Muscular Dystrophy

Question 1

What is muscular dystrophy?

Answer 1

• largest and most common group of PROGRESSIVE neuromuscular disorders
• genetic in origin
• 20-30 per 100,000 live births

Question 2

What are the four most common types of MD?

Answer 2

1. Duchenne’s
2. Becker’s
3. Facioscapulohumeral (FSH)
4. limb-girdle dystrophy (LGD)

Question 3

What are Duchenn’s and Becker’s?

Answer 3

X-linked inherited disorders

• males affected
• females carriers

Question 4

What is Facioscapulohumeral (FSH)?

Answer 4

autosomal dominant

Question 5

What is limb-girdle dystrophy (LGD)?

Answer 5

autosomal recessive

• both parents must be carriers
• 1 in 4 chance of child exhibiting disorder
• 2 in 4 chance of being a carrier

Question 6

What are clinical manifestations of Duchenne’s Muscular Dystrophy?

Answer 6

1. gradual proximal to distal muscle weakness and wasting
2. Gower’s sign (use arms to walk up body from the ground); frequent falls; difficulty climbing stairs; abnormal gain; lumbar lordosis
3. Trendelenberg sign secondary to hip abductor weakness
4. unable to ambulate by 10-12 y.o.
5. shoulder girdle weakness
6. respiratory involvement/failure and chronic heart failure
   PROGNOSIS: fatal by age 20

Question 7

What are the clinical manifestations of Becker’s MD?

Answer 7

1. resembles Duchenne’s but slower progression and longer life expectancy

Question 8

What are therapy interventions of becker and duchenne’s MD?

Answer 8

1. adaptive equipment
2. mobility
3. positioning/splinting
4. AROM/PROM
5. graded exercise programs