1. A 21-year-old male with rapidly- progressive cortical visual loss Teaching NeuroImages Neurology Resident and Fellow Section © 2015 American Academy of Neurology

2. Vignette © 2015 American Academy of Neurology Day GS, et al. A 21-year-old male immigrant from India who had not been vaccinated against measles in childhood presented with visual loss noticed by his parents. Past medical history was notable for a 4-year history of cognitive impairment due to subacute sclerosing panencephalitis (SSPE). SSPE was previously managed with intraventricular α-interferon, delivered three-times weekly via an Ommaya reservoir. Treatments were stopped 18-months earlier given stable disease. Examination confirmed baseline cognitive impairment. Vision was reduced to light perception. The patient denied visual loss. Magnetic resonance imaging of brain was obtained (Figure). CSF measles titers were elevated.

3. Imaging © 2015 American Academy of Neurology Day GS, et al.

4. Recurrent SSPE presenting as Anton Syndrome with “cortical ribboning” SSPE may cause cortical visual loss, presenting as Anton Syndrome. Restricted diffusion of the cortical ribbon may be seen in SSPE in addition to Creutzfeldt Jakob disease, 1 reflecting cortical dysfunction and neuronal loss. 2 SSPE should be included in the differential diagnosis of patients presenting with cortical dysfunction and corresponding focal areas of diffusion restriction on magnetic resonance neuroimaging. Intrathecal α-interferon may stabilize disease, 3 although the use of this medication for the treatment of SSPE remains “off-label”. © 2015 American Academy of Neurology Day GS, et al.