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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Neurological sciences 21 (2000), S. S975 
    ISSN: 1590-3478
    Keywords: Key words Limb-girdle muscular dystrophy ; CT scan ; Clinical scale
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Knowledge of selective muscle-involvement is useful to the clinician. Therefore we investigated in sarcoglycanopathy and dysferlinopathy: (i) the correlation between clinical scale, MRC scale and CT findings and (ii) the muscles involved. Patients with a definite diagnosis of dysferlinopathy and sacroglycanopathy were tested for their clinical functions and muscle strength and assigned a functional grade. Nineteen muscles were evaluated by CT. In dysferlinopathy, distal lower-limb muscles are involved, while in sarcoglycanopathy proximal muscles are more affected. In both groups, muscles with MRC 〈3 often had severe abnormalities on CT imaging, while muscles with MRC 〉4 could be either normal or abnormal. Dysferlinopathy and sarcoglycanopathy have different and selective muscle involvement. This may affect certain types of functional activity. There is a possible relationship between CT findings and MRC grade in muscles with a low (〈3) MRC grade. Abnormality on CT scan may precede the clinical symptoms.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Neurological sciences 21 (2000), S. S919 
    ISSN: 1590-3478
    Keywords: Key words Dystrophinopathies ; Muscular dystrophies ; Therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Muscular dystrophies are a group of hereditary muscle disorders that often result in severe disability. Curative therapy is not yet available for muscular dystrophies (MD). In the near future, it is not expected that gene-replacement therapy will be available. Other strategies to decrease the rate of muscle necrosis and to increase strength in patients are necessary. Therefore the interest in symptomtic drug treatment has recently increased. A few trials have been performed on different types of muscular dystrophies, and some have generated postive results on muscle strength or muscle mass. We review the state of the art in therapy of MD and summarize the drugs that have been used and the evidence and results of such clinical trials.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
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