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Brain structural damage in Friedreich ataxia
  1. R Della Nave (rdellanave{at}sirm.org)
  1. Radiodiagnostic Section, Department of Clinical Physiopathology, University of Florence, Italy
    1. A Ginestroni (andrea.ginestroni{at}virgilio.it)
    1. Radiodiagnostic Section, Department of Clinical Physiopathology, University of Florence, Italy
      1. M Giannelli (m.giannelli{at}ao-pisa.toscana.it)
      1. Medical Physics, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy
        1. C Tessa (ctessa{at}sirm.org)
        1. Radiology Unit, Versilia Hospital, Pietrasanta (Lucca), Italy
          1. E Salvatore (esalv{at}inwind.it)
          1. Department of Neurological Sciences, University of Naples Federico II, Naples, Italy
            1. F Salvi (fabrizio.salvi{at}ausl.bo.it)
            1. Department of Neurology, Ospedale di Bellaria, University of Bologna, Bologna, Italy
              1. M T Dotti (dotti{at}unisi.it)
              1. Department of Neurological and Behavioural Sciences, University of Siena, Italy
                1. G De Michele (demichel{at}unina.it)
                1. Department of Neurological Sciences, University of Naples Federico II, Naples, Italy
                  1. S Piacentini (piacentini{at}unifi.it)
                  1. Department of Neurological and Psychiatric Sciences, University of Florence, Italy
                    1. M Mascalchi (m.mascalchi{at}dfc.unifi.it)
                    1. Radiodiagnostic Section, Department of Clinical PHysiopathology, University of Florence, Italy

                      Abstract

                      Objective: Neuropathological descriptions of the brain in Friedreich ataxia (FRDA) were obtained before availability of the molecular genetic test for this disease. Voxel-based morphometry (VBM) enables an unbiased whole-brain quantitative analysis of differences in gray matter (GM) and white matter (WM) density.

                      Methods: We assessed with VBM the brain structural damage in 22 patients with genetically confirmed FRDA and 25 healthy controls. The results were correlated with the disease duration and the severity of the patients clinical deficits that was evaluated with the International Cerebellar Ataxia Rating Scale and Inherited Ataxia Clinical Rating Scale.

                      Results: VBM showed in FRDA patients a symmetric volume loss in dorsal medulla, infero-medial portions of the cerebellar hemispheres, the rostral vermis and in the dentate region. No volume loss in cerebral hemispheres was observed. The atrophy of the cerebellum and medulla correlated with the severity of the clinical deficit and disease duration.

                      Conclusions: In FRDA significant GM and WM loss is observed only in the cerebellum and dorsal medulla. These structural changes correlate with the severity of the clinical deficit and disease duration.

                      • MRI
                      • ataxia
                      • friedreich’s ataxia
                      • voxel-based morphometry

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