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

F. Perez, O. Anne, S. Debruxelles, P. Menegon, V. Lambrecq, D. Lacombe, M.L. Martin-Negrier, B. Brochet, C. Goizet
Clinical Neurology and Neurosurgery. 2009-01-01; 111(1): 83-86
DOI: 10.1016/J.CLINEURO.2008.06.021

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1. Clin Neurol Neurosurg. 2009 Jan;111(1):83-6. doi: 10.1016/j.clineuro.2008.06.021.
Epub 2008 Oct 9.

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

Perez F(1), Anne O, Debruxelles S, Menegon P, Lambrecq V, Lacombe D,
Martin-Negrier ML, Brochet B, Goizet C.

Author information:
(1)CHU Bordeaux, Fédération des Neurosciences Cliniques, Hôpital Pellegrin,
Bordeaux, France.

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.

DOI: 10.1016/j.clineuro.2008.06.021
PMID: 18848389 [Indexed for MEDLINE]

Auteurs Bordeaux Neurocampus