Cognitive impairment in neuromuscular disorders.

Abstract:

: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.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

D'Angelo MG,Bresolin N

doi

10.1002/mus.20535

subject

Has Abstract

pub_date

2006-07-01 00:00:00

pages

16-33

issue

1

eissn

0148-639X

issn

1097-4598

journal_volume

34

pub_type

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