Susac's syndrome: an update.

Area of Science:

• Ophthalmology
• Neurology
• Rheumatology

Background:

• Susac's syndrome (SS) is an uncommon condition characterized by recurrent branch retinal arterial occlusions (BRAO), encephalopathy, and sensorineural hearing loss.
• Ophthalmic findings, particularly recurrent BRAO without intraocular inflammation, can be the sole presenting signs of SS.
• The classic triad of SS is rarely observed at initial presentation, leading to frequent misdiagnosis and underdiagnosis.

Purpose of the Study:

• To review the literature on Susac's syndrome, focusing on its ophthalmic manifestations, diagnostic challenges, and management.
• To highlight the importance of early diagnosis and treatment in preventing severe neurological and visual sequelae.
• To discuss the role of advanced imaging techniques in diagnosing SS.

Main Methods:

• Comprehensive literature review of Susac's syndrome.
• Analysis of diagnostic features, including characteristic retinal arterial wall plaques (Gass plaques).
• Evaluation of imaging modalities such as ultrawide field fluorescein angiography (FA) and optical coherence tomography (OCT).

Main Results:

• Recurrent multiple BRAO in the absence of intraocular inflammation is a key ophthalmological feature of SS.
• Yellowish retinal arterial wall plaques (Gass plaques) are commonly observed.
• Ultrawide field FA and OCT are valuable tools for diagnosis and monitoring.
• Patients without encephalopathy in the first two years are unlikely to develop it but remain at risk for recurrent BRAO and hearing loss.

Conclusions:

• A high index of suspicion is necessary for prompt diagnosis of SS.
• Early initiation of immunosuppressive therapy is vital for managing SS, although optimal regimens require further definition.
• Timely diagnosis and treatment can delay disease progression and prevent irreversible complications like blindness, deafness, and dementia.