title: Risk factors for osteoporosis, falls and fractures in hereditary myopathies and sporadic inclusion body myositis - A cross sectional survey.
abstract: BACKGROUND
  The risk of osteoporosis is known in myopathies requiring long-term steroid treatment and Pompe disease, but not in other hereditary myopathies or sporadic inclusion body myositis (sIBM).
  
  
  METHODS
  Risk factors of osteoporosis, laboratory parameters of bone metabolism, frequency of falls and fractures, walking ability, and pain were surveyed using questionnaires in 89 patients with sIBM and genetically confirmed myopathies facioscapulohumeral muscular dystrophy (FSHD), myotonic dystrophy types 1 and 2 (DM1, DM2), limb girdle muscular dystrophies (LGMD2A, LGMD2B, LGMD2I),MATR3myopathy, and oculopharyngeal muscular dystrophy (OPMD). Additionally laboratory parameters of bone metabolism were determined.
  
  
  RESULTS
  The mean age at examination per disease group ranged from 32 years in LGMD2A to 70 years in sIBM. Myopathies with a higher degree of walking impairment had a higher risk of falls (sIBM, LGMD2A, LGMD2B). At the time of examination 3.4% had a history of osteoporosis. The 25-OH D3 level was decreased in 20% of patients (and in 55% of patients with LGMDs), 57% of them were ambulatory. The 25-OH D3 level was significantly lower in patients with myopathies than in other neurological disorders (p < 0.001). 2.7 falls per year per person occurred. Fractures were reported in 6.8% of patients within the last year. They involved frequently the tibia bone. The pain score didn't correlate with either the walking disability (WGMS) score or the 25-OH D3 level.
  
  
  CONCLUSIONS
  The risk for osteoporosis and reduced 25-OH D3 level seems to be increased in wheelchair-bound patients with myopathy but also in patients with DM1 and autosomal-recessive myopathies.
authors.affiliation: Department of Neurology, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Str. 40, 06120 Halle (Saale), Germany.
authors.forename: F
authors.initials: F
authors.lastname: Danckworth
authors.affiliation: Villa Metabolica, Centre for Pediatric and Adolescent Medicine, Langenbeckstr. 2, 55131 Mainz, Germany.
authors.forename: N
authors.initials: N
authors.lastname: Karabul
authors.affiliation: Department of Neurology, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Str. 40, 06120 Halle (Saale), Germany.
authors.forename: A
authors.initials: A
authors.lastname: Posa
authors.affiliation: Department of Neurology, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Str. 40, 06120 Halle (Saale), Germany.
authors.forename: F
authors.initials: F
authors.lastname: Hanisch
keywords: 25-Hydroxyvitamin D; Calcium; Fracture; Limb girdle muscular dystrophy; Myopathy; Osteoporosis