- •A developmental stagnation in verbal span was detected in Duchenne muscular dystrophy.
- •Monitoring of neurocognitive development, academic achievement and learning is crucial.
- •Explorative results on the role of Dp140 in DMD neurocognitive performance.
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.
Abbreviation:AD(H)D (attention-deficit (hyperactivity) disorders), ASD (autism spectrum disorders), DMD (Duchenne muscular dystrophy), Dp140 (dystrophin protein 140), FSIQ (Full Scale Intelligence Quotient)
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Published online: January 07, 2020
Accepted: January 3, 2020
Received in revised form: December 18, 2019
Received: June 25, 2019
© 2020 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.