Cyclosporine-Associated Leukoencephalopathy in a Case of Sympathetic Ophthalmitis

Abstract

Purpose: Cyclosporine (CsA) is currently widely used as a primary immunosuppressive agent in ocular disease, particularly in severe uveitis. Posterior reversible encephalopathy syndrome (PRES) is a significant complication of CsA therapy. However, there are no reports of the occurrence of PRES in response to the treatment of uveitis in the ophthalmological area.

Case presentation: We report a case with CsA-associated PRES. A 70-year-old woman with sympathetic ophthalmitis was treated with 50 mg/day of CsA for 1 week. However, the trough level in her blood was too low; thus, we increased the dose to 100 mg/day of CsA with prednisolone. She had headaches, hypertension (systolic blood pressure 180-200 mm Hg), loss of consciousness for several hours, and reduced limb movement, and her MRI showed a high signal intensity in both posterior lobes, consistent with PRES. Examination of the cerebrospinal fluid indicated that it was within normal limits. Her CsA trough level in the blood was within normal ranges on the day of the attack. Her symptoms gradually improved over the next several days; however, she presented with cortical blindness, which lasted for several weeks. Finally, she returned to her baseline values from before the attack. Her MRI findings showed that PRES had essentially disappeared.

Conclusion: PRES is not directly associated with the dosage of CsA administered; however, in general, it is well known that PRES can affect strongly immunosuppressed cases undergoing organ and bone marrow transplantation. Nevertheless, our CsA dose was only 100 mg (1.8 mg/kg). In this study, we report on the occurrence of PRES after the administration of CsA to treat sympathetic ophthalmia. To our knowledge, PRES can also occur after the administration of a small dose of CsA; thus, ophthalmologists using CsA should carefully observe the systemic conditions of CsA-treated patients.

Keywords: Cyclosporine; Posterior reversible encephalopathy syndrome; Sympathetic ophthalmitis.

Fig. 1

a Fundus photography before any treatment. The fundus is nearly invisible because of the opacification of the vitreous cavity. b Optical coherence tomography shows choroidal folds and serious retinal fluid near the posterior pole. c Fundus photography after 6 months of treatment. The fundus is nearly clear. d Optical coherence tomography shows the disappearance of choroidal folds and serious retinal fluid near the posterior pole.

Fig. 2

Clinical time course. Administration of Ca blockers was ceased because the patient's systolic blood pressure was much too low; after the attack, it increased significantly and was too high. PSL = Prednisolone.

Fig. 3

MRI shows hyperintense imaging on T2. Fluid-attenuated inversion recovery sequences. a Axial views show hyperintensity on both posterior lobes on the day of the attack. b Axial views show that dot hyperintensity spots remain on both posterior lobes 6 months after the day of the attack.