Neurodegeneration in friedreich's ataxia is associated with a mixed activation pattern of the brain. A fMRI study

Andrea Ginestroni, Stefano Diciotti, Paolo Cecchi, Ilaria Pesaresi, Carlo Tessa, Marco Giannelli, Riccardo Della Nave, Elena Salvatore, Fabrizio Salvi, Maria Teresa Dotti, Silvia Piacentini, Andrea Soricelli, Mirco Cosottini, Nicola De Stefano, Mario Mascalchi

Research output: Contribution to journalArticle

22 Citations (Scopus)

Abstract

Friedreich's ataxia (FRDA) is associated with a distributed pattern of neurodegeneration in the spinal cord and the brain secondary to selective neuronal loss. We used functional MR Imaging (fMRI) to explore brain activation in FRDA patients during two motor-sensory tasks of different complexity, i.e. continuous hand tapping and writing of "8" figure, with the right dominant hand and without visual feedback. Seventeen FRDA patients and two groups of age-matched healthy controls were recruited. Task execution was monitored and recorded using MR-compatible devices. Hand tapping was correctly performed by 11 (65%) patients and writing of the "8" by 7 (41%) patients. After correction for behavioral variables, FRDA patients showed in both tasks areas of significantly lower activation in the left primary sensory-motor cortex and right cerebellum. Also left thalamus and right dorsolateral prefrontal cortex showed hypo-activation during hand tapping. During writing of the "8" task FRDA patients showed areas of higher activation in the right parietal and precentral cortex, globus pallidus, and putamen. Activation of right parietal cortex, anterior cingulum, globus pallidus, and putamen during writing of the "8" increased with severity of the neurological deficit. In conclusion fMRI demonstrates in FRDA a mixed pattern constituted by areas of decreased activation and areas of increased activation. The decreased activation in the primary motor cortex and cerebellum presumably reflects a regional neuronal damage, the decreased activation of the left thalamus and primary sensory cortex could be secondary to deafferentation phenomena, and the increased activation of right parietal cortex and striatum might have a possible compensatory significance.

Original languageEnglish
Pages (from-to)1780-1791
Number of pages12
JournalHuman Brain Mapping
Volume33
Issue number8
DOIs
Publication statusPublished - Aug 2012

Fingerprint

Friedreich Ataxia
Brain
Hand
Parietal Lobe
Globus Pallidus
Putamen
Motor Cortex
Thalamus
Cerebellum
Sensory Feedback
Somatosensory Cortex
Prefrontal Cortex
Spinal Cord
Research Design
Age Groups
Equipment and Supplies

Keywords

  • Functional MRI
  • Inherited ataxias
  • Motor function

ASJC Scopus subject areas

  • Clinical Neurology
  • Anatomy
  • Neurology
  • Radiology Nuclear Medicine and imaging
  • Radiological and Ultrasound Technology

Cite this

Ginestroni, A., Diciotti, S., Cecchi, P., Pesaresi, I., Tessa, C., Giannelli, M., ... Mascalchi, M. (2012). Neurodegeneration in friedreich's ataxia is associated with a mixed activation pattern of the brain. A fMRI study. Human Brain Mapping, 33(8), 1780-1791. https://doi.org/10.1002/hbm.21319

Neurodegeneration in friedreich's ataxia is associated with a mixed activation pattern of the brain. A fMRI study. / Ginestroni, Andrea; Diciotti, Stefano; Cecchi, Paolo; Pesaresi, Ilaria; Tessa, Carlo; Giannelli, Marco; Nave, Riccardo Della; Salvatore, Elena; Salvi, Fabrizio; Dotti, Maria Teresa; Piacentini, Silvia; Soricelli, Andrea; Cosottini, Mirco; De Stefano, Nicola; Mascalchi, Mario.

In: Human Brain Mapping, Vol. 33, No. 8, 08.2012, p. 1780-1791.

