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Optic Nerve Enhancement in Leber Hereditary Optic Neuropathy: Four Years Later

Vaphiades, Michael S. DO; Newman, Nancy J. MD

Journal of Neuro-Ophthalmology: March 2002 - Volume 22 - Issue 1 - p 66-67
Letters To The Editor

Department of Ophthalmology

University of Arkansas for Medical Sciences

Little Rock, Arkansas

Emory University School of Medicine

Atlanta, Georgia

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In 1999, the first report of a patient with optic nerve enhancement on orbital magnetic resonance imaging (MRI) in Leber hereditary optic neuropathy (LHON) was published (1). He was an 18-year-old man with a mutation at nucleotide position 3460. Visual acuity was count fingers OD, 20/80 OS, and ophthalmoscopy showed elevation with obscuration of the disc margins OU. Gadolinium-enhanced orbital fat-suppressed MRI showed optic nerve enhancement OU (Fig. 1 left). Four years later, visual acuity improved to 20/25 OD, 20/25–2 OS with pale optic discs. Orbital MRI was now normal without optic nerve enhancement (Fig. 1 right). Enhancement with gadolinium administration denotes disruption of the blood-brain barrier within the optic nerve (2). Evidently the blood-brain barrier, as documented by neuroimaging, recovered in the chronic phase of the disease.

FIG. 1.

FIG. 1.

Michael S. Vaphiades, DO

Nancy J. Newman, MD

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1. Vaphiades MS, Newman NJ. Optic nerve enhancement on orbital magnetic resonance imaging in Leber's hereditary optic neuropathy. J Neuroophthalmol 1999; 19:238–9.
2. Guy J, Mancuso A, Quisling RG, Beck R, Moster M. Gadolinium-DPTA-enhanced magnetic resonance imaging in optic neuropathies. Ophthalmology 1990; 97:592–600.
© 2002 Lippincott Williams & Wilkins, Inc.