Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors begin this general review with the pseudohypertrophies due either to an underlying extramuscular process, or to a focal lesion within the muscle, or else to miscellaneous interstitial infiltrates such as those found in cysticercosis, sarcoidosis, amylosis. True hypertrophy is most often observed in the course of muscular dystrophy. It is an usual finding in myopathies, and the prominent symptom of Thomsen disease ; it is the only symptom of hypertrophia musculorum vera and masseters hypertrophy ; it is associated with a peculiar deficit in the "Hereditary Distal Myopathy with Onset in Infancy"; it is a classical symptom of some endocrine myopathies (hypothyroidism and acromegaly). Paradoxically, true hypertrophy may, though very rarely, be encountered in the course of nervous system diseases, most often of the neuritic type : sciatica, hypertrophic neuritis, progressive spinal muscular atrophy. The mechanism of this very unusual muscular reaction remains unknown.
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PMID:[Muscular hypertrophy in clinical neurology (author's transl)]. 722 56

By detection of muscular fibrillation, a sign of motor nerve impairment, through electromyography, diseases of the motor nervous system can be distinguished from muscular disorders, malingering, and psychoneurotic states. The site of lesion can be determined and the degree of impairment and progress of recovery can be estimated. The method has been used successfully in amyotrophic lateral sclerosis, poliomyelitis, peripheral neuritis and neuronitis, and root compression syndrome, as also for indirect evidence in muscular dystrophy and other myopathic conditions.
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PMID:The value of electromyography in neurology. 1315 Feb 2

The Ras superfamily of guanosine-triphosphate (GTP)-binding proteins regulates a diverse spectrum of intracellular processes involved in inflammation and fibrosis. Farnesythiosalicylic acid (FTS) is a unique and potent Ras inhibitor which decreased inflammation and fibrosis in experimentally induced liver cirrhosis and ameliorated inflammatory processes in systemic lupus erythematosus, neuritis and nephritis animal models. FTS effect on Ras expression and activity, muscle strength and fibrosis was evaluated in the dy(2J)/dy(2J) mouse model of merosin deficient congenital muscular dystrophy. The dy(2J)/dy(2J) mice had significantly increased RAS expression and activity compared with the wild type mice. FTS treatment significantly decreased RAS expression and activity. In addition, phosphorylation of ERK, a Ras downstream protein, was significantly decreased following FTS treatment in the dy(2J)/dy(2J) mice. Clinically, FTS treated mice showed significant improvement in hind limb muscle strength measured by electronic grip strength meter. Significant reduction of fibrosis was demonstrated in the treated group by quantitative Sirius Red staining and lower muscle collagen content. FTS effect was associated with significantly inhibition of both MMP-2 and MMP-9 activities. We conclude that active RAS inhibition by FTS was associated with attenuated fibrosis and improved muscle strength in the dy(2J)/dy(2J) mouse model of congenital muscular dystrophy.
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PMID:The Ras antagonist, farnesylthiosalicylic acid (FTS), decreases fibrosis and improves muscle strength in dy/dy mouse model of muscular dystrophy. 2144 59