MUscular Dystrophy

Question 1

What is muscular dystrophy?

Answer 1

group of disorders that produce progressive degeneration and necrosis of SM fibers and eventual replacement with fat and CT

Question 2

what is the Muscular dystrophy disease of?

Answer 2

muscle tissue and prob do not involve NS

Question 3

what are the 3 main types of muscular dystrophy?

Answer 3

1. duchenne MD
2. beckers MD
3. mytonic MD

Question 4

what is muscular dystrophy considered to be associated with?

Answer 4

X- linked inherited disease by deletion of DNA seg or single gene defect on short arm of X-chromosome

Question 5

What protein encoded by cuchenne muscular dystrophy gene was identified?

Answer 5

dystrophin

Question 6

Where is dystrophin present?

Answer 6

in normal cells, and absent/low levels in disease

Question 7

In duchenne muscular dystrophy why do 1/3 of people show mental retardation?

Answer 7

because dystrophin protein is absent in brain

Question 8

who suffers from Duchene muscular dystrophy

Answer 8

only boys with an overall incidence of 1 in 3500 male births. any male child from a female carrier have a 50% risk

Question 9

what is the common progression of duchenne muscular dystrophy?

Answer 9

walking probs->
wheel chair
skeletal deformity
v limited use arms
vent at night
vent 24 hours
death

Question 10

clinical manifestations of muscular dystrophy

Answer 10

1. prog muscle weakness
2. pseudohypertrophy of calf muscles\3. contracturs and wasting of muscle leas to muscular atriophy and spinal deformity
3. cardiopulmonary involvement

Question 11

TM muscular dystrophy

Answer 11

phys therapy
assistive devices
med
surgery