Detection of Atrial Fibrillation After Central Retinal Artery Occlusion.

Background: Central retinal artery occlusion (CRAO) causes sudden, irreversible blindness and is a form of acute ischemic stroke. In this study, we sought to determine the proportion of patients in whom atrial fibrillation (AF) is detected by extended cardiac monitoring after CRAO. Methods: We performed a retrospective, observational cohort study using data from the Optum deidentified electronic health record of 30.8 million people cross-referenced with the Medtronic CareLink database of 2.7 million people with cardiac monitoring devices in situ. We enrolled patients in 3 groups: (1) CRAO, (2) cerebral ischemic stroke, and (3) age-, sex-, and comorbidity-matched controls. The primary end point was the detection of new AF (defined as ≥2 minutes of AF detected on a cardiac monitoring device). Results: We reviewed 884 431 patient records in common between the two databases to identify 100 patients with CRAO, 6559 with ischemic stroke, and 1000 matched controls. After CRAO, the cumulative incidence of new AF at 2 years was 49.6% (95% CI, 37.4%­61.7%). Patients with CRAO had a higher rate of AF than controls (hazard ratio, 1.64 [95% CI, 1.17­2.31]) and a comparable rate to patients with stroke (hazard ratio, 1.01 [95% CI, 0.75­1.36]). CRAO was associated with a higher incidence of new stroke compared with matched controls (hazard ratio, 2.85 [95% CI, 1.29­6.29]). Conclusions: The rate of AF detection after CRAO is higher than that seen in age-, sex-, and comorbidity-matched controls and comparable to that seen after ischemic cerebral stroke. Paroxysmal AF should be considered as part of the differential etiology of CRAO, and those patients may benefit from long-term cardiac monitoring.

Management of Central Retinal Artery Occlusion: A Scientific Statement From the American Heart Association.

PURPOSE: Central retinal artery occlusion (CRAO) is a form of acute ischemic stroke that causes severe visual loss and is a harbinger of further cerebrovascular and cardiovascular events. There is a paucity of scientific information on the appropriate management of CRAO, with most strategies based on observational literature and expert opinion. In this scientific statement, we critically appraise the literature on CRAO and provide a framework within which to consider acute treatment and secondary prevention. METHODS: We performed a literature review of randomized controlled clinical trials, prospective and retrospective cohort studies, case-control studies, case reports, clinical guidelines, review articles, basic science articles, and editorials concerning the management of CRAO. We assembled a panel comprising experts in the fields of vascular neurology, neuro-ophthalmology, vitreo-retinal surgery, immunology, endovascular neurosurgery, and cardiology, and document sections were divided among the writing group members. Each member received an assignment to perform a literature review, synthesize the data, and offer considerations for practice. Multiple drafts were circulated among the group until consensus was achieved. RESULTS: Acute CRAO is a medical emergency. Systems of care should evolve to prioritize early recognition and triage of CRAO to emergency medical attention. There is considerable variability in management patterns among practitioners, institutions, and subspecialty groups. The current literature suggests that treatment with intravenous tissue plasminogen activator may be effective. Patients should undergo urgent screening and treatment of vascular risk factors. There is a need for high-quality, randomized clinical trials in this field.

Myasthenia Gravis Presenting as Lutz Posterior Internuclear Ophthalmoplegia.

Myasthenia gravis can mimic central neurological disorders and should be considered in the differential diagnosis of any form of pupil-sparing ophthalmoplegia. We report an unusual manifestation of myasthenia gravis presenting as bilateral internuclear ophthalmoplegia (INO) of abduction, sometimes referred to as Lutz posterior INO (or reverse INO).

Oculomotor Synkinesis (Aberrant Reinnervation of the Third Cranial Nerve) Associated with Atypical Tolosa-Hunt Syndrome.

A 70-year-old woman with systemic lupus erythematosus developed a painful pupil-involving right third nerve palsy, ipsilateral fourth nerve palsy, and periorbital paraesthesia. Magnetic resonance imaging demonstrated enhancement and thickening of the right third nerve, and she was diagnosed with presumed Tolosa-Hunt syndrome. Repeated imaging seven months later showed resolution of the enhancing thickened oculomotor nerve, but the patient developed signs of oculomotor synkinesis. This presentation demonstrates a rare case of oculomotor synkinesis secondary to inflammation.

Nutritional Optic Neuropathy Caused by Copper Deficiency After Bariatric Surgery.

A 47-year-old woman developed severe bilateral visual loss 4 years after a Roux-en-Y gastric bypass and 24 years after vertical banded gastroplasty. Her serum copper level was 35 µg/dL (normal, 80-155 µg/dL). She was prescribed elemental copper tablets. Because her methylmalonic acid was slightly elevated, she received vitamin B12 injections as well. Five weeks later, she reported that her vision had improved and, at 10 months, her vision had recovered from 20/400 bilaterally to 20/25 in each eye. This case highlights the importance of checking copper levels in addition to the "more routine" vitamin levels, such as B1, B6, B12, E, and serum folate in patients with suspected nutritional optic neuropathy after bariatric surgery, particularly if it involved a bypass procedure.

