Highlights
- •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.
Abstract
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.
Keywords
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)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: January 07, 2020
Accepted:
January 3,
2020
Received in revised form:
December 18,
2019
Received:
June 25,
2019
Identification
Copyright
© 2020 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
