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Migraine Hémiplégique Familiale Et Sporadique

A. Ducros
Published 2008 · Medicine

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Resume La migraine hemiplegique (MH) est une variete rare de migraine avec aura motrice. L’histoire familiale permet de distinguer les cas de migraine hemiplegique familiale (MHF) qui ont au moins un apparente au premier ou au deuxieme degre ayant les memes crises et les cas de migraine hemiplegique sporadique (MHS) qui n’ont aucun apparente atteint. La prevalence de la MH est d’une sur 10 000, avec une frequence egale des cas sporadiques et familiaux. Les crises typiques de MH comportent un deficit moteur toujours associe a au moins un autre signe de l’aura, les plus frequents etant les troubles sensitifs, visuels et du langage. Les symptomes dits basilaires sont egalement frequents (70 % des patients) : vertige, instabilite et acouphenes. Des crises severes de MH surviennent dans la MHF comme la MHS, et comportent une hemiplegie prolongee, une confusion ou un coma, une fievre et des crises comitiales. Le tableau clinique peut egalement inclure des signes cerebelleux permanents (nystagmus, ataxie, dysarthrie) et moins frequemment une epilepsie et une deficience intellectuelle. La MHF est la seule variete de migraine autosomique dominante. Les trois genes connus ( CACNA1A , ATP1A2 et SCNA1 ) codent pour des transporteurs ioniques, et les mutations sont responsables d’une hyperexcitabilite cerebrale. Un diagnostic genetique est possible par analyse de ces genes. Dans la majorite des cas, le pronostic est bon. Les traitements medicamenteux sont ceux utilises dans les autres varietes de migraine avec aura, avec une exception pour les triptans qui restent contre-indiques dans la MHF/MHS. Le traitement preventif par certains antiepileptiques, base sur la meilleure connaissance physiopathologique, semble prometteur.
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