Myopathy

Question 1

Classification of Progressive Muscular Dystrophy

Answer 1

Question 3

CP of Duchenne Muscle Dystrophy

Answer 3

Question 4

CP of Duchenne Muscle Dystrophy

• Which Side?

Answer 4

Bilateral & Symmetrical

Question 5

CP of Duchenne Muscle Dystrophy

• Proximal or Distal?

Answer 5

Proximal more than distal

Question 6

CP of Duchenne Muscle Dystrophy

• Symptoms

Answer 6

• Slow Walking, inability to Run, frequent Falling.
• Difficulty in Climbing stairs, Putting on and Taking off clothes.

Question 7

CP of Duchenne Muscle Dystrophy

• Shoulders

Answer 7

Sloping shoulders

Question 8

CP of Duchenne Muscle Dystrophy

• Scapulae

Answer 8

Winging of scapulae

Question 9

CP of Duchenne Muscle Dystrophy

• Spine & pelvis

Answer 9

• Exaggerated Lumbar Lordosis, protuberant abdomen, pelvis is tilted forward due to: Weak Glutei, so lordosis compensates this tilting and it disappears with sitting.

Question 10

CP of Duchenne Muscle Dystrophy

• Muscle Hypertrophy?

Answer 10

Question 11

CP of Duchenne Muscle Dystrophy

• Gait

Answer 11

Question 12

CP of Duchenne Muscle Dystrophy

• Signs

Answer 12

Question 13

CP of Duchenne Muscle Dystrophy

• Ambulation

Answer 13

Ambulation is lost at age of about 10 years.

Question 14

CP of Duchenne Muscle Dystrophy

• Reflexes

Answer 14

Reflexes are lost → With increasing weakness and progressive muscle atrophy

Question 15

CP of Duchenne Muscle Dystrophy

• No …….

Answer 15

No fasciculations, sphincter disturbance, sensory changes.

Question 16

CP of Duchenne Muscle Dystrophy

• which Muscles are involved?

Answer 16

Question 17

CP of Duchenne Muscle Dystrophy

• Speech

Answer 17

Delayed speech: development may occur.

Question 18

CP of Duchenne Muscle Dystrophy

• IQ

Answer 18

May be affected

Question 19

Investigations in Progressive Muscular Dystrophy

Answer 19

Question 20

Investigations in Progressive Muscular Dystrophy

• EMG

Answer 20

Diminished duration and amplitude of motor action potential.

Question 21

Investigations in Progressive Muscular Dystrophy

• Urine

Answer 21

• Diminished creatinine
• Appearance of creatine.

Question 22

Investigations in Progressive Muscular Dystrophy

• Serum Enzymes

Answer 22

Increase and that’s used to detect
- Female carriers and
- Preclinical diagnosis in males.

Question 23

Investigations in Progressive Muscular Dystrophy

• Muscle Bx

Answer 23

…

Question 24

TTT of Progressive Ms Dystophy

Answer 24

Question 25

TTT of **Progressive Ms Dystophy** - Steroids

Answer 25

It is now accepted that it prolongs ambulation

Question 26

TTT of **Progressive Ms Dystophy** - Myoblast Transfer

Answer 26

Injecting the muscle with a huge number of myoblasts will give a transient improvement (but cost exceeds benefit).

Question 27

Def of **Myasthenia Gravis**

Answer 27

- Disorder of transmission of the myoneural junction characterized clinically by fatigability on repetition of muscular activity which may be relieved by rest and or cholinergic drugs.

Question 28

**Myasthenia Gravis** is closely related to ......

Answer 28

- Thyrotoxicosis - Rheumatoid arthritis - M.S. (Multiple sclerosis) - SLE. (Systemic Lupus Erythematosus) - Biliary cirrhosis - Cancer

Question 29

CP of **Myasthenia Gravis**

Answer 29

...

Question 30

Onset of **Myasthenia Gravis**

Answer 30

Gradual

Question 31

Course of **Myasthenia Gravis**

Answer 31

progressive with remissions.

Question 32

CP of **Myasthenia Gravis** - site

Answer 32

Only skeletal muscles (smooth muscles are not involved).

Question 33

CP of **Myasthenia Gravis** - Symptoms

Answer 33

Question 34

CP of **Myasthenia Gravis** - Descending march

Answer 34

Question 35

CP of **Myasthenia Gravis** - Fluctiations in Myasthenia

Answer 35

Question 36

CP of **Myasthenia Gravis** - Muscle Bulk Affection

Answer 36

No

Question 37

CP of **Myasthenia Gravis** - smooth Musce affection

Answer 37

No

Question 38

CP of **Myasthenia Gravis** - Smile?

Answer 38

Retractors of mouth angle › elevators → Vertical or snarling smile.

Question 39

What induces remission of **Myasthenia Gravis**?

Answer 39

Pregnancy

Question 40

Dx of **Myasthenia Gravis**

Answer 40

Question 41

Dx of **Myasthenia Gravis** - Clinical Fatigue Test

Answer 41

Inducing fatigue by asking the patient to: - Count to 50 (will induce dysarthria) or - Maintain looking up (will precipitate ptosis).

Question 42

Dx of **Myasthenia Gravis** - Prostigmine test

Answer 42

1.5 mg Prostigmine + 0.5 mg Atropine IM → Improve myasthenia.

Question 43

Dx of **Myasthenia Gravis** - EMG

Answer 43

Repetitive stimulation test shows: - Diminished amplitude after repeated stimulation (decremental response).

Question 44

Dx of **Myasthenia Gravis** - Tensilon Test

Answer 44

2 mg IV followed, if no idiosyncrasy, by 8mg → improve myasthenia after a minute.

Question 45

Dx of **Myasthenia Gravis** - Most Sensitive Test

Answer 45

Detection of Anti-Ach R Antibodies

Question 46

Dx of **Myasthenia Gravis** - Chest tomogram

Answer 46

to detect Thymoma or Thymic Hyperplasia.

Question 47

Dx of **Myasthenia Gravis** - Thyroid Function Tests

Answer 47

...

Question 48

Dx of **Myasthenia Gravis** - tests for Collagen Disorders or Cx

Answer 48

...

Question 49

TTT of **Myasthenia Gravis**

Answer 49

Question 50

TTT of **Myasthenia Gravis** - Anticholinestrase

Answer 50

- Mestinon - Prostigmine: titrate until adequate response.

Question 51

TTT of **Myasthenia Gravis** - Prednisone

Answer 51

- 50 mg daily then taper to 5-10 mg per day as a maintenance dose.

Question 52

TTT of **Myasthenia Gravis** - Azathioprine

Answer 52

2mg/ Kg/day.

Question 53

TTT of **Myasthenia Gravis** - Plasmapharesis

Answer 53

to get rid of harmful antibodies.

Question 54

TTT of **Myasthenia Gravis** - IVIG

Answer 54

0.4 gm/ Kg/day divided over five successive days.

Question 55

TTT of **Myasthenia Gravis** - Thymectomy

Answer 55

Obligatory for Thymoma or Thymic hyperplasia.

Question 56

Types of **Crises in Myasthnia**

Answer 56

Question 57

Managment of **Crises**

Answer 57