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Subacute sclerosing panencephalitis, a late complication of measles, is still present during epidemics of this disease due to insufficient vaccination. After a historical review, the importance of the diagnostic criteria and the pathophysiology of SSEP are discussed. Numerous studies on the parameters of innate immunity and interferon responses tend to show a decrease in the activity of cellular immunity. Several hypotheses are formulated based on the publications of the different forms of the disease: Congenital, perinatal, forms with short incubation similar to acute inclusion encephalitis (AIE), rapidly evolving forms, forms of the immunocompromised, or even adults. Familial forms have been identified, suggesting a genetic cause. Based on the duration of the latency period, two groups have been individualized, prompting a retrospective and prospective analysis of the exomes of these patients. The knowledge of the genes involved should be useful for the understanding of the pathophysiology of SSPE and other late neurological RNA virus infections.La panencéphalite sclérosante subaiguë de la rougeole - Une maladie mortelle encore présente et toujours mystérieuse.La panencéphalite sclérosante subaiguë (PESS), une complication tardive de la rougeole, est encore présente lors d’épidémies de cette maladie dues aux insuffisances de la vaccination. Après un rappel historique, nous aborderons la physiopathologie de la PESS et l’importance des critères diagnostiques. De nombreux travaux portant sur les paramètres de l’immunité innée et sur ceux des réponses interféron tendent à montrer une baisse de l’activité de l’immunité cellulaire au cours de cette maladie. Nous formulons ici plusieurs hypothèses s’appuyant sur des publications concernant différentes formes de la maladie : congénitales, périnatales, formes à incubation courte, semblables à l’encéphalite aiguë à inclusions (EAI), formes d’évolution rapide, formes retrouvées chez les immunodéprimés ou chez l’adulte. Des formes familiales ont également été identifiées, suggérant une origine génétique. Selon la durée de la période de latence entre rougeole et la PESS, deux groupes de patients ont été individualisés, incitant à des analyses rétrospective et prospective des exomes de ces malades. La connaissance des gènes participant à la maladie devrait être utile pour la compréhension de la physiopathologie de la PESS mais aussi d’autres infections neurologiques tardives dues à des virus à ARN.

Clinical decision support systems combine knowledge and data from a variety of sources, represented by quantitative models based on stochastic methods, or qualitative based rather on expert heuristics and deductive reasoning. At the same time, case-based reasoning (CBR) memorizes and returns the experience of solving similar problems. The cooperation of heterogeneous clinical knowledge bases (knowledge objects, semantic distances, evaluation functions, logical rules, databases...) is based on medical ontologies. A multi-agent decision support system (MADSS) enables the integration and cooperation of agents specialized in different fields of knowledge (semiology, pharmacology, clinical cases, etc.). Each specialist agent operates a knowledge base defining the conduct to be maintained in conformity with the state of the art associated with an ontological basis that expresses the semantic relationships between the terms of the domain in question. Our approach is based on the specialization of agents adapted to the knowledge models used during the clinical steps and ontologies. This modular approach is suitable for the realization of MADSS in many areas., Comment: in French