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Ann Neurol. 2002 Sep;52(3):374-7.

Leigh-like encephalopathy complicating Leber's hereditary optic neuropathy.

Author information

1
Service de Neurologie, Hôpital Sainte-Anne, Paris, France. funalot@chsa.broca.inserm.fr

Abstract

Leber's hereditary optic neuropathy is a mitochondrial disease caused by point mutations in mitochondrial DNA. It usually presents as severe bilateral visual loss in young adults. We report on a neurological disorder resembling Leigh syndrome, which complicated Leber's hereditary optic neuropathy in three unrelated male patients harboring mitochondrial DNA mutations at nucleotide positions 3460, 14459, and 14484, respectively. This Leigh-like encephalopathy appears to be associated with a much more severe outcome than isolated Leber's hereditary optic neuropathy.

PMID:
12205655
DOI:
10.1002/ana.10299
[Indexed for MEDLINE]

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