Leber’s optic neuropathy associated with disseminated white matter disease: A case report and review

Abstract Leber’s hereditary optic neuropathy (LHON), a mitochondrial disease, is clinically characterized by a bilateral subacute loss of central vision consecutive to optic nerve involvement. In some cases of LHON, neurological features are reported including multiple sclerosis-like (MSL) phenotype...

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Bibliographic Details
Published in:Clinical neurology and neurosurgery 2009-01, Vol.111 (1), p.83-86
Main Authors: Perez, F, Anne, O, Debruxelles, S, Menegon, P, Lambrecq, V, Lacombe, D, Martin-Negrier, M.L, Brochet, B, Goizet, C
Format: Article
Language:English
Subjects:
Adult
Biological and medical sciences
Brain - pathology
DNA, Mitochondrial - genetics
Humans
Immunosuppressive treatment
Leber
LHON
Magnetic Resonance Imaging - methods
Male
Medical sciences
Mitochondrial disorder
MS-like
Multiple sclerosis
Multiple Sclerosis - etiology
Multiple Sclerosis - pathology
Mutation
Nerve Fibers, Myelinated - pathology
Neurology
Neurosurgery
Optic Atrophy, Hereditary, Leber - complications
Optic Atrophy, Hereditary, Leber - genetics
Optic Atrophy, Hereditary, Leber - pathology
Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases
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Summary:Abstract Leber’s hereditary optic neuropathy (LHON), a mitochondrial disease, is clinically characterized by a bilateral subacute loss of central vision consecutive to optic nerve involvement. In some cases of LHON, neurological features are reported including multiple sclerosis-like (MSL) phenotype. We report one additional male patient displaying LHON-MSL associated with the prevalent G11778A mutation and review the cases with expendable data published so far in the literature. We discuss the respective roles of inflammation and energetic metabolism dysregulation in the development of brain lesions. We propose to treat these patients early with both antioxidative and immunosuppressive drugs in order to avoid further handicap.
ISSN:0303-8467
1872-6968
DOI:10.1016/j.clineuro.2008.06.021