quiz #3- neuromuscular disorders

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quiz #3- neuromuscular disorders
2011-04-25 11:17:30
clinical neurology

neuromuscular disorders
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  1. What are two main types of neuromuscular disorders?
    • primary muscle disorder
    • neuromuscular junction disorder
  2. What are two main types of primary muscle disorders?
    • inherited muscular dystrophies
    • inflammatory diseases
  3. What are different types of inherited muscular dystrophies?
    • duchene muscular dystrophy
    • myotonic dystrophy
    • fascio-scapular humeral dystrophy
    • limb girdle dystrophy
  4. How do you get Duchene Muscular Dystrophy?
    • sex linked X chromosome
    • mother is a carrier, so mostly in males
    • if mother is carrier, 25% chance kid will get it
  5. What is the progression of duchene muscular dystrophy?
    • symptoms by 5 years
    • disabled by adolescence
    • death by 3rd decade (respiratory infection)
  6. What are some symptoms of duchene muscular dystrophy?
    • weak quads, sitting on Y ligament, increased lordosis, knee hyperextension
    • Gowers sign- use hands to straigten knees
    • pseudohypertrophy- calves appear muscular but fatty
  7. What happens to the muscles cells in duchene muscular dystrophy?
    • different sizes but surrounded by debri
    • will eventually deteriorate
  8. What is the EMG pattern of a dystrophic muscle
    • few action potentials
    • signals are going to muscles but muscles are dying so signals keep getting weaker
  9. What is myotonic dystrophy (basic)?
    • can't relax muscles
    • weakness
    • (shake hands for too long, because can't relax them to release)
  10. How do you get myotonic dystrophy?
    • inheritied
    • chomosome 19
    • dominant trait
  11. What are signs and symptoms of myotonic dystrophy and when do they appear?
    • appear in 3rd decade but slow progression
    • frontal balding
    • hachet faces- atrophy of temporalis muscle
    • ptosis and drooping mouth- weak facial muscles
    • cataracts
    • SCM wasting
    • gynecomastia
  12. What are other problems that come along with myotonic dystrophy?
    • heart arrhythmia
    • childbirth complications
    • swallowing
    • constipation/cramping
    • increase need for sleep
    • diabetes
    • anesthesia complications
  13. What are symptoms of Fascio-scauplar humeral dystrophy and when do they occur?
    • progressive weakening of face, scapula, arms
    • onset in 2nd decade
  14. How do you get Fascio-scapular humeral dystrophy and what are the chances?
    • inherited, chromosome 4 dominant
    • 1/20,000 people affected
  15. What are characteristics of limb girdle dystrophy?
    • austomal dominant and recessive
    • onset 10-30 yo
    • affects hip and shoulders
    • severity varies
  16. What is an example of an inflammatory disease?
    polymositis (rheumatic)
  17. What is polymositis and what are some of it's symptoms?
    • autoimmune, inflamed muscles
    • painful weakness, malaise, fever
    • progressive for 2 years
  18. How do you treat polymositis?
    • steroids
    • chemotharpy
    • if not treated could be fatal
  19. What is an example of neuromuscular juntion disorder?
    myasthenia gravis
  20. What is myasthenia gravis and its symptoms?
    • autoimmune disorder causing bad muscle weakness
    • ptosis
    • increasing weakness in extremities with activity
    • swallowing problems
  21. What is the treatment for myasthenia gravis?
    • no known cure, just have to control it
    • anticholinesterase drugs
    • thymectomy
    • corticosteroids
    • plasmapherisis