Hand dexterity and pyramidal dysfunction in Friedreich Ataxia, a finger tapping study

Gilles Naeije MD, PhD Antonin Rovai PhD Massimo Pandolfo MD Xavier De Tiège MD, PhD.; Mov Disord Clin Pract. Accepted Author Manuscript. doi:10.1002/mdc3.13126

This study provides evidence for a prominent involvement of pyramidal dysfunction in upper limb dexterity loss as well as a potential outcome measure for clinical studies in FRDA. FRDA patients had slower and more regular FT rate than controls. Eleven FRDA patients showed FT rate slowing. Those patients had longer disease duration and higher SARA scores. Seven patients with FT rate slowing had MEP and all displayed prolonged CMCT, while the four other patients with constant FT rate had normal CMCT.

Progression Characteristics in Friedreich's Ataxia: A 4-Year Observational Study

Reetz, Kathrin and Dogan, Imis and Hilgers, Ralf-Dieter and Giunti, Paola and Mariotti, Caterina and Nanetti, Lorenzo and Durr, Alexandra and Ewenczyk, Claire and Boesch, Sylvia and Nachbauer, Wolfgang and Klopstock, Thomas and Stendel, Claudia and Javier Rodríguez de Rivera Garrido, Francisco and Schöls, Ludger and Hayer, Stefanie and Klockgether, Thomas and Giordano, Ilaria and Didszun, Claire and Rai, Myriam and Pandolfo, Massimo and Schulz, Jörg B. and Group, EFACTS Study. "Preprints with The Lancetare are not Lancet publications or necessarily under review with a Lancet journal. These preprints are early stage research papers that have not been peer-reviewed". doi:10.2139/ssrn.3723615

Background: The European Friedreich’s Ataxia Consortium for Translational Studies (EFACTS) investigates the natural history of Friedreich’s ataxia (FRDA) in a prospective multinational registry study. We aimed to assess progression and metric characteristics of clinical rating scales based on longitudinal 4-year data.