united leukodystropphy foundation july 19, 2013 kathy zackowski, phd, ot

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