Presentation
Like its clinical presentation, the prognosis of patients with Becker muscular dystrophy is variable, with patients who are less affected ultimately dying of other diseases after a near-normal life span. Bushby K, Straub V. [emedicine.medscape.com]
Respiratoric
- Dyspnea
[…] develops Beyond imaging for scoliosis and dyspnea, imaging studies are of little help in making the diagnosis Dual energy x-ray absorptiometry estimates bone mineral density, as individuals with dystrophinopathies can have accelerated osteopenia/osteoporosis [emedicine.medscape.com]
Musculoskeletal
- Osteoporosis
Osteoporosis in Duchenne muscular dystrophy; its prevalence, treatment and prevention. Neuromuscul Disord. 2005 Jan. 15(1):72-9. [QxMD MEDLINE Link]. Apkon S, Coll J. Use of weekly alendronate to treat osteoporosis in boys with muscular dystrophy. [emedicine.medscape.com]
- Fracture
Beyond imaging for scoliosis and dyspnea, imaging studies are of little help in making the diagnosis Dual energy x-ray absorptiometry estimates bone mineral density, as individuals with dystrophinopathies can have accelerated osteopenia/osteoporosis/fracture [emedicine.medscape.com]
Neurologic
- Headache
[…] steady decline Cannot arise from the floor At approximately the same time as independent ambulation is most challenged, the forced vital capacity begins to gradually wane, leading to symptoms of nocturnal hypoxemia such as lethargy and early morning headaches [emedicine.medscape.com]
Workup
[…] waves Transthoracic echocardiography often reveals small ventricles with prolonged diastolic relaxation A Holter monitor is valuable for paroxysmal arrhythmias Cardiac MRI and gadolinium enhancement can better characterize cardiac tissue changes See Workup [emedicine.medscape.com]
Treatment
Antisense oligonucleotides in the treatment of Duchenne muscular dystrophy: Where are we now?. Neuromuscul Disord. 2005 Jun. 15(6):399-402. [QxMD MEDLINE Link]. Yan J, Feng J, Buzin CH. [emedicine.medscape.com]
Prognosis
Like its clinical presentation, the prognosis of patients with Becker muscular dystrophy is variable, with patients who are less affected ultimately dying of other diseases after a near-normal life span. Bushby K, Straub V. [emedicine.medscape.com]
Epidemiology
Epidemiology Occurrence Duchenne muscular dystrophy is by far the most common childhood-onset muscular dystrophy, afflicting 1 in 3500 boys with an overall prevalence of 63 cases per million. [emedicine.medscape.com]
Pathophysiology
Pathophysiology Dystrophin protein is integral to the structural stability of the myofiber. Without dystrophin, muscles are susceptible to mechanical injury and undergo repeated cycles of necrosis and regeneration. [emedicine.medscape.com]
Prevention
Perindopril preventive treatment on mortality in Duchenne muscular dystrophy: 10 years' follow-up. Am Heart J. 2007 Sep. 154(3):596-602. [QxMD MEDLINE Link]. Rhodes J, Margossian R, Darras BT et al. [emedicine.medscape.com]