräftigte diese Hypothese. Die histologische Untersuchung mittels der klassischen Routinefärbungen für Nervenzellen, Glia und Gefäße zeigte, daß es sich bei den Monstrezellen der tuberösen Sklerose um degenerierte Elemente der drei Bauteile des Nervengewebes handelt und daß anderseits — in Analogie zu den Hautveränderungen — eine Fehlbildung der Gefäße im ZNS vorliegt. Auf Grund dieser Befunde wird eine neue pathogenetische Konzeption der Erkrankung formuliert.

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Avec 5 illustrations dans le texte

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Ouvrage couronné du prix Alvarenga de 1963 de l'Académie Nationale de Médicine du Brésil.     

Le réseau de surveillance de la maladie de Creutzfeldt-Jakob

IntroductionTransmissible spongiform encephalopathies (TSE) have been under epidemiological surveillance in France and in Europe since the early 1990s. The observation of iatrogenic Creutzfeldt-Jakob disease (CJD), the outbreak of bovine spongiform encephalopathy (ESB) and its probable transmission to many species gave rise to the surveillance which remains warranted by the emergence of a variant of CJD (vCJD), in 1996.State of artIn France, epidemiological surveillance is coordinated by the InVS which receives input from cases notifications addressed to INSERM Unit 708 directly by clinicians or more often following requests for 14-3-3 detection in CSF. All suspected cases are followed up until a final diagnosis is established. Thanks to the effectiveness of the French network of neuropathology, autopsies are performed in more than half of patients who die with a diagnosis of suspected CJD. Diagnostic criteria allow comparison of the incidence of the different forms of the disease in all countries with a system of surveillance. Sporadic CJD is the most frequent form of the disease with more than 80% of the cases. Its origin remains unknown. To date, cases of iatrogenic CJD referred to the French surveillance network have been caused by dura mater grafts or human growth hormone treatments administrated in the 1980s. Ten percent of TSE are of genetic origin with an autosomic dominant transmission of a mutation or an insertion located on the PRNP gene. The most recent form of the disease is vCJD which is a new form, first described in the United Kingdom in 1994.Prospect and conclusionActive epidemiological surveillance remains a timely issue, particularly in France, because of the development of new cases of iatrogenic CJD after human growth hormone treatment. It is of importance in France and worldwide because of the emergence of post-transfusional cases of vCJD and the possible appearance of vCJD in persons with valine-valine or methionine-valine genotypes at codon 129.     

Épidémiologie de la SLA

Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease. Loss of pyramidal and anterior horn motor neurons leads to progressive limb weakness, disability, dysarthria, dysphagia and respiratory insufficiency with a progressive fatal course. The incidence of ALS ranges between 1.5 to 2.5 for 100,000 per year. Although there are familial cases of ALS, about 90% are sporadic and of unknown etiology. Several exogenous risk factors have been documented. However, no convincing evidence has demonstrated in a reproducible manner an association between an environmental or lifestyle risk factor and ALS. Disease duration varies considerably, ranging from a few months to 10–15 years with a mean survival of about 36 months. Prognostic factors such as age, site of disease onset, nutritional, functional and respiratory status at the diagnosis or delay between beginning of the disease and diagnosis have been reported but they appear to be insufficient to explain prognostic variability. These last 15 years, development of supportive care for ALS patients and management in ALS centers may have contributed to improve survival. Finally, ALS centres, and particularly French ALS centres, have developed databases to improve our knowledge of ALS, phenotypic characterization, more accurate phenotype–genotype correlations and thus contribute to new therapeutics developments.     

Pourquoi la clinique de la douleur est-elle une clinique de la complexité ?

