Motor
Duchenne Muscular Dystrophy
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Duchenne Muscular Dystrophy
See Also
Muscular Dystrophy
Myopathy Causes
Muscle Weakness in Children
(
Pediatric Neuromuscular Disorder
s)
Developmental Coordination Disorder
(
Clumsiness in Children
)
Epidemiology
Exclusively affects males
Pathophysiology
X-linked recessive inheritance
Symptoms
Difficulty performing
Exercise
s
Climbing
Running
Jumping
Hopping
Signs
Symmetric, progressive proximal
Muscle Weakness
Affects hip and
Shoulder
girdle
Palpable Muscle enlargement
Initial muscle hypertrophy
Later fat and connective tissue replace muscle
Associated Conditions
Tendon and muscle contractures
Kyphoscoliosis (progressive)
Impaired pulmonary function
Cardiomyopathy
Intellectual
Impairment
Course
Age 5 years: Onset of symptoms and signs
Age 8-10 years: Leg braces necessary for ambulation
Age 12 years: Nonambulatory
Age 25 years: Survival rare beyond this point
Labs
Muscle Enzymes elevated
Creatine Phosphokinase
(CPK) increased
Aldolase increased
Electromyogram
(EMG)
Myopathy
Muscle Biopsy
Necrotic muscle fibers with regeneration
Fatty replacement of muscle
Electrocardiogram
(EKG)
Cardiomyopathy
Deep
Q Wave
s across precordium
Complications
Respiratory Failure
Respiratory infections
Aspiration Pneumonia
Acute gastric dilatation
Congestive Heart Failure
Cardiac Arrhythmia
Management
Passive muscle
Stretching
Tenotomy
Bracing
Physiotherapy
Mechanical
Assistive Device
s
Avoid prolonged immobility
Reference
Mendell in Wilson (1991) Harrison's IM, p. 2112
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