Le distrofie da neuro-irritazione (impostazione generale del problema)

Riassunto L'A., dopo un'analisi dei dati di ordine sperimentale e clinico, giunge alla conclusione che: con la dizione distrofie da neuroirritazione si vengono sostanzialmente ad intendere condizioni morbose che si esprimono, dal punto di vista istopatologico, soprattutto con fenomeni di infiammazione sierose e fibrinoidosi e, da un punto di vista anatomo-clinico, coi quadri della fibrosite idiopatica (muscolare e periarticolare in ispecie) e più raramente con manifestazioni di tipo sclerodermico o di tipo arteritico o con manifestazioni reattivo-produttive (tipo malattia di Dupuytren) e che insorgono quale effetto dell'azione del sistema nervoso in particolari condizioni dell'eccitamento.Le manifestazioni insorgono elettivamente in determinati organi bersaglio per lo stabilirsi di focolai di irritazione (punti trigger), o in particolari condizioni della psico-affetività (in alcune psico-nevrosi in ispecie). Uno dei quadri più caratteristici è quello della sindrome spalla-mano.Nell'orbita dei quadri reattivi più comuni suddetti la risposta è qualitativamente e quantitativamente variabile a seconda della specie animale, del ceppo, dell'età, del sesso e dell'individuo.L'insorgenza, l'entità e la caratteristiche dei fenomeni in patologia umana sono subordinate al realizzarsi di due condizioni: lo stabilirsi di un focolaio di irritazione e l'esistenza di particolari, individuali, stati di abnorme responsività reattiva dei bersagli. Sono due condizioni—che cioè un punto trigger divenga efficiente e l'organo bersaglio sofferente—che appaiono spesso coordinate fra loro.L'A. analizza le conoscenze raggiunte in proposito; discute i rapporti fra distrofie reattive da neuro-irritazione e patologia della reattività in generale; la coordinazione fra il capitolo delle distrofie suddette, quello delle malattie reumatiche e quello delle mesenchimopatie reattive (cosiddette malattie del collageno) sia del sottogruppo con prevalente fibrinoidosi intravascolare, sia di quello con prevalente fibrinoidosi extravascolare: discute l'importanza di alcuni fattori preparati, favorenti ed aggravanti di vario ordine, quelli di ordine endocrino in ispecie.Richiama l'attenzione sui fenomeni di automantenimento delle distrofie reattive; su quelli delle reazioni a catena; sui problemi del meccanismo dell'azione nervosa: meccanismi vasomotori e azione diretta sull'intimo metabolismo dei tessuti. Discute inoltre l'importanza della strutture reticolari periferiche ultraespansionali.L'A. ricorda le differenze fra le manifestazioni reattive da focolaio periferico di irritazione e le manifestazioni, dello stesso ordine, in alcune psiconeurosi. Accenna infine ad alcune manifestazioni più rare, specie di ordine emorragico.Conclude rilevando la possibilità di superamento di un apparente contrasto fra i dati derivanti dalle ricerche sperimentali e quelli derivanti dall'osservazione clinica.

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Summary Considering experimental and clinical data, the author concludes that by the denomination neuro-irritation dystrophies are actually meant morbous conditions concerning, from a histopathologic point of view, mostly serous inflammation and fibrinoidosis. From an anatomo-clinically point of view, it means the pictures of hidiopathic fibrositis (especially muscular and peri-articular) and, more rarely, sclerodermic or arteritic aspects, or alterations with reactive productive aspects (Dupuytren's disease), occurring because of intervention of the nervous system in particular conditions of excitement. Alterations occur particularly in target organs, in consequence of the settling of irritation focuses (trigger points) or in peculiar conditions of psychoreactivity (especially psycho-neurosis). One of most characteristic aspects is that of the shoulder-hand syndrome.In a reactive pattern, as above described, the answer is qualitatively and quantitatively variable according to animal species, strain, age, sex, and individual conditions.Occurrence, entity and character of the phenomena in human pathology are depending on the realization of the following two conditions: settling of an irritation focus; existence of particular individual conditions of abnormal responsiveness of the targets. There are two conditions which are often concomitent, namely, that the trigger point becomes efficient and that the target organ becomes suffering.The author discusses the actual knowledge concerning the following subjects: the relationship beetween reactive dystrophies caused by neuroirritation and the pathology of reactivity in general; the coordination with the above mentioned dystrophies and reactive mesenchymopathies (so-called collagen diseases), either of the group of intravascular fibroidosis or of the one of extravascular fibroidosis. The importance of preparing, supporting, and aggravating, factors of various kinds, especially the endocrine ones are discussed.Attention is called to self-supporting phenomena in reactive dystrophies, to chain reactions, to problems concerning the mechanism of nervous action, to vasomotor mechanisms and direct action on the inner metabolism of the tissues. The importance of reticular structures is discussed. The author recalls differences between reactive alterations by a peripheral focus and similar alterations occurring in psychoneurosis. More rare aspects, especially hemorragic ones, are mentioned.The author finishes by suggesting, that there is a possibility to overcome an apparent contrast between the data experimentally obtained and those detected by clinical observation.

