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Video Reconstruction of Vasospastic Transient Monocular Blindness

N Engl J Med 2003; 348:1609-1610April 17, 2003

Article

To the Editor:

Video

Sequential Funduscopy of the Left Eye.

Sequential Funduscopy of the Left Eye.

The differential diagnosis of transient monocular blindness includes embolism, hypercoagulability, hemodynamic changes, papilledema, vasculitis, and vasospasm. Although it has been recognized for 150 years, only four clinicians have photographically documented retinal vasospasm.1-4 We describe a patient in whom sequential photographs were taken in order to show the dynamics of the retinal vasculature during transient monocular blindness due to vasospasm.

A 25-year-old man presented with a three-day history of recurrent transient monocular blindness in the left eye, with the daily frequency increasing from 3 to 20 attacks. Each episode started with graying in the nasal quadrant and progressed to complete blindness within 1 minute; the blindness lasted 1 to 3 minutes, and complete recovery occurred within 30 seconds. These attacks could not be triggered by orthostatic maneuvers, the patient's bending over, or pressing of the eyeball. The patients smoked 20 cigarettes per day, drank alcohol regularly, and had taken cocaine three weeks before the onset of transient monocular blindness. General and neuro-ophthalmologic examinations showed no abnormalities.

The patient had an attack of transient monocular blindness in our clinic. Over the course of one minute, the graying of vision spread by about 5 to 10 degrees per second, radiating from the lower nasal quadrant (in a “curtain pattern”), and the blindness lasted for three minutes. There was no direct pupil response, despite a preserved consensual pupil response. Funduscopy during the attack showed a narrowing of the central retinal artery and its branches (Figure 1Figure 1Sequential Funduscopy of the Left Eye at the Beginning (Panel A), Middle (Panel B), and End (Panel C) of an Attack of Transient Monocular Blindness Due to Vasospasm.), followed by slow venular flow with characteristic “cattle trucking” (rouleaux formation). The superior temporal venule had collapsed within the optic disk (Figure 1A). Three minutes later, venous flow recovered and vision returned (Figure 1B and Figure 1C). Four more attacks occurred after treatment with nifedipine (40 mg per day) began. The dose was doubled (to 80 mg per day), and the patient remained free of attacks during one year of follow-up.

Normal filling of cilioretinal arterioles, normal coloring of the capillaries on the disk margin, and a red macula localize the site of spasm to the central retinal artery and its branches in all1,2,4 except one3 of the previous cases. The pattern of visual-field loss is important. The inner retina receives a sectorial blood supply from the central retinal artery and its branches, which corresponds to altitudinal, lateralized (curtain-pattern), or diffuse visual-field loss. In contrast, the posterior ciliary arteries segmentally supply the choroid and outer retina, which, when affected, results in a lacunar (“blob-pattern”) visual-field loss.5 When the visual symptoms (gray or white), funduscopy, and the pattern of transient monocular blindness suggest vasospasm and the frequency of episodes warrants treatment, we recommend a trial with nifedipine before the patient is subjected to more invasive procedures.

Axel Petzold, M.D., Ph.D.
Institute of Neurology, London WC1N 3BG, United Kingdom

Niaz Islam, M.R.C.Ophth.
Gordon T. Plant, M.D.
Moorfields Eye Hospital, London EC1 2PD, United Kingdom

5 References
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    Burger SK, Saul RF, Selhorst JB, Thurston SE. Transient monocular blindness caused by vasospasm. N Engl J Med 1991;325:870-873
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    Winterkorn JMS, Kupersmith MJ, Wirtschafter JD, Forman S. Treatment of vasospastic amaurosis fugax with calcium-channel blockers. N Engl J Med 1993;329:396-398
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    Humphrey WT. Central retinal artery spasm. Ann Ophthalmol 1979;11:877-881
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    Kline LB, Kelly CL. Ocular migraine in a patient with cluster headaches. Headache 1980;20:253-257
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    Bruno A, Corbett J, Biller J, Adams HPJ, Qualls C. Transient monocular visual loss patterns and associated vascular abnormalities. Stroke 1990;21:34-39
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Citing Articles (2)

Citing Articles

  1. 1

    Donna L Hill, Robert B Daroff, Anne Ducros, Nancy J Newman, Val??rie Biousse. (2007) Most Cases Labeled as ???Retinal Migraine??? Are Not Migraine. Journal of Neuro-Ophthalmology 27:1, 3-8
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  2. 2

    Jacqueline M. S Winterkorn. (2007) ???Retinal Migraine??? is an Oxymoron. Journal of Neuro-Ophthalmology 27:1, 1-2
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