Height and body mass index in adult and pediatric patients with Friedreich ataxia


  • Friedreich ataxia (FRDA) has a substantial impact on weight gain and linear growth outcomes in adult and pediatric patients.

Why this matters

  • FRDA is a progressive, multi-system neurodegenerative movement disorder with onset typically between 10 and 15 years of age. FRDA is considered a mitochondrial disorder and characterized by reduced cellular production of ATP.

  • Metabolic stress in FRDA coupled with typical features of mitochondrial disorders, including failure to thrive, exercise intolerance, and excess fatigue, may affect height and weight parameters in patients with FRDA. It is important for clinicians to have a clear understanding of body mass index (BMI) and height outcomes and their relationship to clinical features of FRDA.