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Zusammenfassung Nach einer Analyse der experimentellen und klinische Befunde kommt der Autor zu dem Schluß, daß man unter dem Ausdruck Nervenreiz-Dystrophie im wesentlichen solche krankhaften Zustände versteht, die histologisch durch seröse Entzündung und Fibrinoidose, in anatomischer und klinischer Hinsicht durch Fibrositis (muskulär, besonders aber periarticulär) charakterisiert sind. Seltener zeigen diese Erkrankungen sklerodermische, arteriitische und reaktiv produktive Veränderungen (wie die Dupuytrensche Krankheit) oder solche, die als Reaktion auf besondere Erregungszustände des Nervensystems zurückgehen.Diese Manifestationen entstehen ausschließlich in bestimmten Effektorganen (organi bersaglio) durch die Bildung von Irritationsfoci (trigger-points) oder bei besonderen Bedingungen der Psychoaffektivität (speziell bei einer Psychoneurose). Charakteristisch dafür ist das Schulter-Hand-Syndrom. Diese, wie oben besprochen, reaktiven Veränderungen sind qualitativ und quantitativ von der Tiergattung, dem Stamm, dem Alter, dem Geschlecht und dem Individuum abhängig. Das Auftreten, die Ausdehnung und der Charakter dieser Phänomene in der menschlichen Pathologie sind vom Zusammentreffen zweier Bedingungen abhängig: von der Stabilisierung eines Irritationsfocus und dem Bestehen einer besonderen, individuellen, abnormen Reaktivität der Effektororgane. Es sind also zwei Bedingungen—daß nämlich ein Trigger-Punkt wirkt und daß ein Effektororgan leidend wird—,die oft zusammengeschlossen wirken.Der Autor analysiert das bisher erreichte diesbezügliche Wissen, diskutiert die Beziehungen zwischen reaktiver Dystrophie infolge Nervenreiz und der Pathologie der Reaktivität im allgemeinen, bespricht die Beziehungen zwischen den oben genannten Dystrophien, den rheumatischen Krankheiten und den reaktiven Mesenchymopathien (sogenannte Kollagenkrankheiten), sei es die Untergruppe mit vornehmlich intravasculärer, sei es die mit vor allem extravasculärer Fibrinoidose. Der Referent erwähnt die Bedeutung begünstigender und erschwerender Faktoren verschiedener Ordnung und weist speziell auf die endokrinen hin.Er erwähnt besonders die Erscheinung der Automatisierung der reaktiven Dystrophien; die Kettenreaktion, die Probleme des Mechanismus der nervösen Aktion, nämlich vasomotorische Wirkung und direkte Beziehung auf den Gewebsstoffwechsel. Er bespricht außerdem die Bedeutung der ausgedehnten, peripheren, netzartigen Strukturen. Der Autor zeigt die Unterschiede zwischen den reaktiven Erscheinungen bei peripheren Reizherden und denen gleicher Ordnung bei einigen Psychoneurosen auf. Außerdem weist er noch auf seltene Reaktionen, besonders von hämorrhagischem Charakter, hin. Abschließend wird bewiesen, daß es möglich ist, die widersprechenden Ergebnisse der experimentellen Forschung und der klinischen Beobachtungen auf einen Nenner zu bringen.

