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Electrophysiological evidence for limited progression of the proprioceptive impairment in Friedreich ataxia

Cortico-kinematic coherence (CKC) is the coupling between movement-related proprioceptive inputs and the activity of the contralateral primary sensorimotor cortex activity recorded by magnetoencephalography or electroencephalography. CKC is an electrophysiological marker of spino-cortical proprioceptive function with high test-retest reliability. In a cohort of FRDA patients, the CKC levels were reduced by about 70% and correlated with the size of GAA1 triplet expansion and the age of symptoms onset suggesting that proprioceptive impairment in FRDA is genetically determined and scarcely progressive after symptoms onset. Here, the authors re-tested the same FRDA patients after 1 year to assess whether the CKC levels deteriorate over time or whether they remain stable as a sign of early developmental proprioceptive impairment.

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