Myopathy Flashcards

1
Q

Classification of Progressive Muscular Dystrophy

A
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2
Q
A
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3
Q

CP of Duchenne Muscle Dystrophy

A
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4
Q

CP of Duchenne Muscle Dystrophy

  • Which Side?
A

Bilateral & Symmetrical

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5
Q

CP of Duchenne Muscle Dystrophy

  • Proximal or Distal?
A

Proximal more than distal

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6
Q

CP of Duchenne Muscle Dystrophy

  • Symptoms
A
  • Slow Walking, inability to Run, frequent Falling.
  • Difficulty in Climbing stairs, Putting on and Taking off clothes.
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7
Q

CP of Duchenne Muscle Dystrophy

  • Shoulders
A

Sloping shoulders

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8
Q

CP of Duchenne Muscle Dystrophy

  • Scapulae
A

Winging of scapulae

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9
Q

CP of Duchenne Muscle Dystrophy

  • Spine & pelvis
A
  • Exaggerated Lumbar Lordosis, protuberant abdomen, pelvis is tilted forward due to: Weak Glutei, so lordosis compensates this tilting and it disappears with sitting.
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10
Q

CP of Duchenne Muscle Dystrophy

  • Muscle Hypertrophy?
A
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11
Q

CP of Duchenne Muscle Dystrophy

  • Gait
A
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12
Q

CP of Duchenne Muscle Dystrophy

  • Signs
A
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13
Q

CP of Duchenne Muscle Dystrophy

  • Ambulation
A

Ambulation is lost at age of about 10 years.

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14
Q

CP of Duchenne Muscle Dystrophy

  • Reflexes
A

Reflexes are lost → With increasing weakness and progressive muscle atrophy

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15
Q

CP of Duchenne Muscle Dystrophy

  • No …….
A

No fasciculations, sphincter disturbance, sensory changes.

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16
Q

CP of Duchenne Muscle Dystrophy

  • which Muscles are involved?
A
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17
Q

CP of Duchenne Muscle Dystrophy

  • Speech
A

Delayed speech: development may occur.

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18
Q

CP of Duchenne Muscle Dystrophy

  • IQ
A

May be affected

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19
Q

Investigations in Progressive Muscular Dystrophy

A
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20
Q

Investigations in Progressive Muscular Dystrophy

  • EMG
A

Diminished duration and amplitude of motor action potential.

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21
Q

Investigations in Progressive Muscular Dystrophy

  • Urine
A
  • Diminished creatinine
  • Appearance of creatine.
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22
Q

Investigations in Progressive Muscular Dystrophy

  • Serum Enzymes
A

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

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23
Q

Investigations in Progressive Muscular Dystrophy

  • Muscle Bx
A

24
Q

TTT of Progressive Ms Dystophy

A
25
Q

TTT of Progressive Ms Dystophy

  • Steroids
A

It is now accepted that it prolongs ambulation

26
Q

TTT of Progressive Ms Dystophy

  • Myoblast Transfer
A

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

27
Q

Def of Myasthenia Gravis

A
  • 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.
28
Q

Myasthenia Gravis is closely related to ……

A
  • Thyrotoxicosis
  • Rheumatoid arthritis
  • M.S. (Multiple sclerosis)
  • SLE. (Systemic Lupus Erythematosus)
  • Biliary cirrhosis
  • Cancer
29
Q

CP of Myasthenia Gravis

A

30
Q

Onset of Myasthenia Gravis

A

Gradual

31
Q

Course of Myasthenia Gravis

A

progressive with remissions.

32
Q

CP of Myasthenia Gravis

  • site
A

Only skeletal muscles (smooth muscles are not involved).

33
Q

CP of Myasthenia Gravis

  • Symptoms
A
34
Q

CP of Myasthenia Gravis

  • Descending march
A
35
Q

CP of Myasthenia Gravis

  • Fluctiations in Myasthenia
A
36
Q

CP of Myasthenia Gravis

  • Muscle Bulk Affection
A

No

37
Q

CP of Myasthenia Gravis

  • smooth Musce affection
A

No

38
Q

CP of Myasthenia Gravis

  • Smile?
A

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

39
Q

What induces remission of Myasthenia Gravis?

A

Pregnancy

40
Q

Dx of Myasthenia Gravis

A
41
Q

Dx of Myasthenia Gravis

  • Clinical Fatigue Test
A

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

42
Q

Dx of Myasthenia Gravis

  • Prostigmine test
A

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

43
Q

Dx of Myasthenia Gravis

  • EMG
A

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

44
Q

Dx of Myasthenia Gravis

  • Tensilon Test
A

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

45
Q

Dx of Myasthenia Gravis

  • Most Sensitive Test
A

Detection of Anti-Ach R Antibodies

46
Q

Dx of Myasthenia Gravis

  • Chest tomogram
A

to detect Thymoma or Thymic Hyperplasia.

47
Q

Dx of Myasthenia Gravis

  • Thyroid Function Tests
A

48
Q

Dx of Myasthenia Gravis

  • tests for Collagen Disorders or Cx
A

49
Q

TTT of Myasthenia Gravis

A
50
Q

TTT of Myasthenia Gravis

  • Anticholinestrase
A
  • Mestinon
  • Prostigmine: titrate until adequate response.
51
Q

TTT of Myasthenia Gravis

  • Prednisone
A
  • 50 mg daily then taper to 5-10 mg per day as a maintenance dose.
52
Q

TTT of Myasthenia Gravis

  • Azathioprine
A

2mg/ Kg/day.

53
Q

TTT of Myasthenia Gravis

  • Plasmapharesis
A

to get rid of harmful antibodies.

54
Q

TTT of Myasthenia Gravis

  • IVIG
A

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

55
Q

TTT of Myasthenia Gravis

  • Thymectomy
A

Obligatory for Thymoma or Thymic hyperplasia.

56
Q

Types of Crises in Myasthnia

A
57
Q

Managment of Crises

A