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