Journal of Neuro-Ophthalmology, December 2013, Volume 33, Issue 4

Optic Perineuritis Secondary to Acute Retinal Necrosis

REFERENCES 1. Ahmed RM, King J, Gibson J, Buckland ME, Gupta R, Gonzales M, Halmagyi M. Spinal leptomeningeal lymphoma presenting as pseudotumor syndrome. J Neuroophthalmol. 2013;33:13-16. 2. Moodley KK, Broad R, Chung K, Riordan-Eva P, Sibtain NA, Moran NF. Sheehan syndrome associated with raised intracranial pressure. J Neuroophthalmol. 2013;33:54-57. 3. Mamourian AC, Towfighi J. MR of giant arachnoid granulation, a normal variant presenting as a mass within the dural venous sinus. AJNR Am J Neuroradiol. 1995;16(suppl):901-904. 4. Roche J, Warner D. Arachnoid granulations in the transverse and sigmoid sinuses: CT, MR, and MR angiographic appearance of a normal anatomic variation. AJNRAm J Neuroradiol. 1996;17:677-683. 5. Peters SA, Frombach E, Heyer CM. Giant arachnoid granulation: differential diagnosis of acute headache. Australas Radiol. 2007;51(suppl 1):B18-B20. 6. Arjona A, Delgado F, Fernandez-Romero E. Intracranial hypertension secondary to giant arachnoid granulation, J Neurol Neurosurg Psychiatry. 2003;74:418. 7. Choi HJ, Cho CW, Kim YS, Cha JH. Giant arachnoid granulation misdiagnosed as transverse sinus thrombosis. J Korean Neurosurg Soc. 2008;43:48-50. 8. Zheng Z, Zhou M, Zhao B, Zhou D, He L. Pseudotumor cerebri syndrome and giant arachnoid granulation: treatment with venous sinus stenting. J Vasc Interv Radiol. 2010;21:927-929. Optic Perineuritis Secondary to Acute Retinal Necrosis Optic perineuritis (OPN) has been recognized as a form of idiopathic orbital inflammatory disease, where the specific target is the optic nerve sheath (1-4). Most reported cases are isolated and idiopathic, but some have been associated with specific infectious or inflammatory disorders, including Wegener granulomatosis, giant cell arteritis, syphilis, and viral meningitis (5-8). A recent report by Townsend et al (9) documented OPN as a pre-senting finding in a patient with leukemia. We evaluated a patient with acute retinal necrosis (ARN) who subse-quently developed OPN. A 67-year-old man reported decreased vision in his left eye. Examination revealed visual acuity of 20/80 with cells in the anterior chamber, multiple patchy areas of necrosis in the peripheral retina, retinal vasculitis, optic disc swelling and vitritis (Fig. 1A). He was diagnosed with ARN and treated with acyclovir 800 mg intravenously 3 times a day. Despite antiviral therapy, vision declined to 20/200 in the left eye. Ganciclovir (2 mg/0.1 mL) was injected into the vitreous cavity, and the patient was prescribed oral fam-ciclovir 500 mg, 3 times a day. On day 7, oral prednisolone (60 mg/day) was added. Prophylactic vitrectomy was per-formed with a silicone oil tamponade, and the patient's vision gradually improved to 20/100 (Fig. 1B). Systemic corticosteroids were tapered over 3 weeks and then discontinued, while maintaining oral famciclovir. After 3 days, the patient reported pain with left eye movement and had no light perception in the left eye. The fundus appearance was unchanged, and a fluorescein angiography showed no specific findings to explain the vision loss. Flash visual evoked potential was nonrecordable in the left eye. Although magnetic resonance image (MRI) of the brain appeared normal, there was enhancement of the left optic nerve sheath (Fig. 2). Cerebrospinal fluid analysis was un-remarkable. The patient was given high-dose intravenous methylprednisolone (1 g/day) for 3 days, followed by oral steroids and maintained on famciclovir. Visual acuity in the left eye recovered to 20/500 at 2 months, without further improvement over the following 10 months. Optic nerve involvement in ARN has been reported in 47%-57% of cases (10-12). Proposed mechanisms include intraneural vasculitis, direct viral invasion of the optic nerve, FIG. 1. A, Left fundus shows peripheral retinal necrosis, optic disc swelling, and vitreous haziness. B, After systemic therapy and vitrectomy with silicone oil tamponade, there is resolution of retinal necrosis and optic disc swelling with sheathing of the retinal vessels and optic disc pallor. Letters to the Editor: J Neuro-Ophthalmol 2013; 33: 412-423 419 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. and compression caused by inflammatory exudate within