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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