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

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

3. 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 and women and determine which characteristic(s) have the greatest impact on function. Purpose: To quantify symptom characteristics of AMN in men and women and determine which characteristic(s) have the greatest impact on function.

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

5. Gravity Lessened Anti- gravity Resistive

6. Variable MenWomen Strength group Gravity Lessened Anti-gravityResistive Gravity Lessened Anti-gravityResistive Flexor strength, kg 2.015.624.3*011.217.0* Sample size 5243122553 Age, y 394140505444 Vibration 19.913.310.419.98.46.0 Ankle DF ROM -727-427 *40 y/o male norm: 29.0 kg Demographics *50 y/o female norm: 18.1 kg

7. Sit to Stand slower

8. Standing Balance: eyes open feet apart Gravity LessenedAnti-gravityResistive Percentage values are those per group able to stand for 20 seconds Men Women

9. Gravity Lessened Anti-gravityResistive Standing Balance: eyes closed feet together Men Women Percentage values are those per group able to stand for 20 seconds

10. Dynamic Standing Balance slower

12. Endurance slower

13. Gravity Lessened Anti- gravity Resistive Exercises must match your ability

14. Strength can be improved over one year

15. Women

16. Men

17. Functional Measures

18. 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

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