Vision loss without a relative afferent pupillary defect can localize to the macula.
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A 70-year-old female with bilateral posterior cerebral artery (PCA) infarcts (right greater than left), previous posterior reversible encephalopathy syndrome (PRES), severe intracranial atherosclerotic disease, hypertension, hyperlipidemia, and peripheral vascular disease, presented with 3 months of slowly progressive central vision loss. Initial concern by neurology was for worsening left PCA stroke leading to cortical blindness. In the emergency department, inflammatory markers were negative and imaging showed stable prior infarcts. Specifically, there was no new left PCA infarct.
She was referred to neuro-ophthalmology 3.5 months after symptom onset. Examination revealed visual acuity of 20/150 OD and 20/100 OS. Color plates were 10/15 OD and 13/15 OS. Both eyes had abnormal Amsler grid testing. Her left inferior homonymous quadrantanopia was stable from prior on visual field testing. There was no rAPD.
Optical coherence tomography (OCT) of the maculae revealed new bilateral full-thickness macular holes involving the fovea. Bilateral stage 4 macular holes provided a more consistent explanation for her central visual symptoms than a cortical or optic nerve pathology. She was referred to retina service and underwent pars plana vitrectomy, internal limiting membrane peeling, and SF 6 gas injection. Her vision remained stable at one-month post-operative follow-up.Neurologists are often consulted for patients with vision loss. Central vision loss without a rAPD can localize to the macula. When neurological evaluation and neuroimaging are both unrevealing in a patient with vision loss, it is important for the patient to be promptly referred for a dedicated eye exam. Macular Optical Coherence Tomography is often obtained in many ophthalmological practices and can be exceedingly helpful in patients with macular disease. Localization is critical for the neurologist, as patients with vision loss due to macular disease may be candidates for surgical intervention.