A new window on neurocognitive dysfunction in the childhood form of myotonic dystrophy type 1 (DM1)

被引:41
作者
Angeard, Nathalie [1 ,2 ,3 ]
Jacquette, Aurelia [4 ]
Gargiulo, Marcela [1 ,4 ]
Radvanyi, Helene [5 ]
Moutier, Sylvain [6 ,7 ]
Eymard, Bruno [1 ]
Heron, Delphine [4 ]
机构
[1] Grp Hosp Pitie Salpetriere, AP HP, Inst Myol, F-75651 Paris 13, France
[2] Univ Paris 05, Grp Neuropsychol Dev, Boulogne, France
[3] INSERM, UMR 663, Boulogne, France
[4] Grp Hosp Pitie Salpetriere, Dept Genet, F-75651 Paris 13, France
[5] Hop Ambroise Pare, Boulogne, France
[6] Univ Caen, CEA, CNRS, UMR 6232,CI NAPS, F-75005 Paris, France
[7] Univ Paris 05, F-75005 Paris, France
关键词
Myotonic Dystrophy type 1 (DM1); Childhood phenotype; CTG repeat size; Executive functions; Visuospatial-constructive impairment; Attention-Deficit Disorder (ADD); Frontoparietal circuit; FACIAL EMOTION RECOGNITION; CTG REPEAT; COGNITIVE IMPAIRMENT; CHILDREN; PROFILE; PERSONALITY; EXPANSION; DEFICITS; PATTERN; SIZE;
D O I
10.1016/j.nmd.2011.04.009
中图分类号
R74 [神经病学与精神病学];
学科分类号
100204 [神经病学];
摘要
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. (C) 2011 Elsevier B.V. All rights reserved.
引用
收藏
页码:468 / 476
页数:9
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