Topiramate-induced palinopsia: a case series and review of the literature.

BACKGROUND: To report palinopsia as a possible side effect of topiramate. METHODS: Case series and review of the literature. RESULTS: Nine patients in our series, and 4 previously reported patients, who developed palinopsia while on topiramate, are reviewed. All patients were women, and comorbidities included migraine, idiopathic intracranial hypertension, and bulimia nervosa. Palinopsia resolved in 8 patients after stopping or decreasing the dose of topiramate. The lowest dose of topiramate causing palinopsia was 25 mg twice a day. More than half of our patients reported exacerbation of visual disturbance in early morning or late evening. CONCLUSIONS: Topiramate-induced palinopsia may be underdiagnosed because physicians do not inquire about such visual symptoms.

"Ophthalmoplegic migraine" with reversible MRI enhancement of the cisternal sixth cranial nerve.

A 45-year-old woman reported multiple episodes of reversible left eye pain and diplopia stretching over 12 years. Ophthalmic examinations had repeatedly disclosed a left sixth cranial nerve palsy. Postcontrast brain MRI performed 3 weeks after clinical onset of the most recent episode demonstrated enhancement of the cisternal segment of the left sixth cranial nerve. Five months later, when symptoms and signs had largely abated, postcontrast brain MRI was normal. The clinical diagnosis satisfies the criteria for "ophthalmoplegic migraine." Although reversible cisternal enhancement of the third cranial nerve has been often described in this condition, this is the first report of cisternal enhancement of the sixth cranial nerve.

Fulminant idiopathic intracranial hypertension.

OBJECTIVE: To describe the incidence and characteristics of acute and rapidly progressive visual loss in idiopathic intracranial hypertension (IIH). METHODS: We reviewed the medical records of all patients with IIH seen at two institutions. "Fulminant IIH" was defined as the acute onset of symptoms and signs of intracranial hypertension (less than 4 weeks between onset of initial symptoms and severe visual loss), rapid worsening of visual loss over a few days, and normal brain MRI and MR venography (or CT venogram). RESULTS: Sixteen cases with "fulminant IIH" were included (16 women, mean age 23.8 years [range 14 to 39 years]). All were obese. One patient had iron-deficiency anemia, four had systemic hypertension, and none had known sleep apnea syndrome. Acute or subacute headache, nausea and vomiting, and visual loss were present in all patients. The first lumbar puncture performed for the diagnosis showed a mean CSF opening pressure of 54.1 cm H(2)O (range 29 to 60 cm H(2)O). In addition to the initial lumbar puncture, medical treatment included acetazolamide (1 to 2 g/day) in all patients, and IV methylprednisolone in four patients. Repeat lumbar punctures were performed in 11 of the 16 patients. Surgical treatment (optic nerve sheath fenestration in five cases, lumboperitoneal CSF shunting procedure in nine cases, and ventriculoperitoneal shunting procedure in two cases) was performed because of ongoing visual loss in all cases. The median delay between evaluation in neuro-ophthalmology and surgery was 3 days (range a few hours to 37 days). All patients reported dramatic improvement of headaches and vomiting following surgery. Visual function improved in 14 cases, although 8 patients (50%) remained legally blind. Visual fields remained severely altered in all cases. CONCLUSION: Severe and rapidly progressive visual loss suggests "fulminant idiopathic intracranial hypertension" and should prompt aggressive management. Urgent surgery may be required in these patients, and temporizing measures such as repeat lumbar punctures, lumbar drainage, and IV steroids considered.

Posterior cortical atrophy: a brief review.

Posterior cortical atrophy is a striking clinical syndrome in which a dementing illness begins with visual symptoms. Initially, the problem may seem to be loss of elementary vision, but over time the patient develops features of visual agnosia, topographical difficulty, optic ataxia, simultanagnosia, ocular apraxia (Balint's syndrome), alexia, acalculia, right-left confusion, and agraphia (Gerstmann's syndrome), and later a more generalized dementia. Occasional patients have visual hallucinations and signs of Parkinson's disease or Lewy body dementia. A number of different neuropathologic disorders are associated with posterior cortical atrophy.

Hyperglycemic hemianopia: a reversible complication of non-ketotic hyperglycemia.

Four patients each developed a reversible homonymous hemianopia caused by non-ketotic hyperglycemia. In two patients the homonymous hemianopia was the first manifestation of diabetes mellitus type 2. All four patients had abnormal MRI scans; in the three patients who had late follow-up scans the abnormalities resolved completely. In one patient the progressive visual field defect and unusual MRI findings initially caused concern for a tumor.