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Show Journal of'A] euro- Ophthalmology 21( 1): 18- 21, 2001. © 2001 Lippincott Williams & Wilkins, Inc., Philadelphia Reversible Blindness Resulting From Optic Chiasmitis Secondary to Systemic Lupus Erythematosus Larry P. Frohman, MD, Brett J. Frieman, DO, and Leo Wolansky, MD Objective: To report the diagnosis, radiologic findings, and therapy of a 51- year- old female with systemic lupus erythematosus ( SLE) who, while on hydroxychloroquine maintenance therapy, presented with a junctional scotoma indicative of chiasmal disease. This visual loss developed after she had been tapered off corticosteroids. Materials and Methods: An interventional case report of a female that was given acute therapy with 1- gram daily of intravenous methylprednisolone sodium succinate for 5 days, followed by maintenance methotrexate and a slow taper of oral prednisone. Magnetic resonance imaging ( MRI) scans, visual acuity, color vision, and threshold visual fields were performed. Results: The MRI scan showed chiasmal involvement, which may occur in SLE in absence of any other evidence of systemic activity. Therapy led to visual function returning to 20/ 20 OD and 20/ 20 OS, with normal Ishihara plates OU and only minimal paracentral depressions OU. She has been able to be weaned off prednisone while on methotrexate maintenance. Conclusions: Chiasmal involvement may occur in SLE in absence of any other evidence of systemic activity. Maintenance with hydroxychloroquine may not be adequate to prevent this rare cause of visual loss in SLE. Aggressive therapy of chiasmal involvement in SLE, even when the visual loss is profound, may lead to visual restoration, which was virtually complete in this case. Methotrexate may be an alternate agent for patients who break through with optic neuropathy while on hydroxychloroquine. Systemic lupus erythematosus ( SLE) is an autoimmune disorder that commonly affects the skin, musculoskeletal system, renal system, and, less commonly, the central nervous system and visual system. Most visual complaints from SLE result from retinopathy and anterior uveitis ( 1); optic neuropathy occurs less often. We report a unique case of a patient with SLE whose only Manuscript received December 1, 2000; accepted January 3, 2001. Supported in part by unrestricted grants from Research to Prevent Blindness, Inc. and the Eye Institute of New Jersey. From the Departments of Ophthalmology ( LPF, BJF), Neurosciences ( LPF), and Radiology ( LW), UMD- New Jersey Medical School, Newark, New Jersey. Address correspondence and reprint requests to Larry Frohman, MD, 90 Bergen Street, Newark, NJ 07103; e- mail: frohman@ umdnj. edu. manifestation of disease activity was chiasmal and posterior optic nerve inflammation confirmed by magnetic resonance imaging. Additionally, the patient's symptoms occurred while she had been taking 400 mg of hydroxychloroquine daily. Following high- dose corticosteroids, the patient had recovery of vision. CASE REPORT A 51- year- old female was referred to the neuro-ophthalmologist on June 11, 1996, to be monitored for signs of hydroxychloroquine- induced retinal toxicity. She had a 26- year history of SLE that was diagnosed after she developed a malar rash, arthritis, and mild renal changes; she also had elevated titers of anticardiolipin antibody of IgM idiotype. She was taking chlorthalidone, levothyroxine, estradiol, medroxyprogesterone, and baby aspirin at the time of presentation. Her ocular history was significant for two bouts of optic neuritis ( OU in 1990 and OD in 1994) with complete recovery of vision. She also had a retinal pigment epithelial detachment OD in 1990. The patient was taking 400 mg of hydroxychloroquine daily and had been on a tapering dose of prednisone ( at presentation to us, 5 mg and 2.5 mg on alternate days) since the second bout of optic neuritis. The initial neuro- ophthalmology examination ( 2 years before the chiasmal involvement) showed best- corrected acuity of 20/ 25 OD and 20/ 20 OS. Ishihara color plates were 5.5/ 6 correct OU. The pupillary reactions were normal. The slit lamp exam, intraocular pressure, and ocular rotations were normal. She had 1 mm of ptosis OS with good levator function ( present since 1992). The central 24- degree threshold field OD was normal centrally with some mild general constriction worse nasally. The field OS was normal except for some mild general peripheral constriction. Despite the prior history, the discs were