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Résumé L'auteur, après une analyse des données d'ordre expérimental et clinique, arrive à la conclusion suivante: sous la définition de la «dystrophie par neuroirritation» on comprend surtout des conditions morbides qui, du point de vue histopathologique, sa manifestent surtout par des phénomènes inflammatoires séreuses et fibrinoides et, du point de vue anatomo-clinique, par une fibrosite idiopathique (musculaire et périarticulaire) et, plus rarement, par des manifestations du type sclérodermique ou du type artérique, aussi par des signes réactivo-productifs (type de la maladie de Dupuytren), comme effet d'action du système nerveux dans des conditions particulières d'excitation. Ces manifestations surviennent dans des organescibles où leur apparition élective est caractérisée par des foyers d'irritation (points «trigger») ou sous des conditions particulières de psycho-affectivité (dans quelques psychonévroses en particulier). Un des syndromes les plus caractéristiques est ce-lui de «l'épaule-main».Dans l'ensemble des aspects réactifs plus communs mentionnés, la réponse est qualitativement et quantitativement variable selon l'espèce de l'animal, selon la souche, l'âge, le sexe et l'individu.L'apparition, l'entité et les caractéristiques des phénomènes dans la pathologie humaine sont subordonnées à la réalisation de deux conditions: l'établissement d'un foyer d'irritation et l'existence d'états particuliers et individuels de réponse réactive anormale des «cibles». Il s'agit de deux conditions—c'est à dire qu'un point «trigger» devient «efficient» et que l'organe cible devient souffrant qui semblent souvent coordonnées entre elles.L'auteur considère les connaissances acquises à ce propos; il discute les rapports entre les dystrophies réactives dues à neuro-irritation et la pathologie de la réactivité en général; il discute en outre la coordination entre le chapitre des dystrophies susnommées; celui des mésenchymopathies réactives (soi-disant maladies du collagène) voire du sousgroupement avec préminente fibrinoidose intravasculaire voire du sousgroupement avec préminente fibrinoidose extravasculaire; il discute l'importance de quelques facteurs préparants, favorisants et aggravants d'ordre varié, ceux d'ordre endocrinien et particulier.L'auteur fixe l'attention sur les phénomènes de l'auto-entretien des dystrophies réactives; sur ceux de la «réaction en chaîne»; sur les problèmes du mécanisme de l'action nerveuse; du mécanisme vaso-moteur et de l'action directe sur le métabolisme intime de tissus. Il discute enfin l'importance des structures réticulaires périphérique ultraexpansionelles.L'auteur rappele les différences entre les manifestations réactives dues à un foyer périphériques d'irritation et les manifestations, du même ordre, dans quelques psychonévroses. Il fait mention aussi de quelques manifestations plus rares, surtout d'ordre hémorragique.Il finit son developpement en soulignant la possibilité de surmonter une apparente contradiction entre les données expérimentales et celles cliniques.

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Referat nicht eingelangt     

Disordini del movimento

Neurological Sciences -     

Osservazioni istoanatomiche sopra il plesso nervoso sottosieroso dello stomaco

Sommario Gli AA. hanno studiato con metodi neuroistologici, il plesso sottosieroso dello stomaco ed hanno descritto i suoi componenti. Sono stati rinvenuti nella sottosierosa alcuni recettori da considerarsi recettori di tensione, microgangli con fibre postgangliari destinati alla muscolatura longitudinale e al plesso di Auerbach. Lungo i fascetti del nervo vago sono stati reperiti microgangli con neuroni, probabilmente, sensitivi.

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Summary The neuro-histological study of the plexus located below the serosa of the stomach showed some presso-receptors and some micro-ganglia, whose postganglionic fibres innervate the longitudinal musculature or join the plexus of Auerbach. In addition, micro-ganglia were found between the fine bundles of the vagus nerve; their neurons seem to have a visceral-afferent function.

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Zusammenfassung Die neuro-histologische Untersuchung des Plexus unterhalb der Serosa des Magens ließ einige Pressorezeptoren erkennen, sowie Mikroganglien, deren postganglionäre Fasern zur Längsmuskulatur oder zum Plexus Auerbach ziehen. Außerdem befinden sich in den Bündeln des N. vagus Mikroganglia, deren Neurone anscheinend eine visceral-afferente Funktion haben.

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Résumé L'étude neuro-histologique du plexus subséreux de l'estomac a mis en évidence quelques récepteurs dignes d'attention: presso-récepteurs et micro-ganglions à fibres post-ganglionnaires destinées à la musculature longitudinale et au plexus d'Auerbach. En outre, le long des faisceaux du nerf vague, il existe des micro-ganglions neuroniques probablement sensitifs.

