REPORT FROM FARA

Springfield, VA - April 12, 2010

In June 2009, the Friedreich’s Ataxia Research Alliance (FARA) announced the launch of a clinical trial of varenicline (Chantix®) to investigate both safety and potential efficacy in improving
neurological symptoms such as balance, coordination and sensory perception in a diverse group of adults diagnosed with Friedreich’s ataxia. This pilot study was initiated based on case reports
indicating that uncoordinated movements (ataxia) and balance problems in patients with various forms of ataxia had improved significantly when the individuals were taking Chantix. The double blind, randomized, placebo-controlled pilot study has been led by principal investigator Dr.
Theresa Zesiewicz, Professor of Neurology at the University of South Florida College of Medicine, and co-investigator Dr. David Lynch, Associate Professor of Neurology and Pediatrics at Children’s Hospital of Philadelphia. Chantix® is approved by the U.S. Food and Drug
Administration to help cigarette smokers stop smoking.

The Data Safety Monitoring Board (DSMB) responsible for monitoring the progress and associated risk/benefits of the study convened April 7th 2010 to review the data collected to date. Following that review, the DSMB recommended that the study be stopped as a result of concerns regarding safety and intolerability and insufficient evidence of efficacy. Twenty six subjects had been enrolled into the trial. The primary concern among those who were withdrawn was a worsening of gait and imbalance. FARA, the study sponsor, and the study investigators have decided to act upon the DSMB recommendation. Therefore, no further subjects will be enrolled and all current subjects in both the double blind and open label periods are being instructed to discontinue the study drug. Of note, this DSMB recommendation and this decision by FARA and the investigators pertain solely to this study, which involved only subjects with Friedreich’s ataxia.

At this time, the study investigators and subjects continue to be blinded regarding each subject’s treatment assignment as data collection is ongoing and subjects are still being evaluated and scheduled for final study visits. Once all of the final visits have been completed and data collected, the study team will be able to analyze the data and share the complete results. We all anxiously await this full review and FARA will keep the entire Friedreich’s ataxia community informed, as new information and analyses become available.