{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T19:43:22Z","timestamp":1791315802977,"version":"4.3.1"},"reference-count":57,"publisher":"EDP Sciences","issue":"6-7","license":[{"start":{"date-parts":[[2022,6,29]],"date-time":"2022-06-29T00:00:00Z","timestamp":1656460800000},"content-version":"vor","delay-in-days":28,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Med Sci (Paris)"],"published-print":{"date-parts":[[2022,6]]},"abstract":"<jats:p>La panenc\u00e9phalite scl\u00e9rosante subaigu\u00eb (PESS), une complication tardive de la rougeole, est encore pr\u00e9sente lors d\u2019\u00e9pid\u00e9mies de cette maladie dues aux insuffisances de la vaccination. Apr\u00e8s un rappel historique, nous aborderons la physiopathologie de la PESS et l\u2019importance des crit\u00e8res diagnostiques. De nombreux travaux portant sur les param\u00e8tres de l\u2019immunit\u00e9 inn\u00e9e et sur ceux des r\u00e9ponses interf\u00e9ron tendent \u00e0 montrer une baisse de l\u2019activit\u00e9 de l\u2019immunit\u00e9 cellulaire au cours de cette maladie. Nous formulons ici plusieurs hypoth\u00e8ses s\u2019appuyant sur des publications concernant diff\u00e9rentes formes de la maladie\u00a0: cong\u00e9nitales, p\u00e9rinatales, formes \u00e0 incubation courte, semblables \u00e0 l\u2019enc\u00e9phalite aigu\u00eb \u00e0 inclusions (EAI), formes d\u2019\u00e9volution rapide, formes retrouv\u00e9es chez les immunod\u00e9prim\u00e9s ou chez l\u2019adulte. Des formes familiales ont \u00e9galement \u00e9t\u00e9 identifi\u00e9es, sugg\u00e9rant une origine g\u00e9n\u00e9tique. Selon la dur\u00e9e de la p\u00e9riode de latence entre rougeole et la PESS, deux groupes de patients ont \u00e9t\u00e9 individualis\u00e9s, incitant \u00e0 des analyses r\u00e9trospective et prospective des exomes de ces malades. La connaissance des g\u00e8nes participant \u00e0 la maladie devrait \u00eatre utile pour la compr\u00e9hension de la physiopathologie de la PESS mais aussi d\u2019autres infections neurologiques tardives dues \u00e0 des virus \u00e0 ARN.<\/jats:p>","DOI":"10.1051\/medsci\/2022081","type":"journal-article","created":{"date-parts":[[2022,6,29]],"date-time":"2022-06-29T08:05:05Z","timestamp":1656489905000},"page":"553-561","source":"Crossref","is-referenced-by-count":3,"title":["La panenc\u00e9phalite scl\u00e9rosante subaigu\u00eb de\u00a0la\u00a0rougeole"],"prefix":"10.1051","volume":"38","author":[{"given":"Pierre","family":"Lebon","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antoinette","family":"Gelot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shen-Ying","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Laurent","family":"Casanova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Jacques","family":"Hauw","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"250","published-online":{"date-parts":[[2022,6,29]]},"reference":[{"key":"R1","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1016\/j.coi.2019.03.008","volume":"59","author":"Zhang","year":"2019","journal-title":"Curr Opin Immunol"},{"issue":"7\u201316","key":"R2","first-page":"3","volume":"9","author":"Dawson","year":"1933","journal-title":"Am J Pathol"},{"key":"R3","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1136\/jnnp.8.3-4.101","volume":"8","author":"van Bogaert","year":"1945","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"R4","first-page":"454","volume":"113","author":"Bouteille","year":"1965","journal-title":"Etude anatomoclinique et ultrastructurale. 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