Myopathies overview

Question 1

5 dystrophic myopathies

Answer 1

1. Duchenne
2. Becker
3. Fascioscapulohumeral
4. Limb-girdle
5. Myotonic

Question 2

In normal muscle, where is dystrophin found?

Answer 2

Sarcolemma

Question 3

What is function of dystrophin in normal muscle

Answer 3

Provides mechanical support for muscle membrane cytoskeleton

Question 4

Painless delayed relaxation of skeletal muscles following voluntary contraction

Answer 4

Myotonia

Question 5

What exacerbates myotonia

Answer 5

Cold

Question 6

What relieves myotonia

Answer 6

Exercise, dilantin, procainamide and CCBs

Question 7

Fixed deformity of the extremities a due to intrauterine hypomobility

Answer 7

Arthrogryposis

Question 8

Hallmark sign of myopathy is…

Answer 8

Inability to generate forceful contraction

Question 9

SNAP finding of myopathy

Answer 9

Normal

Question 10

CMAP finding of myopathy

Answer 10

Decreased amplitude, normal latencies and CV

Question 11

EMG finding of short duration, small amplitude MUAP is due to

Answer 11

Loss of muscle fibers

Question 12

Emg finding of long duration, large amplitude MUAP is due to…

Answer 12

Collateral sprouting