Easily understood on a daily basis as a signal for somatic disturbance, pain cannot be apprehended so easily. Definition and clinical of pain remind us of a key principle: there is no physical pain without psychological counterpart, and vice versa; and there is no psychological disorder without a physical correlation. However, the complex nature of the pain clinic does not stop there. We can distinguish is at least six dimensions allowing us to answer the uncertainty and the difficulty of its apprehension: through language, through culture, through representation, through personality organization, through pain's functions and other additional complications due to the confusion taking place in the designations, and through the different nosographic classifications. Pain is the limit of all knowledge, may it be medical, nursing, psychology, and invites us all to confront with other schools of thought and to work together to hear what is said and not said by the subject. In this singular clinic, the specificity of the psychologue and the importance of his view reside precisely in an appreciation of the psychic dynamics of the patient which interact with pain and which express itself within pain beyond somatic aspects.     

Place de la psychothérapie intégrative dans la prise en charge des problématiques de radicalisation

Recent waves of attacks in France have led health and justice professionals to meet, exchange and work together, with a goal that is common: the care of people with radicalization issues. The operationalization of this objective macro makes it possible to distinguish the missions of the different actors at stake. Thus, the question of care requires the definition of the object of intervention, the purpose, rehabilitative and/or therapeutic, and the means available. In this interview, members of the ARCA, Erwan Dieu and Olivier Sorel evoke the place of integrative psychotherapy in the management of radicalization issues. The purpose of this discussion is to clarify the functions and objectives of each of the actors at the interface of people presenting this form of radicalism.     

Les progrès dans la réalisation de la classification quantitative de la psychopathologie

Shortcomings of approaches to classifying psychopathology based on expert consensus have given rise to contemporary efforts to classify psychopathology quantitatively. In this paper, we review progress in achieving a quantitative and empirical classification of psychopathology. A substantial empirical literature indicates that psychopathology is generally more dimensional than categorical. When the discreteness versus continuity of psychopathology is treated as a research question, as opposed to being decided as a matter of tradition, the evidence clearly supports the hypothesis of continuity. In addition, a related body of literature shows how psychopathology dimensions can be arranged in a hierarchy, ranging from very broad “spectrum level” dimensions, to specific and narrow clusters of symptoms. In this way, a quantitative approach solves the “problem of comorbidity” by explicitly modeling patterns of co-occurrence among signs and symptoms within a detailed and variegated hierarchy of dimensional concepts with direct clinical utility. Indeed, extensive evidence pertaining to the dimensional and hierarchical structure of psychopathology has led to the formation of the Hierarchical Taxonomy of Psychopathology (HiTOP) Consortium. This is a group of 70 investigators working together to study empirical classification of psychopathology. In this paper, we describe the aims and current foci of the HiTOP Consortium. These aims pertain to continued research on the empirical organization of psychopathology; the connection between personality and psychopathology; the utility of empirically based psychopathology constructs in both research and the clinic; and the development of novel and comprehensive models and corresponding assessment instruments for psychopathology constructs derived from an empirical approach.     

Phénoménologie de la curiosité comme rupture temporelle : sur la route de la Beat Generation

Jack Kerouac's famous autobiographical novel “On the Road” tells the epic journey through the magnificent landscapes of the American continent through frantic, jazz-rhythmic writing. In this book, beyond the classical framework of a travel journal, Kerouac aspires to tell a style of existence through a style of writing. The perpetual feast of the senses and the erotic evokes the exalted adventure of the Beat Generation and testify to this style of existence characteristic of what phenomenology calls existential curiosity. In the midst of unexplored landscapes, unexpected encounters, in the aftermath of a flight to be pursued without ceasing, Kerouac aimed at the permanent improvisation, that of the “bebop”, that of the “instant literature”. The writing of a road that takes shape at the moment under the wheels of the car that traces its way as fast as it does erase it. And this is a perpetual quest for novelty, in openness to curiosity. It appears then that the phenomenological notion of this experience of curiosity, makes possible to approach the foundations and the wanderings of the beat generation, and more broadly the experience of the generations, which have succeeded.     

