Related Experiment Video
Updated: Jun 28, 2025
05:22
Iris Fixation via External Pentagram Suturing
Published on: May 5, 2022
1.3K
Ophthalmological Outcomes in Patients with Susac Syndrome
Angelika Schuknecht1, Maximilian Robert Justus Wiest1, Sadiq Said1
1Department of Ophthalmology, Universitätsspital Zürich, Switzerland.
Klinische Monatsblatter Fur Augenheilkunde
|April 23, 2024
Summary
Treatment for Susac syndrome, a rare autoimmune condition, significantly improves visual acuity and reduces disease activity. Most patients require systemic steroids combined with other immunosuppressive agents for effective management.
Area of Science:
- Ophthalmology
- Neurology
- Immunology
Background:
- Susac syndrome is a rare autoimmune vascular endotheliopathy affecting the brain, retina, and inner ear.
- Ophthalmological manifestations are a key feature of Susac syndrome, impacting visual acuity.
Purpose of the Study:
- To report ophthalmological outcomes in patients diagnosed with Susac syndrome.
- To describe the treatment regimens employed for Susac syndrome.
- To evaluate the effectiveness of various treatments on visual acuity and disease activity.
Main Methods:
- Retrospective analysis of Susac syndrome patients treated between November 2015 and March 2023.
- Review of multimodal imaging, ophthalmic examinations, neurological and sensorineural data, and therapeutic regimens.
- Assessment of visual acuity using logMAR and disease severity using the clinical activity score (CAS).
Main Results:
- Ten Susac syndrome patients (5 male:5 female) were analyzed with a mean follow-up of 31.2 months.
- Corrected distance visual acuity (CDVA) improved significantly from baseline (0.03 logMAR) to last follow-up (0.00 logMAR) (p=0.029).
- Clinical activity score (CAS) decreased from a mean of 10.65 at baseline to 5.15 at follow-up (p=0.068). Nine patients treated for over 1 month showed improvement in CAS and CDVA.
Conclusions:
- Treatment for Susac syndrome leads to improved ophthalmological outcomes, including enhanced CDVA and reduced CAS.
- Systemic steroids are a primary treatment, often necessitating additional systemic immunosuppressive agents like cyclophosphamide, IVIGs, or anti-CD20 antibodies.
- Interdisciplinary collaboration is vital for timely diagnosis and initiation of therapy in Susac syndrome patients to optimize outcomes.
Related Concept Videos
01:28Angle Closure Glaucoma: Treatment
485
Angle-closure glaucoma, or closed-angle glaucoma, is an eye condition where the iris bulges out and blocks the iridocorneal angle, resulting in a buildup of aqueous humor and increased intraocular pressure. Immediate medical attention is necessary due to the sudden onset of symptoms. The treatment for angle-closure glaucoma includes short-term and long-term approaches. Short-term treatment involves using eye drops like pilocarpine to lower intraocular pressure by increasing aqueous humor...
485
01:25Glaucoma: Overview
560
Glaucoma is an eye condition characterized by increased intraocular pressure that damages the retina and optic nerve, leading to irreversible blindness if left untreated. The human eye has various components, including the cornea, iris, pupil, lens, and optic nerve. Aqueous humor is secreted by the epithelium of the ciliary body in the posterior chamber and flows through the trabecular meshwork and canal of Schlemm, maintaining normal intraocular pressure. The trabecular meshwork and the canal...
560
01:27Open Angle Glaucoma: Treatment
440
In open-angle glaucoma, the iridocorneal angle remains open, but the trabecular meshwork becomes stiff, slowing down the outflow of aqueous humor. This causes a buildup of aqueous humor in the anterior chamber, leading to a sudden increase in intraocular pressure. The treatment for open-angle glaucoma focuses on reducing the elevated intraocular pressure by either decreasing the secretion of aqueous humor or increasing its outflow.
Drugs such as carbonic anhydrase inhibitors, α2- and...
Drugs such as carbonic anhydrase inhibitors, α2- and...
440