For many neuromuscular diseases (NMDs), cardiac disease represents a major cause of
morbidity and mortality. The management of cardiac disease in NMDs is made challenging …

Among the hereditary ataxias, autosomal recessive spinocerebellar ataxias comprise a
diverse group of neurodegenerative disorders. Clinical phenotypes vary from predominantly …

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 …

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

Objective: To examine the potential validity of performance measures and examination-
based scales in Friedreich ataxia (FA) by examining their correlation with disease …

Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments
characterized by an impaired transmission of sound from the cochlea to the brain. This …

Introduction: Friedreich ataxia (FRDA) is a progressive, inherited, neurodegenerative
disease for which there is currently no cure or approved treatment. FRDA is caused by …

Friedreich ataxia is an autosomal recessive neurodegenerative disorder characterized by
ataxia of all four limbs, dysarthria, and arreflexia. A variety of measures are currently used to …