Measuring ataxia in children with Friedreich ataxia

Friedreich ataxia (FA) impacts many aspects of daily life, including walking, speech, arm function, and vision. Friedreich ataxia (FA) impacts many aspects of daily life, including walking, speech, arm function, and vision. The common element underscoring this impact is ataxia or incoordination. Ataxia in FA is measured by clinical rating scales such as the modified Friedreich’s Ataxia Rating Scale (mFARS). In children under the age of 12 years, measurement of ataxia may be difficult as the system that controls movement is still maturing, making it difficult to discern by a rating scale what movements are ataxic and what is related to normal maturation. It is important to develop valid measurements of ataxia in younger children so we can potentially monitor their response to new treatments. Dr. Corben has developed devices that use movement analysis technology to measure ataxia. She and her collaborators plan to evaluate the capacity of these devices called the Ataxia Instrumented Measure -Cup (AIM-C) and Ataxia Instrumented Measure – Pendant (AIM-P) to measure arm movement and balance in 12 children with and 12 children without FA. If they are able to demonstrate that the AIM-C and AIM-P can detect and measure ataxia in young children with FA, they will then conduct a longitudinal trial to evaluate if these devices are useful as outcome measures for young children with FA participating in clinical trials.