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Abstract #1918

Brain white matter involvement in OPA1-dominant optic atrophy and Leber's hereditary optic atrophy: a DTI study

David Neil Manners 1 , Giovanni Rizzo 1,2 , Chiara La Morgia 1,2 , Caterina Tonon 1 , Claudia Testa 1 , Piero Barboni 3 , Emil Malucelli 4 , Maria Lucia Valentino 1,2 , Leonardo Caporali 1,2 , Daniela Strobbe 1,2 , Valerio Carelli 1,2 , and Raffaele Lodi 1

1 Dept of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Emilia Romagna, Italy, 2 IRCCS Istituto delle Scienze Neurologiche di Bologna, Emilia Romagna, Italy, 3 Studio Occulistico d'Azeglio, Bologna, Italy, 4 Dept of Pharmacy and Biotechnology, University of Bologna, Bologna, Emilia Romagna, Italy

Whole brain white matter of 19 OPA1-dominant optic atrophy (OPA1-DOA) patients was compared with 17 Leber's hereditary optic atrophy (LHON) patients and 19 healthy controls, using diffusion tensor imaging, to detect subtle structural alterations. LHON patients presented a preferential involvement of the optic radiation and of the acoustic radiation possibly due to trans-synaptic degeneration, with a protective effect of idebenone therapy. OPA1-DOA patients presented a widespread involvement supporting the view of a multisystemic disorder. The correlation between diffusivity abnormalities and the age of these patients also supports the hypothesis of a congenital and developmental disorder

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