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Acute retinal necrosis (ARN) occurs more often in immunocompetent individualsand is defined clinically by discrete areas of peripheral retinal necrosiswith rapid confluence, vascular sheathing, and prominent inflammation in thevitreous and anterior chamber.1 Pathologically,full-thickness necrosis of the retina is seen in the setting of an obliterativearteritis.2 Fluorescein angiography and histopathologicstudies have shown that the arteritis is not confined to the retinal vesselsbut is seen in virtually all tissues of the eye including the iris, ciliarybody, choroid, and optic nerve. This vasculitic process extending beyond theocular vessels has not been well documented. We report a case of ARN and subclinical,diffuse cerebral vasculitis that was discovered after 3.0-T magnetic resonanceimaging and magnetic resonance angiography were performed.
A 46-year-old man had a 2-week history of decreasing vision in the righteye. He was initially diagnosed with anterior uveitis and treated with topicalprednisolone and cycloplegic drops. His vision worsened to hand motions. Fundusexamination at that time showed vitritis, vascular sheathing with hemorrhages,and patchy retinal opacities that at times coalesced. The diagnosis of ARNwas made. The patient was generally healthy but had suffered recently fromsome nonspecific, diffuse headaches and problems with forgetfulness. However,he reported no focal neurologic symptoms or signs. He had no recent skin painor lesions.
Intravenous acyclovir was given for 10 days. Optic nerve involvementwas suspected, and the patient was transferred to our hospital where high-resolutionmagnetic resonance imaging and magnetic resonance angiography of the orbitand brain were performed using a 3.0-T scanner. There was no optic nerve sheathdistension. No abnormalities of the visual nuclei or radiations were seen.However, signal abnormalities in the right basal ganglia and right thalamuswere consistent with subacute strokes of different ages (Figure 1). The angiography revealed multiple focal abnormalitiesin flow enhancement of the vessels throughout the brain including those supplyingthe areas of the stroke (Figure 2).These observations were consistent with a diffuse vasculitis. Finally, theophthalmic arteries demonstrated irregular flow enhancement that was moresevere on the right side consistent with a vasculitic process.
Axial fluid-attenuated inversionrecovery images (repetition time = 10 000 milliseconds, echotime = 160 milliseconds, T1 = 2250 milliseconds, echotrain length = 30, field of view = 22 × 22cm2, matrix size = 320 × 192, 2 average,3 T) through the basal ganglia and thalami showing T2-hyperintense signalabnormalities in the right basal ganglia (A) and thalamus (B).
Maximum intensity projection reconstructedmaps of the 3-dimensional, time of flight magnetic resonance angiography (repetitiontime = 30 milliseconds, echo time = 4.4 milliseconds,flip angle = 25°, field of view = 22 × 16cm2, matrix size = 512 × 256, slice thickness = 1mm, 1 average, 3 T) through the circle of Willis showing evidence of vasculitisby the narrowing in vessels of (A) the posterior circulation including theright intracranial vertebral artery (horizontal arrow) and the left posteriorcerebral artery (vertical arrow) and (B) the anterior circulation includingthe anterior cerebral arteries (upward arrow) and branches of the left middlecerebral artery (downward arrow).
Cerebrospinal fluid studies revealed a pleocytosis of white cells anda positive IgG antibody titer to varicella-zoster virus. Serologic study resultswere unremarkable. The patient did not develop any focal neurologic signsor symptoms during the course of his follow-up.
On review of the literature, we found only 1 report of “necrotizingherpetic retinitis” associated with cerebral vasculitis. Rousseau etal3 reported 3 patients with human immunodeficiencyvirus who developed bilateral retinal necrosis despite intravenous antiviraltherapy. Given the highly immunocompromised state of these patients and thefulminant course despite therapy, it is more likely that the retinal necrosisin these patients represented progressive outer retinal necrosis rather thanARN. Additionally, each of these patients had a herpes zoster rash, whichis important because herpes zoster ophthalmicus has often been associatedwith ipsilateral cerebral angiitis.4 The patientsin the report by Rousseau et al did develop clinical manifestations and radiologicevidence of central nervous system strokes. Cerebral arteriography was performedin 1 of these patients with evidence of a focal vasculitis.
In contrast, our patient was healthy and had no prior zoster rash. Further,he had no clinical signs of a central nervous system stroke. Since this diffusecerebral vasculitis was subclinical, it is plausible that other patients withARN may have had similar central nervous system processes that were not discovered.This has clinical implications since parenteral corticosteroids have beenused in addition to antivirals in treating central nervous system vasculitides,notably those caused by varicella-zoster virus.4
Although this may be the first report of cerebral vasculitis and ARN,the association of these entities might be expected given that ARN is, inpart, a vasculitic process. Further, varicella-zoster virus has been linkedto each separately. It is a known cause of ARN and as mentioned previously,herpes zoster ophthalmicus–related cerebral angiitis is well documented.Finally, animal models demonstrate that anterior chamber inoculation of herpesvirusesresults in spread to the brain.5
This case is unique in that clinical symptoms or an antecedent zosterrash did not herald the cerebral vasculitis. It demonstrates that cerebralvasculitis may occur when ARN is the only manifestation of a presumed herpesvirusinfection, and screening may be warranted using high-resolution magnetic resonanceimaging and magnetic resonance angiography, especially in cases where varicella-zostervirus is the implicated cause.
Correspondence: Dr Pulido, Department ofOphthalmology, University of Illinois at Chicago, 1855 W Taylor St, Chicago,IL 60612 (firstname.lastname@example.org).
Financial Disclosure: None.
Funding/Support: This study was supported inpart by an unrestricted grant from Research to Prevent Blindness, Inc, NewYork, NY, to the University of Illinois at Chicago and the Mayo Clinic, Rochester,Minn, and by core grant EY01792 from the National Eye Institute, Bethesda,Md.
Chung GW, Thulborn KR, Pulido JS, Merrill PT, Tessler HH. Magnetic Resonance Imaging of Diffuse Cerebral Vasculitis AssociatedWith Acute Retinal Necrosis. Arch Ophthalmol. 2004;122(11):1719-1720. doi:10.1001/archopht.122.11.1719