Neurocognitive deficits are frequently described in Duchenne muscular dystrophy (DMD), but it is unknown how these progress over time. Our aim was to longitudinally assess verbal span capacity and information processing speed in DMD and to explore a genotype-phenotype relation. Verbal span and processing speed scores were available of 28 males with DMD on two time-points, with a mean time interval of 28.34 months (SD = 16.09). The cohort contained of six patients missing only dystrophin isoform Dp427, sixteen missing Dp427 and Dp140, and six were undeterminable. A lower verbal span capacity was found at the first and second assessment, whereas processing speed was normal at both time-points. Post-hoc analyses suggested lower scores on verbal span and processing speed for patients missing Dp427 and Dp140. In DMD, a developmental stagnation in verbal span capacity, irrespective of normal processing speed, is detected through longitudinal follow-up. This appears more pronounced in patients missing Dp427 and Dp140.

中文翻译：

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杜氏肌营养不良症言语跨度和处理速度的纵向随访

Duchenne 型肌营养不良症 (DMD) 中经常描述神经认知缺陷，但尚不清楚这些缺陷如何随时间进展。我们的目的是纵向评估 DMD 中的言语跨度能力和信息处理速度，并探索基因型 - 表型关系。28 名患有 DMD 的男性在两个时间点的言语广度和处理速度得分，平均时间间隔为 28.34 个月（SD = 16.09）。该队列包含 6 名仅缺少肌营养不良蛋白同种型 Dp427、16 名缺少 Dp427 和 Dp140 的患者，以及 6 名无法确定。在第一次和第二次评估中发现较低的言语跨度能力，而处理速度在两个时间点都正常。事后分析表明，缺失 Dp427 和 Dp140 的患者在言语广度和处理速度方面得分较低。在 DMD 中，无论正常的处理速度如何，都可以通过纵向跟踪检测到语言跨度能力的发展停滞。这在缺少 Dp427 和 Dp140 的患者中显得更为明显。