united leukodystropphy foundation july 19, 2013 kathy zackowski, phd, ot
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Functional stages in AMN. United Leukodystropphy Foundation July 19, 2013 Kathy Zackowski, PhD, OT Motion Analysis Laboratory Kennedy Krieger Institute Johns Hopkins University School of Medicine. What we know:. Central nervous system changes. Our past studies show. - PowerPoint PPT PresentationTRANSCRIPT
United Leukodystropphy FoundationJuly 19, 2013
Kathy Zackowski, PhD, OT
Motion Analysis LaboratoryKennedy Krieger Institute
Johns Hopkins University School of Medicine
Functional stages in AMN
• Sensory loss (vibration) feet >> hands
• Progressive weaknesslegs >> arms
secondary disuse
• Spasticity, legs
• Difficulty walking
• Poor balance
Our past studies show
What we know:
Central nervous system changes
This is what we did:
Subjects: 60 men, 80 women with AMN
• Single test session
• In the motion analysis laboratory at
Kennedy Krieger
• Clinical exam
• Individualized exercise program
Purpose:To quantify symptom characteristics of AMN in men andwomen and determine which characteristic(s) have the greatest impact on function.
Purpose:To quantify symptom characteristics of AMN in men andwomen and determine which characteristic(s) have the greatest impact on function.
Impairments Functional Tests
What impairment effects movement performance most?
Variable Men WomenStrength
groupGravity
Lessened Anti-gravity Resistive Gravity Lessened Anti-gravity Resistive
Flexor strength,
kg2.0 15.6 24.3* 0 11.2 17.0*
Sample size 5 24 31 2 25 53
Age, y 39 41 40 50 54 44
Vibration 19.9 13.3 10.4 19.9 8.4 6.0
Ankle DFROM -7 2 7 -4 2 7
*40 y/o male norm: 29.0 kg
Demographics
*50 y/o female norm: 18.1 kg
Sit to Stand
slower
Standing Balance: eyes open feet apart
Percentage values are those per group able to stand for 20 seconds
Men
Women
Standing Balance: eyes closed feet together
Men
Women
Percentage values are those per group able to stand for 20 seconds
Dynamic Standing Balanceslower
slow
er
Endurancesl
ower
Exercises must match your ability
Strength can be improved over one year
Women
Men
Functional Measures
Summary
• Of all the impairments, weakness is the most prevalent feature effecting movement performance
• Weakness affects both men and women• Strength varies from person to person• Preliminary data shows strength is changeable• Important to individualize an exercise program
Acknowledgements
Motion Analysis Lab:Jennifer KellerRhul MarasiganJoe Wang
Neurogenetics:Jerry RaymondKim HollandsworthKaty GibbonsLena Bezman
* All the people with AMN (and their families) who enthusiastically participated in this study