25. Duchenne’s Dystrophy
Definition
Duchenne’s dystrophy is a chronic, progressive, severe pseudohypertrophic muscular dystrophy—the most commonly occurring type. It is characterized by increasing weakness of pelvic and shoulder girdle musculature, pseudohypertrophy of the muscles, then atrophy and lordosis. People with the disease have a peculiar, particular swinging gait (waddling gait) with the legs kept wide apart.
Diagnostic Criteria for Duchenne’s Dystrophy
• Absence of bowel or bladder dysfunction
• Hyperlordosis with wide-based gait
• Hypertrophy of weak muscles
• Progressive course over time
• Reduced muscle contractility and electrical stimulation in advanced disease
• Sensory distribution or febrile illness
• Weakness, with onset in the legs
Signs and Symptoms of Rhabdomyolysis
• Hyperphosphatemia
• Hyperuricemia
• Lactic acidosis
• Loss of P wave or sine wave
• Peaked T waves
• Prolonged P-R and QRS intervals
• Rising creatinine phosphokinase levels
• Severe hyperkalemia
• Tea-colored urine
Incidence
This form of muscular dystrophy occurs at the rate of 1:3300 to 1:3500 births, and affects males much more often than females.
Etiology
Buy Membership for Anesthesiology Category to continue reading. Learn more here
