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Show Recurrent Neuroretinitis in an Adolescent with Ulcerative Colitis Mohammad Shoari, MD, and Bradley J. Katz, MD, PhD Abstract: Neuroretinitis refers to an optic neuropathy in He had a small afferent pupillary defect and decreased color which optic disc edema is accompanied by peripapillary or vision OS. Ophthalmoscopy revealed 360° of optic nerve macular hard exudates. Most cases involve a single episode swelling and mild diffuse pallor OD. The vitreous was and have no associated systemic abnormalities. In rare quiet. In the OS, the optic nerve was swollen for 360° with instances, neuroretinitis may be recurrent and lead to a stellate pattern of lipid exudates surrounding the macula. progressive visual loss. We describe a patient with recurrent Serologic testing, including complete blood count, angio-neuroretinitis who concurrently developed ulcerative co- tensin converting enzyme, B. henselae titer, and litis, the first report of this association. B. burgdorferi titer, was within normal limits. Brain computed . , » r _ , , , , „„„^ „ „„, „ OON tomography ( CT) scan was normal except for fluid distention { JNeuro- Ophthalmol 2005; 25: 286- 288) r. i 1 1 \ 1 « r1 • 1 ot the lett optic nerve sheath. Fluorescein angiography was normal OD; angiography OS revealed diffuse optic nerve leakage. Late in the angiogram, leakage was noted from the Neuroretinitis refers to an optic neuropathy associated optic disc toward the fovea. The diagnosis was neuroretinitis. with the unique combination of optic disc edema and Without treatment, he experienced marked visual improve-peripapillary or macular hard exudates ( 1). Some cases of ment OS within several weeks. neuroretinitis are associated with a systemic disease. Most One year later, he experienced blurred vision OD. patients experience spontaneous remission and restoration Visual acuity was 20/ 400 OD and 20/ 20 OS. Ophthalmos-of normal visual function. In rare instances, patients may copy now revealed 360° of optic nerve swelling with suffer recurrent neuroretinitis that results in a progressive loss obscuration of retinal vessels at the periphery of the disc of visual function. In contrast to single- episode neuroretinitis, and at the center of the disc. Exudates were observed track-recurrent neuroretinitis is thought to be an autoimmune ing from the nerve toward the fovea ( Fig. 1). Brain MRI and disease. We report a patient with recurrent neuroretinitis who lumbar puncture were normal. Serologies for cytomegalo-concurrently developed ulcerative colitis. To the best of our virus, coccidioides, cysticercosis, Bartonella, Aurelia, toxo-knowledge, this is the first report of recurrent neuroretinitis plasma, and toxocara were all negative. He was diagnosed associated with inflammatory bowel disease. with a second episode of neuroretinitis, hospitalized and treated with intravenous corticosteroids ( 250 mg methyl- PASP nppnuT prednisolone IV every 6 hours for three days) and an oral . . . ... , . taper ( 60 mg prednisone orally tapered over 11 days). Sev- A 14- year- old boy presented to an optometrist with i i i * 1 /~> T-> 1 J J * 1 . - , . ,, " L„ , r . , . ™ , ™ ^ T^ . eral weeks later, visual acuity OD had returned to normal acute, parntul visual loss OS. Visual acuity was 20/ 20 OD and , ,. , , , . , ™ , ™ ,;^ o ^ • r . . . . . and optic disc edema had improved. 20/ 200 OS. The patient was referred to our institution four _, * i i * i . J A J 1 • r . . „ ,, , • Eleven months later, he reported a 4- day history of to six weeks after visual loss was first noted by the patient. ,, . A, ,~. o , r i „„,.„ A n , , . , _ , , . ,, ™ „ ^ ^ T ^ 1 ™ /^ ^ o blurred vision in the OS. Visual acuity was 20/ 30 OD and Visual acuity had improved to 20/ 15 OD and 20/ 40 OS. i n n n n r n u i IJ 1 1 20/ 20 OS. Ophthalmoscopy revealed an early macular star and recurrent disc swelling OS. He was treated with 1 g ~ . . 