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Démences fronto-temporales ou Maladie de Pick
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Les démences fronto-temporales ou DFT constituent un groupe de maladie neurodégénératives caractérisées par des troubles du comportement et du langage associés à une détérioration intellectuelle.
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En France, on estime qu’environ 6.000 personnes sont atteintes de DFT. Il s’agit de maladies rares qui font parties de la liste des maladies répertoriées par ORPHANET.
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La maladie
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Le début de la maladie est insidieux et l’évolution est progressive. On retrouve un trouble des comportements sociaux avec une difficulté à respecter les conventions sociales. Il y a une atteinte des processus de contrôle et de régulation des conduites
On retrouve également un trouble de l’humeur avec une perte de la compréhension des événements et de leur contexte.
Les DFT se différencient de la maladie d’Alzheimer principalement en ce que :
- dans la maladie d’Alzheimer, la perte de la mémoire ancienne et la difficulté à retenir une information nouvelle sont prédominantes, et les difficultés à se situer dans l’espace importantes.
- alors que dans les DFT, ce sont les comportements sociaux ainsi que le langage qui sont affectés en premier. La mémoire est peu affectée dans les premiers temps de la maladie.
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