Longitudinal evaluation of iron concentration and atrophy in the dentate nuclei in friedreich ataxia

Phillip G. D. Ward PhD, Ian H. Harding PhD, Thomas G. Close PhD, Louise A. Corben PhD, Martin B. Delatycki MBBS, FRACP, PhD, Elsdon Storey DPhil
Nellie Georgiou‐Karistianis PhD, Gary F. Egan PhD; Movement Disorders : Official Journal of the Movement Disorder Society [09 Jan 2019] DOI: 10.1002/mds.27606

Progressive dentate nucleus abnormalities are evident in vivo in Friedreich ataxia, and the rates of change of iron concentration and atrophy in these structures are sensitive to the disease stage. The findings are consistent with an increased rate of iron concentration and atrophy early in the disease, followed by iron accumulation and stable volume in later stages. This pattern suggests that iron dysregulation persists after loss of the vulnerable neurons in the dentate. The significant changes observed over a 2‐year period highlight the utility of quantitative susceptibility mapping as a longitudinal biomarker and staging tool

 Longitudinal evaluation of iron concentration and atrophy in the dentate nuclei in friedreich ataxia