the optic nerve sheath (11,13,14). Our case is consistent with the last proposed mechanism producing the neuro-imaging findings of OPN. Sergott et al (11) demonstrated enlargement of the optic nerve sheath in 2 patients with ARN. Because their report preceded the advent of MRI, they based the proposed mechanism on operative findings of optic nerve fenestration in 1 patient. After incision of the nerve sheath, they described: "a large gush of serosangui-nous CSF," which led to an improvement in visual acuity. Once antiviral therapy has been initiated, systemic steroid therapy often is used in patients with ARN and ARN-associated optic neuropathy to reduce inflammation. However, the effect and duration of steroid treatment remains controversial, because there are no controlled studies of its efficacy. Optic perineuriti associated with ARN has not been reported previously. In our patient, it developed during antiviral treatment and shortly after steroid therapy was discontinued. It may have been because of relapse or delayed onset of disease within the optic nerve. Ik Soo Byon, MD Department of Ophthalmology, Pusan National University Yangsan Hospital, Yangsan, Korea Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, Korea Jae Ho Jung, MD, PhD Department of Ophthalmology, Pusan National University Yangsan Hospital, Yangsan, Korea Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, Korea Medical Institute, School of Medicine, Pusan National University, Busan, Korea Ho Yun Kim, MD Sung Who Park, MD Department of Ophthalmology, Pusan National University Hospital, Busan, Korea Biomedical Research Institute, Pusan National University Hospital, Busan, Korea Ji Eun Lee, MD, PhD Medical Institute, School of Medicine, Pusan National University, Busan, Korea Department of Ophthalmology, Pusan National University Hospital, Busan, Korea Biomedical Research Institute, Pusan National University Hospital, Busan, Korea [e-mail] The authors report no conflicts of interest. REFERENCES 1. Kennerdell JS, Dresner SC. The nonspecific orbital inflammatory syndrome. Surv Ophthalmol. 1984;29:93-103. 2. Sekhar GC, Mandal AK, Vyas P. Nonspecific orbital inflammatory diseases. Doc Ophthalmol. 1993;84:155-170. 3. Miller NR, Newman NJ. Walsh and Hoyt's Clinical Neuro- Ophthalmology, 5th edition. Baltimore, MA: Lippincott Williams & Wilkins, 1998. 4. Purvin V, Kawasaki A, Jacobson DM. Optic perineuritis: clinical and radiologic features. Arch ophthalmol. 2001;119:1299- 1306. 5. Nassani S, Cocito L, Arcuri T, Favale E. Orbital pseudotumor as a presenting sign of temporal arteritis. Clin Exp Rheumatol. 1995;13:367-369. 6. Oh HG, Yang KI. A case of optic perineuritis associated with seronegative neurosyphilis. J Korean Geriatr Soc. 2007;11:98- 100. 7. Dutton JJ, Anderson RL. Idiopathic inflammatory periodic neuritis simulating optic nerve sheath meningioma. Am J Ophthlamol. 1985;100:424-430. 8. Rush JA, Ryan EJ. Syphilitic optic perineuritis. Am J Ophthalmol. 1981;91:404-406. 9. Townsend J, Dubovy SR, Pasol J, Lam BL. Transient optic perineuritis as the initial presentation of central nervous system involvement by pre-B cell lymphocytic leukemia. J Neuroophthalmol. 2013;33:162-164. 10. Muthiah MN, Michaelides M, Child CS, Mitchell SM. Acute retinal necrosis: a national population-based study to assess the incidence, methods of diagnosis, treatment strategies and outcomes in the UK. Br J Ophthalmol. 2007;91:1452-1455. 11. Sergott RC, Belmont JB, Saino PJ, Fischer DH, Bosley TM, Schatz NJ. Optic nerve involvement in the acute retinal necrosis syndrome. Arch Ophthalmol. 1985;103:1160-1162. 12. Batisse D, Eliaszewicz M, Zazoun L, Baudrimont M, Pialoux G, Dupont B. Acute retinal necrosis in the course of AIDS: study of 26 cases. AIDS. 1996;10:55-60. 13. Culbertson WW, Blumenkranz MS, Pepose JS, Stewart JA, Curtin VT. Varicella zoster virus is a cause of the acute retinal necrosis syndrome. Ophthalmology. 1986;93:559-569. 14. Witmer MT, Pavan PR, Fouraker BD, Levy-Clarke GA. Acute retinal necrosis associated optic neuropathy. Acta Ophthalmol. 2011;89:599-607. FIG. 2. Postcontrast T1 axial orbital magnetic resonance imaging with fat suppression shows enhancement of the left optic nerve sheath (arrow). 420 Letters to the Editor: J Neuro-Ophthalmol 2013; 33: 412-423 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.