pink, sharp, with a cup to disc ratio of 0.1 OU. The vessels and periphery were normal. There was residuum of a retinal pigment epithelial detachment along the superotemporal arcade OD. The retina was otherwise normal. She was followed and was stable for 2 years. Her rheumatologist weaned her off prednisone in early June 1998 after improvement of 18 LUPUS CHIASMITIS 19 FIG. 1. A: Axial Short Tl ( tau) Inversion Recovery ( STIR) image ( repetition time [ TR] 3500, Tl ( tau) 165, echo time [ TE] 75). B: Oblique axial reformatted volume acquisition T2- weighted image. C: Coronal T2- weighted image ( TR 4000, TE 75). D: Axial post-contrast T1- weighted images ( TR 600, TE 22). E: Coronal post-contrast T1- weighted images ( TR 600, TE 26). On the long TR images, abnormal hyperintensity is seen in the optic chiasm, tracts, and nerves ( black arrowheads in A, B, and C) in comparison to the normal signal seen in the cerebral white matter ( A). There is a striking size difference of the spared, hypointense, intraorbital segments of the optic nerves ( white arrows in A and B) in comparison to the edematous cisternal segments ( black arrowheads in A and B). Also noted is relative sparing of the inferior surface of the cisternal segment bilaterally ( C). Postcon-trast, there is extensive enhancement of the chiasm and nerves ( arrowheads in D and E). J Neuro- Ophthalmol, Vol. 21, No. 1, 2001 20 L. P. FROHMAN ET AL. her arthralgias. She remained on 400 mg of hydroxychloroquine daily. On July 8, 1998, 1 month after discontinuing corticosteroids, she was seen emergently for bilateral painless visual loss that she had noted for 2 weeks. Upon examination, the patient was light perception with projection OD and 20/ 70 OS. Color plates were 0/ 6 in the left. The right pupil barely reacted to light at 6 mm; the left was 6 mm. A right afferent pupillary defect was present. Extraocular movements were full. The slit lamp examination was unremarkable, and the intraocular pressures were 14 OU. The visual field OS showed a nearly complete temporal hemifield defect ( e. g., a junctional scotoma was present). The fundus examination did not show any new changes except for mild temporal pallor of the disc OD. The cranial nerve examination was normal as was the rest of the neurologic exam. The patient was admitted for treatment with intravenous steroids and further work- up. She was started on a 5- day course of 1 g of intravenous methylprednisone. The day after admission, her visual acuity declined to light perception OD and 20/ 80 OS. Laboratory results included a complete blood count that showed an elevated white blood count at 13.1 T/ uL ( normal 4.5- 11.0 T/ uL). Chemistry 12 panel showed only an elevated glucose of 201 mg/ dl ( normal 70- 109 mg/ dL). Thyroid function test, prothrombin time, activated partial thromboplastin time, and urinalysis were all within normal limits. IgG was 1700 mg/ dL ( normal 691- 1618 mg/ dL) and IgM was 334 mg/ dL ( normal 60- 265 mg/ dL). Complement 3 was 76 mg/ dL ( normal 88- 201 mg/ dL), whereas complement 4 was 18.7 mg/ mL ( normal 15.7^ 17.2 mg/ dL). C- reactive protein was negative. Vitamin B12 was 964 pg/ mL ( normal 190- 1060 pg/ mL). Antinuclear antibody screen was positive with a titer greater than 1: 640 ( negative 1: 40). Anti- double-stranded DNA was negative with a titer of 1 to 10. Varicella IgG was positive. Epstein- Barr virus IgG was positive, whereas IgM was negative. Lyme titer was also negative. MRI showed a diffuse thickening, edema, and enhancement of the optic chiasm, tracts, and cisternal segments of the optic nerves ( Fig. 1). Lumbar puncture was performed, the cerebrospinal fluid ( CSF) was clear, CSF glucose was 84 mg/ dL ( normal 40- 80 mg/ dL), and the CSF protein was 74 mg/ mL ( normal 15^ 45 mg/ dL). The red and white cell counts were normal and the Venereal Disease Research Laboratory ( VDRL) was nonreactive. Gram stain and all cultures were negative. She was discharged on 80 mg of oral prednisone daily and 15 mg of methotrexate weekly. One week later, her vision improved to 20/ 800 OD and 20/ 80 OS, and the visual field OS improved to show a cecocentral scotoma. Two weeks after discharge, a slow prednisone taper was begun. By 1 month after discharge, her vision improved to 20/ 25 OD and 20/ 20 OS, and her color vision had also improved to 1/ 6 OD and 4.5/ 6 OS. Her right visual field showed a central and paracentral depression and a couple of scattered islands of depression. The OS was normal with the exception of diminished