The hereditary ataxias are a highly heterogeneous group of disorders phenotypically
characterized by gait ataxia, incoordination of eye movements, speech, and hand …

One hundred and fifty years since Nikolaus Friedreich's first description of the degenerative
ataxic syndrome which bears his name, his description remains at the core of the classical …

Objective Hereditary spastic paraplegias (HSPs) are genetically driven disorders with the
hallmark of progressive spastic gait disturbance. To investigate the phenotypic spectrum …

Background Friedreich's ataxia is a rare autosomal recessive neurodegenerative disorder.
Here we report cross-sectional baseline data to establish the biological and clinical …

Background and Objectives The understanding of the natural history of Friedreich ataxia
(FRDA) has improved considerably recently, but patterns of neurologic deterioration are not …

Background We assessed the clinimetric properties of ataxia rating scales and functional
tests, and made recommendations regarding their use. Methods A systematic literature …

Summary Background The European Friedreich's Ataxia Consortium for Translational
Studies (EFACTS) is a prospective international registry investigating the natural history of …

Objective We conducted a 6‐month, randomized, double‐blind, placebo‐controlled study to
assess safety, tolerability, and efficacy of deferiprone in Friedreich ataxia (FRDA). Methods …

Typical Friedreich ataxia (FRDA) is characterized by progressive ataxia with onset from early
childhood to early adulthood with mean age at onset from 10 to 15 years (range: age two …

Friedreich ataxia (FRDA) is due to a triplet repeat expansion in FXN, resulting in deficiency
of the mitochondrial protein frataxin. Resveratrol is a naturally occurring polyphenol …