Tolérance à la détresse et expérience de la douleur chronique

IntroductionChronic pain is a highly prevalent condition that is associated with distressing somatic and emotional experiences. Consequently, an individual's distress tolerance, the perceived capacity to tolerate negative psychological and physical states, may influence their pain experience. This effect could be explained in part by a reduction in the catastrophic interpretation of pain which is associated with increased pain intensity and interference in everyday activities.AimsThe first aim of this study was to explore the association between the components of the 5-factor model of distress tolerance and (1) pain intensity and (2) pain interference in everyday activities. The secondary aim was to assess the potential mediating effect of pain catastrophizing in the eventual association between components of distress tolerance and (1) pain intensity or (2) pain interference in everyday activities.MethodThis is a cross-sectional study of adult (18 years or older) university students and staff with chronic pain (3 months). They were invited to complete the online questionnaire through an email invitation. Pain intensity and interference in everyday functioning were assessed with the corresponding subscales of the Brief Pain Inventory. The following instruments were used to assess the components of the 5-factor model of distress tolerance: Ambiguity Tolerance Scale (tolerance to ambiguity), Intolerance to Uncertainty Scale (reversed score: tolerance to uncertainty), Discomfort Intolerance Scale (reversed score: discomfort tolerance), Distress Tolerance Scale (tolerance to negative emotions), Frustration Discomfort Scale (tolerance to frustration). Participants also completed the Pain Catastrophizing Scale.ResultsEighty participants were recruited (57 % women, mean age = 33.09; standard deviation = 12,87). Tolerance to negative emotions was the only component of distress tolerance that was associated with pain (ß = ?0.04; 95% CI): ?0.07–?0.01; t (78) = ?3.06, p < 0.01) or pain interference in everyday functioning (ß = ?0.07; 95% CI: ?0.10–?0.03; t (78) = ?3.97, p < 0.01), independently of the others. Combined with age, these factors explained 16.2 % of the variance in pain intensity and 19.4 % of the variance in pain interference. Pain catastrophizing partially mediated the association between tolerance to negative emotions and pain interference in everyday functioning, but it was not involved in the association between tolerance to negative emotions and pain intensity.ConclusionTolerance to negative emotions appears to be the most relevant aspect of distress tolerance in the context of chronic pain and is a potential clinical target that is independent and complementary from pain catastrophizing.     

La place de la compréhension des proverbes et des métaphores dans la compréhension des troubles du cours de la pensée dans la schizophrénie : apport de l’évaluation clinique,de l’imagerie cérébrale fonctionnelle et de la neurophysiologie

According to Bleuler (1911), language impairments were indicative of thought disorders, a fundamental feature of schizophrenia. The objectives of the present review are twofold: (1) to present the clinical interest of the use of the tests of proverbs and metaphors comprehension for the evaluation of the characteristics of thought disorders in schizophrenia, and to highlight the heterogeneity of the forms of their interpretations; (2) to present the results from neuroimaging and neurophysiological studies of metaphor comprehension in order to identify the neurocognitive mechanisms underlying metaphorical language comprehension of patients with schizophrenic disorders. Results from the reviewed clinical applications of proverbs tests showed that thought disorders in individuals with schizophrenia are illustrated by different forms of proverb interpretations: literal, concrete, idiosyncratic, over-inclusiveness and unconventional. Results from the reviewed brain imaging and neurophysiological studies showed that these different forms of figurative misinterpretations may be related to different neurocognitive mechanisms: concreteness appears to be related to the dysfunction of the left inferior frontal gyrus and inverted brain lateralization during metaphor processing in individuals with schizophrenia compared to healthy individuals. During novel metaphor comprehension, which is highly effortful, individuals with schizophrenia, relative to controls, demonstrate increased activation of the right precuneus, a region that mediates complex and highly integrated functions including retrieval of episodic memory and mental imagery. These results may suggest that individuals with schizophrenia use mental imagery to support comprehension of both literal and metaphoric language. Furthermore, it was demonstrated that over-inclusive thinking, which is exhibited by individuals with schizophrenia during figurative language comprehension, could be related to very early right hemispheric hyper-activation (when a left early activation is expected) and to reduced bilateral brain activity during semantic processing. Such as reversed brain activation may explain why the individuals with schizophrenia are relatively over reliant on early-stage coarse or large semantic processing and may be prone to form meanings that are idiosyncratic and highly unconventional.     

