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Neuro-Ophthalmology and Stroke

Biousse, Valérie MD

doi: 10.1097/WNO.0000000000000531

Departments of Ophthalmology and Neurology, Emory University School of Medicine, Atlanta, Georgia.

Address correspondence to Valérie Biousse, MD, Neuro-ophthalmology Unit, Emory Eye Center, The Emory Clinic, 1365-B Clifton Road NE, Atlanta, GA 30322; E-mail:

Supported in part by an unrestricted departmental grant (Department of Ophthalmology) from Research to Prevent Blindness, Inc, New York, and by NIH/NEI core grant P30-EY06360 (Department of Ophthalmology).

The author reports no conflict of interest.

Neuro-ophthalmologic presentations of acute ischemic stroke are common. Ocular symptoms and signs as well as visual disturbances from intracranial visual pathway dysfunction often precede devastating neurologic events. Two articles in this issue of the Journal of Neuro-Ophthalmology highlight the need for eye care providers to be aware of ocular symptoms preceding cerebral ischemia (1,2). A third report (3) raises the question of when is an eye examination necessary in stroke patients, and a review by Chancellor and Ishida (4) is a welcome update on the management of acute ischemic stroke.

Lenassi et al (1) report a unique patient with persistent placoid maculopathy (PPM) who developed focal neurologic signs 2 months after visual loss. The patient was eventually diagnosed with cerebral vasculopathy, presumably related to cerebral vasculitis. Cases of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and PPM complicated by cerebral infarctions rarely have been reported (1,5). Although optimal management of PPM and APMPPE remain debated, it has been suggested that high-dose corticosteroids may be beneficial in some patients, especially those with concomitant cerebral ischemia. Similar to the patient reported by Lenassi et al (1), a few other cases developed neurologic symptoms and signs during steroid taper or shortly after steroids were discontinued, emphasizing the need for careful long-term follow-up of these patients (5). As often seen with cerebral vasculitis, a majority of placoid maculopathy patients with cerebral ischemia complained of headaches before the onset of focal neurologic deficits. This is an important symptom to recognize because isolated PPM or APMPPE is not typically associated with eye pain or headaches. Given how rarely cerebral ischemia complicates placoid maculopathies, it is not necessary to systematically obtain a brain MRI in all patients seen for isolated visual loss from these entities. However, new onset headaches in this group of patients should prompt further evaluation with a brain MRI and MRA and treatment with high-dose corticosteroids.

More disturbing is another report of acute cerebral pontine infarction preceded by eye pain for 24 hours. Described as “salt and pepper” eye pain because it is often compared with eye pain similar to what would be triggered by rubbing pepper into the eye, this usually isolated pain syndrome classically precedes acute brainstem ischemia involving the paramedian area of the pons. In the patient described by Lyons et al (2), the pain occurred less than 24 hours before the development of hemiparesis. In other reports summarized in the same article, eye pain typically preceded stroke symptoms by several hours for 2–3 days and up to 15 days. In the patient of Lyons et al (2), the eye pain was contralateral to the pontine infarction, but other reports have documented the pain as being ipsilateral or even bilateral. Although the pathophysiology of the pain is unclear, it likely involves the trigeminovascular system. However, the pain characteristics are not typical enough to be easily recognized as a warning sign of impending stroke. This rare pain syndrome must be differentiated from the eye pain that often occurs simultaneously with a contralateral homonymous hemianopia from acute occipital infarction. Described by Knox and Cogan in 1962 (6), this pain is classically a dull and deep eye or brow pain on the side of the occipital infarction, present at the time of the infarction and often lasting a few hours. It is attributed to the sensory innervation of the dura by recurrent branches of the ophthalmic division of the trigeminal nerve that spread over the ipsilateral tentorium cerebelli and posterior falx.

The article by Kuonen and Borruat (3) emphasizes the opposite situation. They report a patient with acute neurologic signs revealing multiple cerebral ischemic lesions suggesting cerebral vasculitis. Although the patient did not have any visual complaints, careful examination disclosed retinal abnormalities suggesting vasculopathy likely related to retinal emboli. The patient was diagnosed with cardiac myxoma responsible for numerous cerebral and ocular emboli. This observation reminds neurologists that the eye is indeed a “window to the brain” and that careful systematic ocular examination may help understand the mechanism of acute neurologic deficits and cerebral ischemia.

The past 20 years have revolutionized the diagnosis and acute management of stroke making it challenging for neuro-ophthalmologists to stay up to date in this ever-changing field. The review by Chancellor and Ishida (4) provides much needed new information about recent stroke studies. Paroxysmal atrial fibrillation is an increasingly recognized cause of stroke and its detection is made easier by the use of prolonged 30-day cardiac monitoring in patients with unexplained transient ischemic attack and stroke. The ability to diagnose paroxysmal atrial fibrillation also is increased by longer monitoring of up to 6 months to 1 year with external and implantable loop recorders. This is important because newly developed anticoagulants have become available, many with superior efficacy and safety compared with standard warfarin in stroke patients with atrial fibrillation. Recent randomized controlled trials provide Level 1 evidence for the use of endovascular treatments and thrombolytics as the standard of care in eligible patients. These recent developments reinforce the need for neuro-ophthalmologists to appropriately refer patients with suspected ischemic stroke to certified stroke centers.

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1. Lenassi E, Kojovic M, Mekjavic PJ, Sega S, Valentincic NV. Persistent placoid maculopathy complicated by cerebral vasculitis. J Neuroophthalmol. 2017;273–275.
2. Lyons LJ, Law SW, Kubie JL. “Salt and pepper” pontine infarct. J Neuroophthalmol. 2017;276–280.
3. Kuonen A, Borruat FX. Ocular and cerebral emboli from an atrial myxoma. J Neuroophthalmol. 2017;309–310.
4. Chancellor BK, Ishida K. New standards of care in ischemic stroke. J Neuroophthalmol. 2017;320–331.
5. Luneau K, Newman NJ, Srivastava S, Biousse V. A case of acute posterior multifocal placoid pigment epitheliopathy with recurrent stroke. J Neuroophthalmol. 2009;29:111–118.
6. Knox DL, Cogan DG. Eye pain and homonymous hemianopia. Am J Ophthalmol. 1962;54:1091–1093.
© 2017 by North American Neuro-Ophthalmology Society