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A 37-year-old man with a black spot in his vision
Digital Journal of Ophthalmology 2010
Volume 16, Number 1
February 9, 2010
DOI: 10.5693/djo.03.2009.12.001
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Ann-Marie Lobo, MD | Massachusetts Eye & Ear Infirmary and Harvard Medical School
George N. Papaliodis, MD | Massachusetts Eye and Ear Infirmary, Massachusetts General Hospital, and Harvard Medical School
Differential Diagnosis
• Bilateral branch retinal artery occlusions: Although rare, bilateral retinal artery occlusions have been reported in patients with hypercoagulable states such as antiphospholipid antibody syndrome. Often in these cases, there is both a retinal vein and retinal artery occlusion. Hypercoagulable work up in young patients and carotid doppler ultrasounds and echocardiograms in older patients are recommended to determine underlying etiology for bilateral retinal vascular occlusions.
• CNS vasculitis with retinal vasculitis: CNS vasculitis may present with headache and encephalopathy and may have associated retinal vasculitis. Protein levels in the CSF are elevated. Ischemic changes may be evident on neuroimaging and alterations in vessel caliber may be seen on cerebral angiography.
• Susac syndrome: This arteriolar microangiopathy affects pre-capillary arterioles in the brain, retina, and cochlea leading to encephalopathy, vision loss, and hearing loss.
• Multiple sclerosis: Focal neurologic signs and symptoms combined with white matter lesions on neuroimaging may be indicative of a demyelinating disease such as multiple sclerosis. Multiple sclerosis can also present with inflammation of the blood vessels in the retina with a perivasculitis.
• Systemic lupus erythematosus: SLE can involve multiple organ systems and cause vasculitis in the brain and retina. Inflammatory markers such as ANA are usually positive in this disease process; however, there are cases of SLE with negative antinuclear antibody tests. Other clinical signs of lupus, including skin rash, arthritis, and renal dysfunction are supporting signs for diagnosis of SLE.
• Behçet’s disease: Behçet’s disease can be associated with an occlusive vasculitis in the brain and retina. In addition to oral and genital ulcerations, ocular inflammation, and skin lesions, these patients may also have high frequency sensorineural hearing loss. The central nervous system may be affected in Behçet’s syndrome with development of confusional syndrome, meningoencephalitis, or brainstem involvement.
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