Susac's syndrome: clinical course and epidemiology in a Central European population.

OBJECTIVE: Susac's syndrome is characterized by inflammation and occlusion of pre-capillary arterioles with the clinical triad of branch retinal artery occlusion (BRAO), encephalopathy and hearing loss. No epidemiological data are available for the disease. METHODS: All neurology departments in Austria were addressed to report adult patients who were on immunosuppressive treatment for a diagnosis of Susac's syndrome between 1 August 2010 and 1 August 2015. Clinical course, treatment regimens, period and point prevalence rates, and annual incidence of Susac's syndrome in Austria in people over 19 years of age are reported. RESULTS: Ten patients with Susac's syndrome were identified, and eight of them were newly diagnosed within the five-year timeframe. Minimum five-year period prevalence of the disease is 0.148/100,000 (95% confidence interval (CI) 0.071-0.272), annual incidence is 0.024/100,000 (95% CI 0.010-0.047). Minimum point prevalence rates varied from 0.030/100,000 (95% CI 0.004-0.108) to 0.088/100,000 (95% CI 0.032-0.192). Of all 10 patients, 8 showed typical callosal or internal capsule magnetic resonance imaging lesions at first presentation, 7 presented with BRAO and 5 had hearing loss or tinnitus at the beginning of the disease. Four patients developed the complete clinical triad of Susac's syndrome during the observation period. CONCLUSIONS: We provide for the first time population-based data about the clinical course, prevalence and incidence of Susac's syndrome.

Surgical management of severe complications arising from uveitis in juvenile idiopathic arthritis.

PURPOSE: Evaluate outcomes from severe ocular complications of juvenile idiopathic arthritis following surgery. METHODS: Eleven eyes of 7 patients underwent complete vitrectomy and peeling of the inner limiting membrane. Inclusion criteria were: anteroposterior segment involvement, hypotony, inflammation control of less than 3 months, compliance issues, rapidly progressive disease. Phacoemulsification was allowed if the patient was >6 years old and inflammation free >3 months. The alternative was a complete lensectomy. RESULTS: Visual acuity improved from a logMAR of 1.48 to 0.37 (p < 0.0001), and 0.20 at 6 and 12 months (p < 0.0001). No flare-up was observed within the first 6 months. Five eyes developed inflammation between 7 and 19 months. Glaucoma developed in 5 eyes at a median of 16 months. No patient developed cystoid macular edema. CONCLUSION: Extensive pars plana vitrectomy and cataract extraction can lead to significant improvement in visual acuity. Patients continue to require long-term immunosuppression and adequate follow-up.

Intravitreal methotrexate in the management of presumed tuberculous serpiginous-like choroiditis.

PURPOSE: To report on the use of intravitreal methotrexate (IVT MTX) as part of treatment of presumed tuberculous serpiginous-like choroiditis progressing despite the use of tuberculostatics. METHODS: Case series of patients suffering from serpiginous-like choroiditis with positive tuberculin skin test who received IVT injections of MTX as part of treatment. Ocular disease was active despite the use of systemic tuberculostatic (isoniazid, rifampicin, pyrazinamide, and ethambutol), and choroidal lesions showed signs of progression. A single injection of IVT MTX (400 µg/0.1 mL) was administered in the eye with macular-threatening features. Change in visual acuity, appearance of the lesion, and staining patterns on angiography were among the main outcome measurements. RESULTS: Three eyes from two patients were included. Both cases presented bilateral involvement with mild vitritis. In all three eyes, choroidal lesions healed within the first month after an IVT MTX injection with visual acuity improvement in two. No adverse reaction was related to the medication or to the procedure. Patients were followed for a mean of 13.5 months after being injected. CONCLUSION: The use of IVT MTX seems effective in the management of the inflammatory component of tuberculous serpiginous-like choroiditis, whereas systemic tuberculostatics are aimed at controlling the infectious one.

Intravitreal ganciclovir in the management of non-AIDS-related human cytomegalovirus retinitis.

OBJECTIVE: To report and evaluate intravitreal ganciclovir injections in non-AIDS patients with human cytomegalovirus (HCMV) retinitis. DESIGN: Retrospective chart review. PARTICIPANTS: Two SLE patients and one patient post chemotherapy for a non Hodgkin's lymphoma presented with myelosuppression and persistent cytomegalovirus retinitis despite systemic ganciclovir therapy. METHODS: Patients were treated with 100 microL of intravitreal ganciclovir (4 mg/dL), initially given weekly. Systemic anti-CMV medication was stopped, and following quiescence, intravitreal injections were tapered and ultimately stopped based on therapeutic response. Patients were followed periodically for signs of recurrence. RESULTS: Intravitreal ganciclovir was well tolerated and led to remission of the retinitis in 2 patients. One patient had persistent smouldering disease and reached quiescence using an intravitreal ganciclovir implant. Fluorescence-activated cell sorting analysis in one patient showed the presence of low CD4 and CD8 while treated with systemic ganciclovir, which improved with intravitreal treatment. In another, the low ratio was maintained against cytomegalovirus-specific antigens. CONCLUSIONS: Intravitreal ganciclovir injections should be considered as a treatment option in selected iatrogenically immunocompromised patients with HCMV retinitis. Responses may vary and will require an adjusted approach to treatment.