title: A new window on neurocognitive dysfunction in the childhood form of myotonic dystrophy type 1 (DM1).
abstract: Not much is known about the neurocognitive deficits in the childhood phenotypic expression of DM1. Twenty-four children and adolescents with no mental retardation were administered an extensive neuropsychological battery to investigate cognition in terms of memory, executive functions and visuo-spatial abilities. The results showed discrepancies between Wechsler's indexes with higher scores in Verbal Comprehension than Perceptive Organization and Speed of Processing. Memory assessment using Signoret's Memory Battery revealed a clear difference between verbal and visuospatial memory but no impairment between short and long-term memory. Concerning executive abilities, DM1 subjects showed greater deficits in processing speed than in mental flexibility, inhibition or working memory. This pattern of deficits could implicate a frontoparietal circuit in accordance with the neural networks involved in the adult form of DM1 and reopens the question of a continuum between childhood and adulthood neurocognitive impairments.
authors: Institut de Myologie, Groupe Hospitalier Pitié-Salpêtrière, AP-HP, 47-83, Boulevard de l'Hôpital, 75651 Paris Cedex 13, France. nathalie.angeard@parisdescartes.fr
authors: Nathalie
authors: N
authors: Angeard
authors: Aurélia
authors: A
authors: Jacquette
authors: Marcela
authors: M
authors: Gargiulo
authors: Hélène
authors: H
authors: Radvanyi
authors: Sylvain
authors: S
authors: Moutier
authors: Bruno
authors: B
authors: Eymard
authors: Delphine
authors: D
authors: Héron