Karpatkin's class - Krasilovsky motor neuron disease

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  1. motor neuron disease affects what part of the spinal cord, 3 examples
    • AHC - anterior horn cells
    • ex: polio, SMA, ALS
  2. ALS def
    • loss of muscle growth and
    • degen of ant horn cell and lateral corticospinal tracts
  3. onset and presentation of ALS
    • onset: usually over 50 y/o
    • presentation: starts w localized distal weakness in one extremity, progresses to all four and bulbar musculature
    • fasciculations present w the atrophy
    • NO mental or sensory loss
  4. progression of ALS / life span
    death in 4-5 yrs, usually due to respiratory/swallowing problems
  5. 4 main symptoms in ALS, and percent of ALS pts who get them
    • UMN and LMN weakness: 100
    • atrophy: 93
    • fasciculations: 92 (not a problem if w/o other sx)
    • cramps: 50
    • spasticity, babinsky: 50
  6. which CN are involved in ALS
    • 7 - facial
    • 9 - glossopharangeal
    • 10 - vagus
    • 11 - accessory
    • 12 - hypoglossal
  7. def vs probable vs possible ALS
    • def: LMN & UMN signs in 3 regions w evidence of progression
    • prob: signs in 2 regions, some UMN signs above the site w LMN signs, w evidence of progression
    • poss: LMN or UMN in 1 region or UMN in 2, no sign of progression
  8. 5 things still intact w ALS
    • mental
    • sensation
    • sphincter
    • sexual
    • visual (and other sensory)
  9. categories in ALS rating scale
    • speech
    • salivation
    • swallowing
    • handwriting
    • cutting food/using utensils (diff scales for pts w/wo gastrotomy)
    • dressing & hygiene
    • turning in bed & adjusting bed clothes
    • walking
    • climbing stairs
    • breathing
  10. basic descriptions of the ALS stages
    • I: indep w mild weakness
    • II: mod weakness in a few muscle groups
    • III: amb but w AD, legs affected more than arms tho hands are weak, mild-mod limitations in ADL, difficult w sit --> stand, use AFO
    • IV: WC bound, severe weakness, can assist in transfers, ADL, some indep in UE ADL
    • V: WC w headrest, needs hoyer lift for transfers, pain due to lack of ROM, spasticity, cramps
    • VI: bed bound, max assist, resp distress, swallowing difficulties
  11. when is ALS painful?
    stage V, due to lack of ROM, which is due to spasticity and muscle cramps
  12. # 1 genetic cause of death in infants and toddlers?
    spinal muscular atrophy (SMA)
  13. SMA is caused by...
    defects in the survival motor neuron 1 gene that is cretical to health and survival of anterior horn cell (AHC)
  14. SMN1 and SMN2?
    SMN1 makes the good survival motore neuron protein. SMN2 is backup, but it produces lower levels of the SMN protein --> SMA
  15. SMA types I, II, III are what kind of inheritance?
    autosomal recessive
  16. onset of SMA type I
    early presentation
    acute infantile (Werdig-Hoffman's disease aka floppy baby syndrome)

    decreased intr-uterine mvmnts and floppy at birth OR normal, then weakness by 6 months
  17. prognosis for SMA type I
    death by 2-3 yrs 2/2 resp complications
  18. CPK in SMA type I
    • WNL
    • decreased interference pattern
  19. AHN is LMN or UMN?
  20. SMA type II onset
    • kids are normal at birth
    • symptomatic by age 7 months (could be 0-18 mo), then there's a motor decline
  21. SMA type II lifespan
    mid adult - 77% reach 20 y/o
  22. kyphosis, hip disloc in which SMA?
    type II
  23. eval tool for SMA (esp type II?)
    Modified Hammersmith Functional Motor Scale for SMA -- has 20 tasks, graded 0-2
  24. management of SMA type II?
    more about contractures, scoliosis, motion, than management for type I, which is more about respiration
  25. SMA type III - onset when? nickname?
    • Kugelberg-Welander or Late Onset Juvenile
    • onset on or after 18 mo, WC bound ~15 yrs after diagnosis
  26. SMA type III progression
    • slow
    • prox first
    • LE>UE
    • looks like DMD w Gower's and difficulty w stairs
    • CNs not involved
    • EMG showes denervation w reinnervation
  27. SMA type III - which muscle fibers are more involved?
    type I -- this is the only time we see more type I than II involvement
  28. SMA III management
    focus on activities, functional ability, vocation, strengthening, GCE
  29. SMA type IV
    • aduclt onset of prox weakness by 30 y/o
    • slowest progression
    • normal lifespan
  30. which SMAs get scoliosis and kyphoscoliosis
    I and II -- they lead to resp problems
Card Set:
Karpatkin's class - Krasilovsky motor neuron disease
2013-05-15 02:51:41

spring 2013
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