A possible role of dystrophin in neuronal excitability: A review of the current literature

Ruben G. F. Hendriksen*, Govert Hoogland*, Sandra Schipper*, Jos G. M. Hendriksen*, Johan S. H. Vles*, Marlien W. Aalbers*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

34 Citations (Web of Science)

Abstract

Duchenne muscular dystrophy (DMD) is a recessive hereditary form of muscular dystrophy caused by a mutation in the dystrophin gene on the X chromosome. Clinical observations show that in addition to progressive muscular degeneration, DMD is more often accompanied by neurocognitive symptoms and learning disabilities, especially in automatisation of reading, attention processes, and expressive language skills. Additionally, three studies reported a higher prevalence of epilepsy in DMD, suggesting that the absence of dystrophin might be related to increased CNS excitability. In this article, we aim to review current clinical and experimental evidence for a potential role of brain dystrophin in seizure generation.
Original languageEnglish
Pages (from-to)255-262
JournalNeuroscience and Biobehavioral Reviews
Volume51
DOIs
Publication statusPublished - Apr 2015

Keywords

  • Duchenne muscular dystrophy
  • Dystrophin
  • Epilepsy
  • Seizures

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