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Dysferlinopathy course and sportive activity: clues for possible treatment.

Dysferlinopathy course and sportive activity: clues for possible treatment.

Authors :
Angelini C
Peterle E
Gaiani A
Bortolussi L
Borsato C
Source :
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology [Acta Myol] 2011 Oct; Vol. 30 (2), pp. 127-32.
Publication Year :
2011

Abstract

LGMD2B 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.

Details

Language :
English
ISSN :
1128-2460
Volume :
30
Issue :
2
Database :
MEDLINE
Journal :
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology
Publication Type :
Academic Journal
Accession number :
22106716