Susac Syndrome: A Systematic Review and Survival Analysis.

Area of Science:

• Neurology
• Ophthalmology
• Oto-rhino-laryngology

Background:

• Susac syndrome is a rare neurological disorder characterized by the triad of encephalopathy, branch retinal artery occlusions (BRAOs), and sensorineural hearing loss.
• The syndrome presents a significant clinical challenge due to its heterogeneous manifestations and potential for severe sequelae.

Purpose of the Study:

• To conduct a systematic review and survival analysis of all published Susac syndrome cases.
• To analyze the clinical characteristics, treatment outcomes, and survival rates of patients with Susac syndrome.

Main Methods:

• Systematic review and survival analysis conforming to the PRISMA statement.
• Inclusion of data from 176 studies, encompassing 435 patients diagnosed with Susac syndrome.
• Analysis of overall survival (OS) and progression-free survival (PFS).

Main Results:

• Neurological symptoms were most frequent (87.4%), followed by audio-vestibular (84.3%) and ophthalmological symptoms (78.9%).
• The complete triad at onset was present in only 20.1% of cases.
• Complete recovery rates varied by symptom involvement: 59.5% for neurological, 39.7% for ophthalmological, and 17.7% for audio-vestibular.
• One-year and three-year OS were 94% and 1-year and 3-year PFS were 89% and 83%, respectively.
• In a subgroup with follow-up data, 82.1% remained stable, 12.2% relapsed, and 5.7% died.

Conclusions:

• Susac syndrome exhibits highly heterogeneous presentations and outcomes.
• Mortality associated with Susac syndrome is uncommon, but the potential for relapse and long-term sequelae is significant.
• Increased awareness and a deeper understanding of Susac syndrome are crucial for improved patient management.