7Z i mc DTI^ j n u i , i i TT7 i methylprednisolone IV daily for three days and prednisone Departments ot Neurology ( MS, BJK) and Ophthalmology and Visual J r J J c Sciences ( BJK), John A. Moran Eye Center ( BJK), University of Utah 60 mg/ d tapered Over 11 days. Five months later, visual Health Sciences Center, Salt Lake city, Utah. acuity and visual fields were stable, and his optic nerve Supported in part by an unrestricted grant to the Department of swelling and exudates had Completely resolved. Ophthalmology and Visual Sciences from Research to Prevent Blindness, A& M& mQ& t Kcent ^ ^ Qf n e u r o r e t i r i i t i s w a s r e . '',,, ' , ', D ,, T „ , , m « „ , , . . , solving, approximately three years after his initial attack of Address correspondence to Bradley J. Katz, MD, PhD, John A. Moran °' r r J J Eye Center, 75 N Medical Drive, Salt Lake city, UT 84132; E- mail: neuroretinitis, he developed bloody diarrhea and abdominal bradley. katz@ hsc. utah. edu cramps. Colonoscopy revealed ascending and transverse colon inflammation. The terminal ileum was normal. Biopsies of the inflamed colon were consistent with ulcerative colitis. The patient was treated with azathioprine and mesal-amine. His abdominal cramps and bloody diarrhea improved and the azathioprine dose was reduced. Within a few months of tapering the azathioprine dose, the patient noted loss of vision OD. Visual acuity was 20/ 200 OD and 20/ 60 OS. Ophthalmoscopic examination OD showed optic disc edema with a macular star. Examination of the OS revealed diffuse pallor of the optic disc. Visual field testing revealed superior and inferior arcuate scotomas OU. The dose of azathioprine was increased with periodic monitoring of blood count and liver enzymes. In addition, he was again treated with a 3- day course of intravenous methylprednisolone 1 gm/ d followed by methyl-prednisolone 24 mg/ d tapered over six days. Two months later, visual acuity had improved to 20/ 70 OD and 20/ 40 OS. He could identify only 1/ 7 Hardy Rand Rittler ( HRR) color plates in OU; automated perimetry showed superior and inferior arcuate defects, enlarged blind spots, and central scotomas in OU. Five weeks later, he reported blurred vision in the OS. Acuity was 20/ 70 OD and 20/ 200 OS. The optic disc OD showed diffuse pallor without edema. The optic disc OS was diffusely pale and swollen, and a new macular star had appeared. A similar course of IV methylprednisolone with an oral methylprednisolone taper was prescribed. The patient is currently serving as a missionary and has moved out of state. By report, his visual acuity improved after his most recent corticosteroid treatment, but he subsequently suffered another attack of neuroretinitis in the OD that was treated with a similar course of IV methylprednisolone followed by oral methylprednisolone. His azathioprine dose has been increased from 200 mg/ d to 250 mg/ d. DISCUSSION Neuroretinitis was first described by Leber in 1916 as a retinopathy associated with unilateral vision loss, optic disc swelling, and an exudative maculopathy in young healthy patients ( 2,3). Patients range in age from 9 to 55 years with no gender preference. Visual acuity can range from 20/ 50 to 20/ 200 ( 4). An afferent pupillary defect and decreased color vision are usually present. The most common visual field defects are cecocentral and central scotomas ( 5). Almost all cases affect only one eye, but bilateral cases have been described. Most patients who develop neuroretinitis do not experience a subsequent attack in the same eye, and only a small percentage of patients who have experienced an attack in one eye subsequently develop a similar attack in the other eye ( 1). Neuroretinitis often resolves spontaneously ( 6), and most patients enjoy a full restoration of visual function. Many inflammatory and infectious conditions have been associated with neuroretinitis, but approximately 50% of cases remain idiopathic ( 7). There is a growing list of reports of associations with infectious, autoimmune, and neoplastic conditions ( 4,8). Many of these conditions are treatable, and accurate diagnosis can result in visual rehabilitation. The infectious conditions associated with neuroretinitis are summarized in Table 1. In contrast to patients with the typical monophasic neuroretinitis, a minority of patients develop a more severe illness with recurrent episodes of neuroretinitis involving the same eye, the fellow eye, or both. This recurrent form of the disease is characterized by a poor visual outcome. The cause of this disorder has not been identified and has simply ileocolitis ( 22). To the best of our knowledge, neuroretinitis been termed recurrent idiopathic neuroretinitis. Although has not been previously reported in inflammatory bowel laboratory testing has not revealed a systemic disease in disease, these patients, some researchers suspect an autoimmune disorder involving the optic disc ( 4,9,10). Unlike the favorable prognosis that applies to the REFERENCES monophasic illness, recurrent neuroretinitis is characterized 1. Beck RW. Optic neuritis. In: Miller NR, Newman NJ, eds. Walsh and by repeated acute episodes that may lead to progressive and H° yfs clinical Neum- Ophthalmology. 