Cerebral compensation during motor function in Friedreich ataxia: The IMAGE-FRDA study

Harding, I. H., Corben, L. A., Delatycki, M. B., Stagnitti, M. R., Storey, E., Egan, G. F. and Georgiou-Karistianis, N. (2017), Mov. Disord.. doi: 10.1002/mds.27023

Compensatory activity is evident in the cerebral cortex in individuals with Friedreich ataxia. Early compensation followed by later decline in premotor/ventral attention systems demonstrates capacity-limited neural reserve, while the additional engagement of higher order brain networks is indicative of compensatory task strategies. Network-level changes in cerebral brain function thus potentially serve to mitigate the impact of motor impairments in Friedreich ataxia.

Contemporary theories, models, and evidence of neural compensation and neural reserve firmly support the notion that neurofunctional changes underlying degenerative diseases are not only detrimental but also may be compensatory. This idea has driven a groundswell of research into novel therapeutic approaches designed to stimulate or maintain these compensatory cerebral processes through, for example, noninvasive brain stimulation, or cognitive therapies. Given the evidence that cerebral compensation may also operate in FRDA, these novel means of intervention represent exciting pathways for future research.

The present study motivates a reconceptualization of cerebral contributions to FRDA. In particular, cerebral alterations are likely not just secondary repercussions of cerebellar and spinal insults. Rather, cerebral changes may represent capacity limited compensatory processes operating at the network level of brain function, which serve to mitigate the behavioral impacts of progressive pathology. Critically, these novel findings inform development of new avenues of clinical intervention for FRDA.


Cerebral compensation during motor function in Friedreich ataxia: The IMAGE-FRDA study