He initially presented with acute onset of somnolencefollowing flu-like symptoms for one week. MRI showed symmetric abnormalities in the external capsules, claustra, lateral geniculate nuclei, thalami, hippocampi, brainstem, cerebral cortex, and hypothalamus. Cerebrospinal fluid study showed WBC 9/uL, protein 83 mg/dL, and glucose 52 mg/dL. His visual acuity was 20/40 in the right eye (OD), and 20/20 in the left eye (OS). A relative pupillary defect (rAPD) was absent in both eyes (OU). Extraocular movements were full OU without nystagmus or hypometric saccades. He had 10 prism diopters of exotropia. Bilateral optic atrophy was present. Goldmann visual field showed binasal defects. Further study ruled out keratoconus, retinal infarcts, and internal carotid artery narrowing. Genetic study was positive for a RANBP2 mutation, which lead to the diagnosis of ANE1. At 17 years of age, he continued to have decreased near VA in the right eye, dyschromatopsia OS>OD, absent rAPD and a comitant large angle exotropia. There was subtle end gaze nystagmus. Binasal hemianopsia and slight pallor of the right optic nerve was stable. Repeat MRI showed evolution of abnormalities without new lesions. He experienced significant difficulty tracking and was recently determined unsafe to drive.