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 journalArticlepeer-review


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
Issue number8
Publication statusPublished - Aug 2012


  • Functional MRI
  • Inherited ataxias
  • Motor function

ASJC Scopus subject areas

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


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