1,720,994 research outputs found
Old and new therapeutic developments in steroid treatment in Duchenne muscular dystrophy
No full textMRI imaging in muscular dystrophies
No full textMuscle imaging is useful for defining muscle involvement in several dystrophies. Itsalso used to define the clinical progression and to study the natural history of disease,relevant to possible diagnostic of their pathogenesis and report a new clinical tool. .Specific pattern of muscle degeneration are seen and in some sequences (STIR)myoedema can be documented. Also fatty infiltration and connective tissue replacementcan be seen and atrophy of muscle can be investigated in upper and lower limbs.Specific muscular dystrophy pattern of Duchenne muscular dystrophy, limb girdlemuscular dystrophy, myotonic dystrophies, Hyaline body myopathy, congenital muscularmyopathies, Emery-Dreifuss dystrophy, and Bethlem myopathy are presented. Althoughtime consuming, imaging techniques appear to be useful in follow up of musculardystrophy patients. © 2013 Nova Science Publishers, Inc. All rights reserved
Old and new therapeutic developments in steroid treatment in Duchenne muscular dystrophy
No full textSteroids have been used since two decades and several trials were conducted to establish their efficacy in DMD patients with various regimens. The clinical outcomes showed increased function in the treated boys, and in a single trial with deflazacort, prolongation of ambulation but with different side effects. Steroids clinical efficacy is now established. The main concern is to increase steroid efficacy and decrease side effect and toxicity. A trial comparing daily prednisone, deflazacort and intermittent glucocorticoids (prednisone 10 days on/10 days off) (FOR-DMD) is starting under NIH grant. The primary outcomes will be muscle strength, forced vital capacity and patient/parents satisfaction
L’entrata del terzo. Persona, lingua, poesia, in P. Peterle e S. de Gaspari, a cura di, Archivi poetici: disgregazione e potenzialità del Novecento italiano, Rio de Janeiro, 7Letras, 2015, pp. 21-40
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Dysferlinopathy course and sportive activity: Clues for possible treatment
No full textLGMD2B is a frequent proximo-distal myopathy with rapid evolution after age 20. Exacerbating factors may be physical exercise and inflammation. There is very little information about the effect of sportive activity in LGMD2B, since eccentric exercise frequently results in muscle damage. LGMD2B has often an onset with myalgia and MRI imaging (STIR-sequences) shows myoedema. In a prolonged observational study of a series of 18 MM/LGMD2B patients we have studied the pattern of clinical and radiological evolution. The disease has an abrupt onset in the second decade and most patients perform sports before definite disease onset. On the basis of Gardner-Medwin and Walton scale, grade 4 is reached two years faster in patients who performed sports (over 1000 hours). Other considerations regarding pathogenetic mechanism and response to treatment show a poor response to immunosuppressive treatment of muscle inflammation. Preventing a strenuous physical activity should be recommended in patients with high CK and diagnosed or suspected to have dysferlin deficiency
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