Robotic Devices Give Accurate Clinical Assessments for Patients With Friedreich’s Ataxia

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by Kara Elam |

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A collaborative team of international researchers led by the Don Carlo Gnocchi Onlus Foundation, in Milan, Italy have shown that outcomes measured using robotic devices can improve the sensitivity of clinical evaluations for patients with Friedreich’s Ataxia (FA) in regards to dexterity and disease progression over time.  The study, entitled, “Robotic and clinical evaluation of upper limb motor performance in patients with Friedreich’s Ataxia: an observational study,” was recently published in the Journal of Neuroengineering and Rehabilitation.

The researchers used the InMotion Arm Robot, a robot designed for clinical neurological applications, to assess limb performance in a sample of 14 children and young adults with FA, and a comparison group of 18 healthy study participants matched by age and gender.  The aim of the clinical investigation was to evaluate the reliability and sensitivity of measurements of upper limb performance computed by using the robotic device, as well as to assess the correlation with clinical and functional markers of disease severity.

The primary results of the study showed that the robotic measurements were highly and reliably discriminative between healthy study participants and FA patients. Secondary study findings included:

  • Patients with FA exhibited slower movements as well as loss of accuracy and smoothness
  • Duration of Movement, Normalized Jerk, and Number of Submovements were the best discriminative indices, as they were directly and easily measurable and correlated with the status of the disease,

In a statement concerning the results of the study the authors wrote, “Our results suggest that outcome measures obtained by means of robotic devices can improve the sensitivity of clinical evaluations of patients’ dexterity and can accurately and efficiently quantify changes over time in clinical trials, particularly when functional scales appear to be no longer sensitive.”

About Friedreich’s Ataxia

FA is a rare inherited disorder that is caused by defects in an important gene that encodes for the protein, frataxin.  Frataxin is a protein that regulates the amount of iron in the mitochondria, and the defects caused by FA lead to iron overload and irreversible damage to the mitochondria’s DNA. Patients diagnosed with FA have a progressive degeneration of the central and peripheral nervous systems, impaired coordination and gait, and fatigue from energy deprivation and muscle loss. According to the National Institutes of Health (NIH), 20,000 people in the US and Europe have FA.  There are currently no FDA approved therapies for FA.