With disease-modifying drugs on the horizon for degenerative ataxias, ecologically valid,
finely granulated, digital health measures are highly warranted to augment clinical and …

Friedreich ataxia is the most frequent hereditary ataxia, with an estimated prevalence of 3–4
cases per 100,000 individuals. This autosomal-recessive neurodegenerative disease is …

Objective Friedreich ataxia (FA) is a progressive genetic neurodegenerative disorder with no
approved treatment. Omaveloxolone, an Nrf2 activator, improves mitochondrial function …

Objective Previous studies have demonstrated that suppression of Nrf2 in Friedreich ataxia
tissues contributes to excess oxidative stress, mitochondrial dysfunction, and reduced ATP …

Background Although cardiac dysfunction is widely accepted as the most common cause of
mortality in Friedreich ataxia (FRDA), no studies have evaluated this since the advent of …

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 …

Objective To assess the efficacy of idebenone on neurological function in patients with
Friedreich ataxia. Design Randomized, double-blind, placebo-controlled intervention trial …

Objective Friedreich ataxia (FRDA) is a progressive neurodegenerative disorder of adults
and children. This study analyzed neurological outcomes and changes to identify predictors …

Objective This study was undertaken to identify magnetic resonance (MR) metrics that are
most sensitive to early changes in the brain in spinocerebellar ataxia type 1 (SCA1) and type …