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Both underweight and overweight occur in mitochondrial disorders, each with adverse health outcomes. We investigated the longitudinal evolution of anthropometric abnormalities in Friedreich ataxia and the hypothesis that both weight loss and weight gain are associated with faster disease progression.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Participants were drawn from the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS). Age\u2010 and sex\u2010specific BMI and height scores were calculated using the KIGGS\u2010BMI percentiles for children. Height correction was applied for scoliosis. Longitudinal data were analysed using linear mixed effects models and incremental standard deviation scores and growth mixture models identified subclasses with varying BMI trajectories.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Five hundred and forty\u2010three adults and fifty\u2010nine children were assessed for up to 5\u2009years. In children, severe underweight (26%), underweight (7%), severe short stature (16%) and short stature (23%) were common. The corrected BMI percentile was stable in children, although 48% had negative incremental BMI scores over 1\u2009year and 63% over 3\u2009years versus 10%\/year in a normal reference cohort. Overweight was common in adults (19%), with a slight increase in BMI over time. Longer GAA repeat size was linked to lower BMI in adults. Weight trajectory was not associated with ataxia progression in adults.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Significant anthropometric abnormalities were identified, with underweight and short stature prevalent in children and overweight in adults. These findings highlight the need for regular nutritional monitoring and interventions to manage underweight in children and promote healthy weight in adults.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.70011","type":"journal-article","created":{"date-parts":[[2025,1,11]],"date-time":"2025-01-11T09:00:44Z","timestamp":1736586044000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Longitudinal analysis of anthropometric measures over 5\u2009years in patients with Friedreich ataxia in the <scp>EFACTS<\/scp> natural history study"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1165-9153","authenticated-orcid":false,"given":"Stella Andrea","family":"Lischewski","sequence":"first","affiliation":[{"name":"Department of Neurology RWTH Aachen University  Aachen 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