Cognitive impairment in neuromuscular disorders

Research output: Contribution to journalArticlepeer-review


Several studies have suggested the presence of central nervous system involvement manifesting as cognitive impairment in diseases traditionally confined to the peripheral nervous system. The aim of this review is to highlight the character of clinical, genetic, neurofunctional, cognitive, and psychiatric deficits in neuromuscular disorders. A high correlation between cognitive features and cerebral protein expression or function is evident in Duchenne muscular dystrophy, myotonic dystrophy (Steinert disease), and mitochondrial encephalomyopathies; direct correlation between tissue-specific protein expression and cognitive deficits is still elusive in certain neuromuscular disorders presenting with or without a cerebral abnormality, such as congenital muscular dystrophies, congenital myopathies, amyotrophic lateral sclerosis, adult polyglucosan body disease, and limb-girdle muscular dystrophies. No clear cognitive deficits have been found in spinal muscular atrophy and facioscapulohumeral dystrophy.

Original languageEnglish
Pages (from-to)16-33
Number of pages18
JournalMuscle and Nerve
Issue number1
Publication statusPublished - Jul 2006


  • Adult polyglucosan body disease
  • Amyotrophic lateral sclerosis
  • Becker muscular dystrophy
  • Cognitive impairment
  • Congenital muscular dystrophy
  • Duchenne muscular dystrophy
  • Facioscapulohumeral dystrophy
  • Limb-girdle muscular dystrophy

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)


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