5th ed. Baltimore: Williams * • , , ,1 1 S T~ • , \. r and Wilkins; 1998: 634- 9. permanent visual loss ( 11). The incomplete recovery of 2 Casson RJ Q, Day :> Crompton JL. Lebefs idiopathic stellate visual function has been ascribed to permanent damage to neuroretinitis: differential diagnosis and approach to management. the optic nerve, not the macula. In comparison to mono- Amt N ZJ Ophthalmol 1999; 27: 65- 9. . . . . . . . . _ . . . 3. Hamard P, Hamard H, Ngohou S. [ Leber's idiopathic stellate phasic neuroretinitis, the evaluation of patients With recur- neuroretinitis: Aproposof9 cases]. JFrOphtalmol 1994; 17: 116- 23. rent disease is almost always unrevealing. Purvin et al ( 11) 4. Ray S, Gragoudas E. Neuroretinitis. Int Ophthalmol Clin 2001; 41: have suggested that the most likely cause in recurrent cases 83- 102. .. 1 1 1 - 1 , - r- • 1- 5. Gass JD. Leber's idiopathic stellate neuroretinitis and multifocal is a very discrete and localized form of autoimmune dis- retinitis In: Gass JD> ed Stereoscopic Mas of Macular Diseases: ease. However, recurrent cases have also been associated Diagnosis and Treatment. 4th ed. St. Louis, MO: Mosby; 1997: with toxoplasmosis ( 12,13), melanocytoma ( 14,15), and 996- 9. • 1 • / i r 1 - i\ rm r- • • • 1 i 6. King MH, Cartwright MJ, Carney MD. Leber's idiopathic stellate Sarcoidosis ( 16,17). Three cases of neuroretinitis have been neuroretinitis. Ann Ophthalmol 1991; 23: 58- 60. described in patients with multiple sclerosis ( 18), but re- 7. Lee AG, Brazis PW. Optic disc edema with a macular star and current neuroretinitis in association with multiple Sclerosis neuroretinitis. In: Lee AG, Brazis PW, eds. Clinical Pathways in . . . Neuro- Ophthalmology: An Evidence- Based Approach. New York: has not been reported. Thieme. 1998: 41_ 7. Purvin e t a l ( 11) also Studied the effects of long- term 8. Ormerod LD, Skolnick KA, Menosky MM, et al. Retinal and immunosuppression in patients with recurrent neuroretini- choroidal manifestations of cat- scratch disease. Ophthalmology 1998- 105- 1024- 31 tis. They retrospectively reviewed seven patients with re- 9 Drey; r RF> Hopen G> Gass JD> et al Leber> s idiopathic stellate current neuroretinitis who were treated with prednisone, neuroretinitis. Arch Ophthalmol 1984; 102: 1140- 5. azathioprine, Or both. The interval between attacks was 10- P u r v i n VA> c h i o r a n G- Recurrent neuroretinitis. Arch Ophthalmol F . ' . . 1994; 112: 365- 71. quite variable in their series, ranging from one month to n Purvin y Ranson N> Kawasaki A Idiopathic recurrent neuroretinitis: 9.8 years. For the entire group, the attack rate decreased effects of long- term immunosuppression. Arch Ophthalmol 2003; 72% after initiation of immunosuppressive therapy from 121: 65- 7. „ ,.„ , , r . . . . . r . 12. Kucukerdonmez C, Akova YA, Yilmaz G Ocular toxoplasmosis 0.58 attacks per year before the initiation of immunosup- presenting as neuroretinitis: report of two cases. Ocul Immunol pressive treatment to 0.16 attacks per year after treatment. inflamm 2002; 10: 229- 34. They concluded that long- term immunosuppressive therapy 13- Perrotta s> Nobili B> Grassia c> et al- Bilateal neuroretinitis in a , . i - i • 6- year- old boy with acquired toxoplasmosis. Arch Ophthalmol was a reasonable approach in these patients. 20031211493- 6 Our patient with recurrent neuroretinitis eventually 14. Garcia- Arumi J, Salvador F, Corcostegui B, et al. Neuroretinitis developed ulcerative colitis. Ophthalmic complications of associated with melanocytoma of the optic disk. Retina I994; l4: inflammatory bowel disease were first reported by Crohn l5 shLmugam MP, Khetan y sinha p. Optic disk melanocytoma with ( 19) in 1925, and he hypothesized that the two patients he neuroretinitis. 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