A 60-year-old male judge with hypertension and diabetes describes a single episode of monocular transient visual loss OD. A dark curtain descended from superior to inferior OD, lasted 10 minutes and then spontaneously resolved. The patient was seen by an outside ophthalmologist and a diagnosis of “retinal migraine” was made. The next day the patient suffers two more episodes of similar visual loss and calls the ophthalmologist back and is given a referral for a two week appointment to neurology for “migraine”. That night the patient suffers a hemispheric stroke with hemiparesis and aphasia. A carotid study shows near occlusion of the right internal carotid artery.\nThe diagnosis of “retinal migraine” is one of exclusion. In elderly patients the diagnosis should be made with caution in the absence of headache history, prior attacks without residual or sequelae, and in patients who have vasculopathic risk factors. The International Headache Society (IHS) criteria for the diagnosis include: “≥ 2 attacks of “fully reversible” monocular visual loss with migraine headache”. The presumptive vasospasm mechanism for migraine headache and visual aura has fallen out of favor and spreading depression of cortical neuronal activity is a more plausible explanation for the visual symptoms in migraine. Unfortunately there is no diagnostic test for the migraine aura and a convincing history is required for the diagnosis. The role of the ophthalmologist is to exclude permanent visual loss after the event by performing formal visual field, documenting that there is no permanent residual loss, and ruling out alternative etiologies or eye markers for ischemic disease (e.g., central or branch retinal artery occlusion, Hollenhorst plaque). The most characteristic historical features for migraine are positive (e.g., scintillation or fortification scotoma) rather than negative (e.g., a black curtain) visual phenomenon; march and build up across the visual field in a nonvascular distribution, stereotyped quality with repeated events without permanent sequelae; and preferably the headache follows the aura after a few minutes duration. The most worrisome historical features for ischemia as the cause for amaurosis fugax include older aged, vasculopathic patient with first onset of an altitudinal (e.g., curtain over my vision) onset and disappearance, duration of minutes (1-10”), and seconds in onset. I would consider ordering a work up in transient monocular visual loss for patients with altitudinal history (e.g., curtain of visual loss), visible embolus, residual visual field defect, vasculopathic risk factors, and an ischemic time course. I would expedite the evaluation if the course was one of an increasing crescendo of events. On the other hand, I often defer work up for visual loss symptoms which have been present for years (e.g., hundreds of events with no residual loss) or have longer duration (e.g., 1-2 hours to days); are non-altitudinal events, the patient has no vasculopathic risk factors, or gives only a vague history of blurring. The clinician should also try to elicit a history suggestive of fluctuating rather than transient visual loss that might suggest the more common benign etiologies like ocular surface disorders or dry eyes. I will give these latter patients a pinhole to try during the events and if it alleviates the problem then it can be safely assumed to be ocular surface or refractive in nature. I also ask the patients to keep a diary of their events to establish some of the historical details above and I often give an empiric artificial tear trial.
A 14-year-old girl presents with headache, visual loss to counting fingers vision, and severe bilateral disc edema with macular star figure OU. The outside ophthalmologist makes the diagnosis of “bilateral neuroretinitis” due to cat scratch disease and sends the Bartonella titer. There is an incomitant esotropia and the secondary diagnosis of “sensory esotropia” is made. The cat scratch titer returns 2 weeks later but is completely negative. The patient is referred to the neuro-ophthalmology service where an MRI reveals a large intracranial tumor producing papilledema OU with significant leakage of exudate into the macula bilaterally.\nBilateral optic disc edema with a macular star figure (ODEMS) is NOT by itself diagnostic for infectious neuroretinitis. The ophthalmoscopic findings are not pathognemonic and papilledema can mimic the fundus appearance of ODEMS. It is my recommendation to consider neuroimaging for all bilateral optic disc edema cases.