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Con 12 Figure     

Nuestra experiencia en el diagnóstico etiológico del retraso global del desarrollo y discapacidad intelectual: 2006-2010

Introduction

Global developmental delay (GDD) and intellectual disability (ID) are common reasons for consultation in paediatric neurology. Results from aetiological evaluations of children with GDD/ID vary greatly, and consequently, there is no universal consensus regarding which studies should be performed.

Material and method

We review our experience with determining aetiological diagnoses for children with GDD/ID who were monitored by the paediatric neurology unit over the 5-year period between 2006 and 2010.

Results

During the study period, 995 children with GDD/ID were monitored. An aetiological diagnosis was established for 309 patients (31%), but not in 686 (69%), despite completing numerous tests. A genetic cause was identified in 142 cases (46% of the total aetiologies established), broken down as 118 cases of genetic encephalopathy and 24 of metabolic hereditary diseases. Our data seem to indicate that diagnosis is easier when GDD/ID is associated with cerebral palsy, epilepsy, infantile spasms/West syndrome, or visual deficit, but more difficult in cases of autism spectrum disorders. Genetic studies provide an increasing number of aetiological diagnoses, and they are also becoming the first step in diagnostic studies. Array CGH (microarray-based comparative genomic hybridisation) is the genetic test with the highest diagnostic yield in children with unexplained GDD/ID.

Discussion

The cost-effectiveness of complementary studies seems to be low if there are no clinically suspected entities. However, even in the absence of treatment, aetiological diagnosis is always important in order to provide genetic counselling and possible prenatal diagnosis, resolve family (and doctors’) queries, and halt further diagnostic studies.     

Afasia progresiva primaria: del síndrome a la enfermedad

Introduction

Primary progressive aphasia (PPA) is a clinical syndrome characterised by a progressive decline in language and speech of neurodegenerative origin. Major breakthroughs made in recent years have lent us a better understanding of this syndrome, which may be the first manifestation of any of a number of neurodegenerative diseases.

Development

We reviewed the main aspects of PPA epidemiology, clinical manifestations, diagnosis, aetiology and treatment. Most cases manifest sporadically and the typical age of onset is between 50 and 70 years. Three clinically distinct variants have been described: nonfluent or agrammatic PPA, semantic PPA and logopenic PPA. Each of these variants tends to be associated with specific histopathological findings, but clinical diagnostic methods are imperfect predictors of underlying pathology. Anatomical and functional neuroimaging can provide useful biomarkers. Several treatments have been proposed, and while no clear benefits have been demonstrated, acetylcholinesterase inhibitors may be useful, especially in the logopenic variant.

Conclusions

PPA is an emerging syndrome which may be more prevalent than we might expect. It was previously listed as part of the frontotemporal dementia spectrum, and it is also related to Alzheimer disease. Clinical diagnosis, complemented by a biomarker evaluation, may predict the underlying pathology, which in turn will improve treatment possibilities.     

La réaction de Marchi: Aspects ultrastructuraux

A secondary demyelination process was brought about in the optic tract by unilateral enucleation of 10 adult rats which were sacrificed at 8, 15, 20, 30 and 45 days respectively, after the intervention. The Marchi reaction, which is identifiable by the presence of granular bodies, is positive at all stages, but tends to lessen towards the 45th day. The granular bodies are inside the disintegrating myelin sheath, and gradually fill the space made available by the degenerate axoplasm. The granular bodies are at first bulky and compact before breaking up and disappearing. The remains of the myelin sheath are then resorbed. Satellite cells occur from the 15th day onwards. The interest of the Marchi reaction for the tracing of nervous pathways is confirmed by these findings.     

La rue, l'adolescent

The adolescent is pushed toward the outside world, both in search of himself and in search of an encounter with the other. The adolescent is fascinated by the world outside. The street is the symbol of the new space that he alone can occupy. From the time that he leaves the intimate space of the family unit until he has been able to create another space, the street personifies the intermediary state between the two. It is there, in the course of his sexual development, that he encounters the sexual and carnal aspect of the other. It is also there that he exhibits his intimacy: his public display of the personal, ‘extimacy’, is also expressed through his use of the portable telephone. Violence is continually present both at a sexual level and in the adolescent’s relations with others, in an outside environment that he has to make a continual attempt to conquer. The world outside, i.e. the street, is the place where an encounter takes place with the opposite sex, but also with violence at a symbolic level. It is a necessary passage and training ground adulthood, and which requires complete commitment on the part of the individual concerned.