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Dysferlinopathy course and sportive activity: clues for possible treatment.
Dysferlinopathy course and sportive activity: clues for possible treatment.
- 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.
- Subjects :
- Adolescent
Adult
Age of Onset
Disease Progression
Exercise
Female
Humans
Hyperkinesis metabolism
Hyperkinesis pathology
Hyperkinesis physiopathology
Immunosuppressive Agents therapeutic use
Inflammation metabolism
Inflammation pathology
Inflammation physiopathology
Magnetic Resonance Imaging
Male
Middle Aged
Muscle, Skeletal metabolism
Muscle, Skeletal pathology
Muscle, Skeletal physiopathology
Outcome Assessment, Health Care
Patient Selection
Risk Factors
Secondary Prevention
Severity of Illness Index
Tomography Scanners, X-Ray Computed
Creatine Kinase metabolism
Dystrophin metabolism
Hyperkinesis complications
Inflammation complications
Muscle, Skeletal injuries
Muscular Dystrophies, Limb-Girdle epidemiology
Muscular Dystrophies, Limb-Girdle etiology
Muscular Dystrophies, Limb-Girdle pathology
Muscular Dystrophies, Limb-Girdle physiopathology
Muscular Dystrophies, Limb-Girdle therapy
Sports
Subjects
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