Muscular Dystrophy

Question 1

Types of muscular dystrophy

Answer 1

Becker’s
Congenital
Duchenne’s
Distal
Emery-Dreifus
Facioscapulohumeral
Limb-girdle
Myotonic
Oculopharyngeal

Question 2

Becker’s MD

Answer 2

Age of onset
- adolescence to early adulthood

Symptoms
- almost identical to Duchenne’s, but less severe and progresses slower
- survival into middle age

Question 3

Duchenne’s MD

Answer 3

Age of onset
- 2-6

Symptoms
- general muscle weakness and wasting
- affects pelvis, upper arms, and upper legs
- eventually involves all voluntary muscles
- survival beyond 20s is rare

• the worst form
• X linked
• Gower’s sign

Question 4

Facioscapulohumeral MD

Answer 4

Age of onset
- childhood to early adult

Symptoms
- facial muscle weakness
- weakness and wasting of shoulders and upper arms
- progression is slow with periods of rapid deterioration
- lifespan may be many decades after onset

Question 5

Limb-girdle MD

Answer 5

Age of onset
- late childhood to adulthood

Symptoms
- weakness and wasting of shoulder girdle and pelvic girdle
- progression is slow
- death is usually due to cardiopulmonary complications

Question 6

Gower’s sign

Answer 6

Question 7

Medical and surgical management of MD

Answer 7

No cure
Respiratory Maintenance
- Antibiotics-penicillin
- Monitor
Skin integrity and Pain management
Nutrition
- Eating
- Controlling weight
Drugs
- Corticosteroids – stop inflammatory process and slow destruction
- Immunosuppressants – stop inflammatory process and slow destruction
- Anticonvulsants
- Spinraza and Emflaza
Surgery to address symptomatic care
- Scoliosis repair, contracture release
- Tracheostomies as needed
Future options
- Gene replacement
- Genetic modification
- Drug based

Question 8

MD impact to occupation

Answer 8

Depends on type
Adaptive Track
ADL equipment
Health management-directing their care
Education
- Loss time, social, adaptations
Work
Play
Leisure and social