Titre :
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Brain alteration, cognition and psychological aspects in adult-onset myotonic dystrophy type 1 and type 2
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Auteurs :
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IXèmes Journées Annuelles de la Société française de Myologie (SFM) (3-5 novembre 2011; Angers) ;
Meola G
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Type de document :
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Article
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Année de publication :
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2011
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Pages :
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p. 24
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Langues:
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Anglais
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Mots-clés :
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atrophie cérébrale
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cognition
;
colloque
;
dépression
;
dystrophie myotonique de type 1
;
dystrophie myotonique de type 2
;
imagerie médicale
;
perspective de recherche
;
revue de la littérature
;
trouble psychologique
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Résumé :
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Myotonic dystrophy type 1 and 2 are variable autosomal dominant inherited predominantly neuromuscular disorders with multisystemic manifestations including cerebral involvement. Recent studies of central nervous system in myotonic dystrophies and resulting cognitive and psychological consequences have increased, especially in myotonic dystrophy type 1. Neuroimaging in both types demonstrated generalized atrophy, subcortical white matter lesions or reduced cerebral blood flow and hypometabolism suggesting malfunctions of prefrontal-subcortical circuits, leading to similar executive deficits in both disorders. Critical literature review reveals no general cognitive impairment in either adult onset Myotonic dystrophy type 1 or 2. Apathy and higher depression scores especially in type 1 are not well differentiated being more likely reactive disorders or resulting directly from brain alterations. Indicators of specific personality traits have to be identified. Future research should focus on evaluating relevance of cerebral involvement in everyday life, psychosocial consequences and implications for treatment.
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Voir aussi :
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