{"id":140,"date":"2025-04-28T17:42:32","date_gmt":"2025-04-28T17:42:32","guid":{"rendered":"https:\/\/honeydew-hyena-350809.hostingersite.com\/?page_id=140"},"modified":"2025-05-08T14:49:21","modified_gmt":"2025-05-08T14:49:21","slug":"about-spg3a","status":"publish","type":"page","link":"https:\/\/kidswithspg3a.com\/fr\/about-spg3a\/","title":{"rendered":"A propos de SPG3A"},"content":{"rendered":"
<\/div>\n\n\n\n

QU’EST-CE QUE LA PARAPL\u00c9GIE SPASTIQUE 3A ?<\/h1>\n\n\n\n

La SPG3A (\u00e9galement connue sous le nom de ATL1-HSP) est le type le plus courant de HSP autosomique dominante chez les enfants. L\u2019\u00e2ge moyen d\u2019apparition de la SPG3A est de 4 ans, et plus de 80 % des cas pr\u00e9sentent des sympt\u00f4mes avant l\u2019\u00e2ge de 10 ans.

La SPG3A est un sous-type d\u2019un groupe de maladies neurologiques rares appel\u00e9es Parapl\u00e9gies Spastiques H\u00e9r\u00e9ditaires. Les parapl\u00e9gies spastiques h\u00e9r\u00e9ditaires sont souvent divis\u00e9es en deux types : pures et complexes. Les types purs affectent uniquement les membres inf\u00e9rieurs, tandis que les types complexes impliquent \u00e9galement d\u2019autres parties du corps, comme les membres sup\u00e9rieurs et les muscles moteurs oraux, et peuvent pr\u00e9senter d\u2019autres sympt\u00f4mes tels que des crises convulsives et des alt\u00e9rations des fonctions intellectuelles.

Des mutations dans le g\u00e8ne ATL-1 (Atlastine-1) causent la SPG3A. Ces mutations entra\u00eenent probablement une activit\u00e9 anormale de l\u2019atlastine-1, ce qui alt\u00e8re le fonctionnement des neurones, y compris la distribution des mat\u00e9riaux \u00e0 l\u2019int\u00e9rieur de ces cellules. Ce manque de prot\u00e9ine atlastine-1 fonctionnelle peut \u00e9galement limiter la croissance des axones. Au sein des longs neurones des voies corticospinales, ces probl\u00e8mes peuvent entra\u00eener la mort cellulaire. En cons\u00e9quence, les neurones sont incapables de transmettre les impulsions nerveuses, en particulier vers d\u2019autres neurones et muscles des membres inf\u00e9rieurs. Cette fonction nerveuse alt\u00e9r\u00e9e conduit aux signes et sympt\u00f4mes de la parapl\u00e9gie spastique de type 3A.

La plupart des individus atteints de SPG3A (>95 %) ont un parent affect\u00e9. En d\u2019autres termes, ils h\u00e9ritent de la mutation g\u00e9n\u00e9tique d\u2019au moins un parent. Chez certains individus, la mutation survient \u00ab de novo \u00bb, c\u2019est-\u00e0-dire que le g\u00e8ne mute al\u00e9atoirement pendant le d\u00e9veloppement embryonnaire et n\u2019est pas h\u00e9rit\u00e9 d\u2019un parent.<\/p>\n\n\n\n

Source : Institut National de la Sant\u00e9, Biblioth\u00e8que Nationale de M\u00e9decine, MedlinePlus<\/p>\n\n\n\n

\"\"
Source : Parapl\u00e9gie Spastique H\u00e9r\u00e9ditaire : Une Mise \u00e0 Jour par le Dr Arun Meyyazhagan<\/figcaption><\/figure>","protected":false},"excerpt":{"rendered":"

QU’EST-CE QUE LA PARAPL\u00c9GIE SPASTIQUE 3A ? La SPG3A (\u00e9galement connue sous le nom de ATL1-HSP) est le type le plus courant de HSP autosomique dominante chez les enfants. L\u2019\u00e2ge moyen d\u2019apparition de la SPG3A est de 4 ans, et plus de 80 % des cas pr\u00e9sentent des sympt\u00f4mes avant l\u2019\u00e2ge de 10 ans. La […]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"ghostkit_customizer_options":"","ghostkit_custom_css":"","ghostkit_custom_js_head":"","ghostkit_custom_js_foot":"","ghostkit_typography":"","footnotes":""},"class_list":["post-140","page","type-page","status-publish","hentry"],"blocksy_meta":{"background":{"background_type":"image","background_pattern":"type-1","background_image":{"attachment_id":142,"x":0,"y":0,"url":"https:\/\/kidswithspg3a.com\/wp-content\/uploads\/2025\/04\/635bd3421cfdc0509b3cd923_Artboard-min.png"},"gradient":"linear-gradient(135deg,rgba(6,147,227,1) 0%,rgb(155,81,224) 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