Myopathy Flashcards
Classification of Progressive Muscular Dystrophy
CP of Duchenne Muscle Dystrophy
CP of Duchenne Muscle Dystrophy
- Which Side?
Bilateral & Symmetrical
CP of Duchenne Muscle Dystrophy
- Proximal or Distal?
Proximal more than distal
CP of Duchenne Muscle Dystrophy
- Symptoms
- Slow Walking, inability to Run, frequent Falling.
- Difficulty in Climbing stairs, Putting on and Taking off clothes.
CP of Duchenne Muscle Dystrophy
- Shoulders
Sloping shoulders
CP of Duchenne Muscle Dystrophy
- Scapulae
Winging of scapulae
CP of Duchenne Muscle Dystrophy
- Spine & pelvis
- Exaggerated Lumbar Lordosis, protuberant abdomen, pelvis is tilted forward due to: Weak Glutei, so lordosis compensates this tilting and it disappears with sitting.
CP of Duchenne Muscle Dystrophy
- Muscle Hypertrophy?
CP of Duchenne Muscle Dystrophy
- Gait
CP of Duchenne Muscle Dystrophy
- Signs
CP of Duchenne Muscle Dystrophy
- Ambulation
Ambulation is lost at age of about 10 years.
CP of Duchenne Muscle Dystrophy
- Reflexes
Reflexes are lost → With increasing weakness and progressive muscle atrophy
CP of Duchenne Muscle Dystrophy
- No …….
No fasciculations, sphincter disturbance, sensory changes.
CP of Duchenne Muscle Dystrophy
- which Muscles are involved?
CP of Duchenne Muscle Dystrophy
- Speech
Delayed speech: development may occur.
CP of Duchenne Muscle Dystrophy
- IQ
May be affected
Investigations in Progressive Muscular Dystrophy
Investigations in Progressive Muscular Dystrophy
- EMG
Diminished duration and amplitude of motor action potential.
Investigations in Progressive Muscular Dystrophy
- Urine
- Diminished creatinine
- Appearance of creatine.
Investigations in Progressive Muscular Dystrophy
- Serum Enzymes
Increase and that’s used to detect
- Female carriers and
- Preclinical diagnosis in males.
Investigations in Progressive Muscular Dystrophy
- Muscle Bx
…
TTT of Progressive Ms Dystophy
TTT of Progressive Ms Dystophy
- Steroids
It is now accepted that it prolongs ambulation
TTT of Progressive Ms Dystophy
- Myoblast Transfer
Injecting the muscle with a huge number of myoblasts will give a transient improvement (but cost exceeds benefit).
Def of Myasthenia Gravis
- 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.
Myasthenia Gravis is closely related to ……
- Thyrotoxicosis
- Rheumatoid arthritis
- M.S. (Multiple sclerosis)
- SLE. (Systemic Lupus Erythematosus)
- Biliary cirrhosis
- Cancer
CP of Myasthenia Gravis
…
Onset of Myasthenia Gravis
Gradual
Course of Myasthenia Gravis
progressive with remissions.
CP of Myasthenia Gravis
- site
Only skeletal muscles (smooth muscles are not involved).
CP of Myasthenia Gravis
- Symptoms
CP of Myasthenia Gravis
- Descending march
CP of Myasthenia Gravis
- Fluctiations in Myasthenia
CP of Myasthenia Gravis
- Muscle Bulk Affection
No
CP of Myasthenia Gravis
- smooth Musce affection
No
CP of Myasthenia Gravis
- Smile?
Retractors of mouth angle › elevators → Vertical or snarling smile.
What induces remission of Myasthenia Gravis?
Pregnancy
Dx of Myasthenia Gravis
Dx of Myasthenia Gravis
- Clinical Fatigue Test
Inducing fatigue by asking the patient to:
- Count to 50 (will induce dysarthria) or
- Maintain looking up (will precipitate ptosis).
Dx of Myasthenia Gravis
- Prostigmine test
1.5 mg Prostigmine + 0.5 mg Atropine IM → Improve myasthenia.
Dx of Myasthenia Gravis
- EMG
Repetitive stimulation test shows:
- Diminished amplitude after repeated stimulation (decremental response).
Dx of Myasthenia Gravis
- Tensilon Test
2 mg IV followed, if no idiosyncrasy, by 8mg → improve myasthenia after a minute.
Dx of Myasthenia Gravis
- Most Sensitive Test
Detection of Anti-Ach R Antibodies
Dx of Myasthenia Gravis
- Chest tomogram
to detect Thymoma or Thymic Hyperplasia.
Dx of Myasthenia Gravis
- Thyroid Function Tests
…
Dx of Myasthenia Gravis
- tests for Collagen Disorders or Cx
…
TTT of Myasthenia Gravis
TTT of Myasthenia Gravis
- Anticholinestrase
- Mestinon
- Prostigmine: titrate until adequate response.
TTT of Myasthenia Gravis
- Prednisone
- 50 mg daily then taper to 5-10 mg per day as a maintenance dose.
TTT of Myasthenia Gravis
- Azathioprine
2mg/ Kg/day.
TTT of Myasthenia Gravis
- Plasmapharesis
to get rid of harmful antibodies.
TTT of Myasthenia Gravis
- IVIG
0.4 gm/ Kg/day divided over five successive days.
TTT of Myasthenia Gravis
- Thymectomy
Obligatory for Thymoma or Thymic hyperplasia.
Types of Crises in Myasthnia
Managment of Crises