color vision. She was removed from prednisone 13 months after discharge. At last follow- up examination in January 2001, she was on methotrexate ( 15 mg each week), calcitonin, estradiol, medroxyprogesterone, baby aspirin, and 400 mg of hydroxychloroquine. Her visual acuity was 20/ 20 OD and 20/ 20 OS. Ishihara color plates were 100% correct OU. She no longer had a relative afferent pupillary defect. She had mild temporal disc pallor OU. Her threshold visual field had minimal paracentral depression OU. She has not had any further manifestations of her lupus. DISCUSSION Upon review of the literature, two reports mention patients with SLE and chiasmal involvement. Sklar et al. ( 2) mention a case of SLE involving the optic chiasm but did not present details of the case. They discussed the benefits of MRI with gadopentetate dimeglumine and fat suppression to detect optic neuropathy. Jabs et al. ( 3) report data of a patient with retrobulbar optic neuropathy and chiasmal syndrome that occurred with a systemic flare of SLE. We have previously reported a case of chiasmitis with swollen chiasm demonstrated on metriz-amide cisternogram in a patient with autoimmune optic neuropathy, an illness where patients may have some features of SLE but do not meet clinical criteria for its diagnosis ( 4). To the authors' knowledge, this article is the first case report of a patient where chiasmal involvement in SLE occurred without other active systemic manifestations of SLE. The many ocular manifestations of SLE include retinopathy, anterior ischemic optic neuropathy, retrobulbar ischemic optic neuropathy, papillitis, or retrobulbar neuritis ( 1,3). These presentations of SLE are rare and are not included in the criteria for classification of SLE established by the American Rheumatoid Association ( 5). Based on the patient's prior symptoms, she fit the criteria for, and was diagnosed with, SLE before the development of optic neuropathy ( 6). After other causes were ruled out, SLE was determined to be the cause of her chiasmal optic neuritis. SLE is believed to be caused by an autoimmune- induced vaso- occlusion occurring in small vessels ( 3,7). The inflammatory response may result from the presence of antiphospholipid antibodies that are often found in patients with SLE; alternately, these antibodies may be a marker for disease activity ( 8). Hydroxychloroquine did not protect our patient against the recurrence of optic neuritis. She responded well to corticosteroids, however, and her visual outcome was excellent. The overall response to steroids in previous cases has varied from full recovery to complete loss of vision ( 7,9). To prevent any reoccurrence, the patient was placed on a slow taper of steroids and methotrexate. Methotrexate is an alternative immunosuppressant that may be used in cases where a flare of SLE, such as optic neuropathy, occurs in a patient on hydroxychloroquine ( 10). / Neuro- Ophthalmol, Vol. 21, No. 1, 2001 LUPUS CHIASMITIS 21 REFERENCES 1. Drosos AA, Petris CA, Petroutsos GM, et al. Unusual eye manifestations in systemic lupus erythematosus patients. Clin Rheumatol 1989; 8: 49- 53. 2. Sklar EM, Schatz NJ, Glaser JS, et al. MR of vasculitis- induced optic neuropathy. AJNR Am J Neuroradiol 1996; 17: 121- 8. 3. Jabs DA, Miller NR, Newman SA, et al. Optic neuropathy in systemic lupus erythematosis. Arch Ophthalmol 1986; 104: 564- 8. 4. Frohman LP, Pinto R, Warren F, et al. Metrizamide computed tomography cisternography: a high- field diagnostic procedure for chiasmal syndromes with equivocal computed tomographic or magnetic resonance scans. A'euro- ophthalmology 1987; 7: 251- 66. 5. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982; 25: 1271- 7. 6. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997; 40: 1725. 7. Roodpeyma AH, Azarmina M, Soheilian M, et al. Bilateral simultaneous optic neuritis in childhood systemic lupus erythematosus. A case report. J Neuroophthalmol 1994; 14: 84- 6. 8. Cordeiro MF, Lloyd ME, Spalton DJ, et al. Ischaemic optic neuropathy, transverse myelitis, and epilepsy in an anti- phospholipid positive patient with systemic lupus erythematosus. J Neurol Neu-rosurg Psychiatry 1994; 57: 1142- 3. 9. Smith CA, Pinals RS. Optic neuritis in systemic lupus erythematosus. J Rheumatol 1982; 9: 963- 6. 10. Rahman P, Humphrey- Murto S, Gladman DD, et al. Efficacy and tolerability of methotrexate in antimalarial resistant lupus arthritis. J Rheumatol 1998; 25: 243- 6. 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