La régulation de la circulation périphérique

Résumé Le système nerveux végétatif joue le rôle principal dans la régulation de la circulation sanguine périphérique. La vasodilatation qui se produit dans les organes au travail est déclenchée par tous les acides produits par le métabolisme, par des produits intermédiaires non-acides de ce métabolisme, ainsi que par l'acétylcholine, l'adénosine-triphosphate et l'histamine. Les artères afférentes à ces organes sont dilatées par un réflexe, le réflexe nutritif ascendant deFleisch, dont le mécanisme est le suivant: les métabolites mentionnés plus haut excitent une sensibilité nutritive tissulaire, ce qui détermine des impulsions nerveuses qui se remontent aux artères afférentes. En bloquant cette sensibilité nutritive tissulaire par la novocaïne, on inhibe la dilatation artérielle qui se produit normalement pendant le travail. En même temps que les artères des organes au travail se dilatent, il se produit une vasoconstriction collatérale dans les organes au repos. Cette vasoconstriction est déclenchée par trois mécanismes: 1. Les réflexes nutritifs ascendants élèvent le tonus du centre vasomoteur; 2. Ce tonus est également augmenté par l'élévation du taux du CO₂ dans le sang; 3. La production d'adrénaline déclenche une casoconstriction dans les organes au repos.

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Summary The vegetative nervous system plays the principal role in the regulation of the peripheral circulation. The vasodilatation which arises in organs during work is produced by all acid products of metabolism, and also by non-acid intermediary products, as well as by acetylcholine, adenosine-triphosphate and histamine. The afferent arteries to these organs are dilated by a reflex, the ascendant nutritive reflex ofFleisch, the mechanism is as follows:The aforementioned metabolites excite a nutritive sensibility existing in the tissues, which now determines nervous impulses going to the afferent arteries. On blocking this nutritive tissue sensibility with novocaine, we inhibit the arterial dilatation normally produced during work. At the same time that the arteries of the organs at work dilate, there is a collateral vasoconstriction produced in the organs at rest. This vasoconstriction is a result of three mechanisms: 1. The ascending nutritive reflexes which raise the tonus of the vaso-motor center 2. This tonus is also augmented by the elevation of the amount of CO₂ in the blood. 3. The secretion of adrenaline gives rise to a vasoconstriction in the organs at rest.

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Zusammenfassung Das neurovegetative Nervensystem bildet die Grundlage der Regulierung des peripheren Kreislaufs. Die lokale Gefäßerweiterung in arbeitenden Organen wird bewirkt durch alle beim Stoffwechsel gebildeten Säuren und durch nichtsaure intermediäre Produkte, ferner durch Acetylcholin, Adenosintriphosphat und Histamin. Die zum arbeitenden Organ führenden Arterien werden durch die aszendierenden nutritiven Kreislaufreflexe (Fleisch) erweitert. Ihre Funktionsweise ist folgende: Die oben genannten Stoffwechselprodukte erregen eine im Gewebe befindliche nutritive Sensibilität, von wo aus dilatierende Impulse zu den zuführenden Arterien aufsteigen. Blockierung dieser Sensibilität durch Novocain unterdrückt die arterielle Gefäßerweiterung bei Arbeit.Die Gefäßerweiterung in arbeitenden Organen ist begleitet von einer kollateralen Vasokonstriktion in den ruhenden Organen. An ihrer Entstehung sind drei Mechanismen beteiligt: 1. Die aszendierenden nutritiven Reflexe erhöhen den Tonus des Vasomotorenzentrums 2. Dasselbe wird bewirkt durch die Anreicherung von CO₂ im Blute. 3. Adrenalinausschüttung erzeugt Vasokonstriktion in ruhenden Organen.