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Cognitive impairment in neuromuscular disorders
Author(s) -
D'Angelo Maria Grazia,
Bresolin Nereo
Publication year - 2006
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.20535
Subject(s) - myotonic dystrophy , spinal muscular atrophy , amyotrophic lateral sclerosis , medicine , cognition , muscular dystrophy , neuroscience , facioscapulohumeral muscular dystrophy , mitochondrial encephalomyopathies , physical medicine and rehabilitation , psychology , disease , pathology , mitochondrial myopathy , biology , biochemistry , mitochondrial dna , gene
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. Muscle Nerve, 2006

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