Institut de Myologie, Groupe Hospitalier Pitié-Salpêtrière, AP-HP, 47-83, Boulevard de l'Hôpital, 75651 Paris Cedex 13, France.
Neuromuscul Disord. 2011 Jul;21(7):468-76. doi: 10.1016/j.nmd.2011.04.009. Epub 2011 May 17.
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.
关于 1 型肌营养不良症（DM1）儿童表型的神经认知缺陷，我们知之甚少。我们对 24 名无智力障碍的儿童和青少年进行了广泛的神经心理学测试，以调查他们在记忆、执行功能和视觉空间能力方面的认知能力。结果表明，韦氏智力测验索引中，言语理解的得分高于知觉组织和处理速度。使用 Signoret 记忆电池进行的记忆评估显示，言语记忆和视觉空间记忆之间存在明显差异，但短期和长期记忆之间没有差异。关于执行能力，DM1 患者在处理速度方面的缺陷比在心理灵活性、抑制或工作记忆方面更为明显。这种缺陷模式可能涉及额顶叶回路，与成人型 DM1 所涉及的神经网络一致，并重新提出了儿童和成人神经认知损伤之间是否存在连续性的问题。
