a congenital disorder characterized by progressive, bilateral wasting of skeletal muscles ( no neural or sensory deffects ) occurs during early childhood and death within 10 - 15 years.
S/S - 1st affects leg and pelvic muscles
( waddling gait, toe walking and lordosis. )
Muscle deteriation is progressive and contractures develop.
Tx - orthopedic appliances, PT, exercises, surgery.
Prognosis - poor, wheel chair bound by age 9 - 12 with death occurring from cardiac or respiratory complications.