Research output: Contribution to journalArticle

Ginestroni, A, Diciotti, S, Cecchi, P, Pesaresi, I, Tessa, C, Giannelli, M, Nave, RD, Salvatore, E, Salvi, F, Dotti, MT, Piacentini, S, Soricelli, A, Cosottini, M, De Stefano, N & Mascalchi, M 2012, 'Neurodegeneration in friedreich's ataxia is associated with a mixed activation pattern of the brain. A fMRI study', Human Brain Mapping, vol. 33, no. 8, pp. 1780-1791. https://doi.org/10.1002/hbm.21319
Ginestroni, Andrea ; Diciotti, Stefano ; Cecchi, Paolo ; Pesaresi, Ilaria ; Tessa, Carlo ; Giannelli, Marco ; Nave, Riccardo Della ; Salvatore, Elena ; Salvi, Fabrizio ; Dotti, Maria Teresa ; Piacentini, Silvia ; Soricelli, Andrea ; Cosottini, Mirco ; De Stefano, Nicola ; Mascalchi, Mario. / Neurodegeneration in friedreich's ataxia is associated with a mixed activation pattern of the brain. A fMRI study. In: Human Brain Mapping. 2012 ; Vol. 33, No. 8. pp. 1780-1791.
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abstract = "Friedreich's ataxia (FRDA) is associated with a distributed pattern of neurodegeneration in the spinal cord and the brain secondary to selective neuronal loss. We used functional MR Imaging (fMRI) to explore brain activation in FRDA patients during two motor-sensory tasks of different complexity, i.e. continuous hand tapping and writing of {"}8{"} figure, with the right dominant hand and without visual feedback. Seventeen FRDA patients and two groups of age-matched healthy controls were recruited. Task execution was monitored and recorded using MR-compatible devices. Hand tapping was correctly performed by 11 (65{\%}) patients and writing of the {"}8{"} by 7 (41{\%}) patients. After correction for behavioral variables, FRDA patients showed in both tasks areas of significantly lower activation in the left primary sensory-motor cortex and right cerebellum. Also left thalamus and right dorsolateral prefrontal cortex showed hypo-activation during hand tapping. During writing of the {"}8{"} task FRDA patients showed areas of higher activation in the right parietal and precentral cortex, globus pallidus, and putamen. Activation of right parietal cortex, anterior cingulum, globus pallidus, and putamen during writing of the {"}8{"} increased with severity of the neurological deficit. In conclusion fMRI demonstrates in FRDA a mixed pattern constituted by areas of decreased activation and areas of increased activation. The decreased activation in the primary motor cortex and cerebellum presumably reflects a regional neuronal damage, the decreased activation of the left thalamus and primary sensory cortex could be secondary to deafferentation phenomena, and the increased activation of right parietal cortex and striatum might have a possible compensatory significance.",
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AU - Pesaresi, Ilaria

AU - Tessa, Carlo

AU - Giannelli, Marco

AU - Nave, Riccardo Della

AU - Salvatore, Elena

AU - Salvi, Fabrizio

AU - Dotti, Maria Teresa

AU - Piacentini, Silvia

AU - Soricelli, Andrea

AU - Cosottini, Mirco

AU - De Stefano, Nicola

AU - Mascalchi, Mario

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N2 - Friedreich's ataxia (FRDA) is associated with a distributed pattern of neurodegeneration in the spinal cord and the brain secondary to selective neuronal loss. We used functional MR Imaging (fMRI) to explore brain activation in FRDA patients during two motor-sensory tasks of different complexity, i.e. continuous hand tapping and writing of "8" figure, with the right dominant hand and without visual feedback. Seventeen FRDA patients and two groups of age-matched healthy controls were recruited. Task execution was monitored and recorded using MR-compatible devices. Hand tapping was correctly performed by 11 (65%) patients and writing of the "8" by 7 (41%) patients. After correction for behavioral variables, FRDA patients showed in both tasks areas of significantly lower activation in the left primary sensory-motor cortex and right cerebellum. Also left thalamus and right dorsolateral prefrontal cortex showed hypo-activation during hand tapping. During writing of the "8" task FRDA patients showed areas of higher activation in the right parietal and precentral cortex, globus pallidus, and putamen. Activation of right parietal cortex, anterior cingulum, globus pallidus, and putamen during writing of the "8" increased with severity of the neurological deficit. In conclusion fMRI demonstrates in FRDA a mixed pattern constituted by areas of decreased activation and areas of increased activation. The decreased activation in the primary motor cortex and cerebellum presumably reflects a regional neuronal damage, the decreased activation of the left thalamus and primary sensory cortex could be secondary to deafferentation phenomena, and the increased activation of right parietal cortex and striatum might have a possible compensatory significance.

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