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United Leukodystropphy Foundation July 19, 2013 Kathy Zackowski, PhD, OT

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.

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United Leukodystropphy Foundation July 19, 2013 Kathy Zackowski, PhD, OT

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  1. Functional stages in AMN United LeukodystropphyFoundation July 19, 2013 Kathy Zackowski, PhD, OT Motion Analysis Laboratory Kennedy Krieger Institute Johns Hopkins University School of Medicine

  2. What we know: Central nervous system changes Our past studies show • Sensory loss (vibration) • feet >> hands • Progressive weakness • legs >> arms • secondary disuse • Spasticity, legs • Difficulty walking • Poor balance

  3. This is what we did: Purpose: To quantify symptom characteristics of AMN in men and women and determine which characteristic(s) have the greatest impact on function. • Subjects: 60 men, 80 women with AMN • Single test session • In the motion analysis laboratory at Kennedy Krieger • Clinical exam • Individualized exercise program

  4. What impairment effects movement performance most? Impairments Functional Tests

  5. Demographics *40 y/o male norm: 29.0 kg *50 y/o female norm: 18.1 kg

  6. Sit to Stand slower

  7. Standing Balance: eyes open feet apart Men Women Percentage values are those per group able to stand for 20 seconds

  8. Standing Balance: eyes closed feet together Men Women Percentage values are those per group able to stand for 20 seconds

  9. DynamicStandingBalance slower

  10. slower

  11. Endurance slower

  12. Exercises must match your ability

  13. Strength can be improved over one year

  14. Women

  15. Men

  16. Functional Measures

  17. 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

  18. Acknowledgements Neurogenetics: Jerry Raymond Kim Hollandsworth Katy Gibbons Lena Bezman Motion Analysis Lab: Jennifer Keller RhulMarasigan Joe Wang * All the people with AMN (and their families) who enthusiastically participated in this study

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