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Clin Neurophysiol. 2019 May 11;130(8):1299-1310. doi: 10.1016/j.clinph.2019.05.003. [Epub ahead of print]

Altered neocortical tactile but preserved auditory early change detection responses in Friedreich ataxia.

Author information

1
Service of Neurology, CUB Hôpital Erasme, Université libre de Bruxelles (ULB), Brussels, Belgium; Laboratoire de Cartographie fonctionnelle du Cerveau (LCFC), UNI - ULB Neuroscience Institute, Université libre de Bruxelles (ULB), Brussels, Belgium. Electronic address: gilles.naeije@erasme.ulb.ac.be.
2
Laboratoire de Cartographie fonctionnelle du Cerveau (LCFC), UNI - ULB Neuroscience Institute, Université libre de Bruxelles (ULB), Brussels, Belgium; Magnetoencephalography Unit, Department of Functional Neuroimaging, Service of Nuclear Medicine, CUB Hôpital Erasme, Université libre de Bruxelles (ULB), Brussels, Belgium.
3
Laboratoire de Cartographie fonctionnelle du Cerveau (LCFC), UNI - ULB Neuroscience Institute, Université libre de Bruxelles (ULB), Brussels, Belgium; Laboratoire Cognition Langage et Développement, UNI - ULB Neuroscience Institute, Université libre de Bruxelles (ULB), Brussels, Belgium; BCBL, Basque Center on Cognition, Brain and Language, 20009 San Sebastian, Spain.
4
Service of Neurology, CUB Hôpital Erasme, Université libre de Bruxelles (ULB), Brussels, Belgium; Laboratoire de Neurologie Expérimentale, UNI - ULB Neuroscience Institute, Université libre de Bruxelles (ULB), Brussels, Belgium.

Abstract

OBJECTIVE:

To study using magnetoencephalography (MEG) the spatio-temporal dynamics of neocortical responses involved in sensory processing and early change detection in Friedreich ataxia (FRDA).

METHODS:

Tactile (TERs) and auditory (AERs) evoked responses, and early neocortical change detection responses indexed by the mismatch negativity (MMN) were recorded using tactile and auditory oddballs in sixteen FRDA patients and matched healthy subjects. Correlations between the maximal amplitude of each response, genotype and clinical parameters were investigated.

RESULTS:

Evoked responses were detectable in all FRDA patients but one. In patients, TERs were delayed and reduced in amplitude, while AERs were only delayed. Only tactile MMN responses at the contralateral secondary somatosensory cortex were altered in FRDA patients. Maximal amplitudes of TERs, AERs and tactile MMN correlated with genotype, but did not correlate with clinical parameters.

CONCLUSIONS:

In FRDA, theamplitude of tactile MMN responses at SII cortex are reduced and correlate with the genotype, whileauditory MMN responses are not altered.

SIGNIFICANCE:

Somatosensory pathways and tactile early change detection are selectively impaired in FRDA.

KEYWORDS:

Cerebellum; Friedreich ataxia; Magnetoencephalography; Mismatch negativity; Secondary somatosensory cortex

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