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Clinical Characterization and Prognostic Risk Factors of Susac Syndrome: A Retrospective Multicenter Study.

Lior Fuchs¹, Adi Wilf-Yarkoni^1,2, Hadar Kolb^1,3

¹The Faculty of Medical & Health Sciences, Tel Aviv University.

Neurology(R) Neuroimmunology & Neuroinflammation

|December 18, 2024

Summary

Susac syndrome (SuS) is a rare neurological disorder. Severe brain involvement, male sex, and high CSF protein predict worse outcomes, necessitating intensive treatment for Susac syndrome patients.

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Area of Science:

Neurology
Ophthalmology
Otolaryngology

Background:

Susac syndrome (SuS) is a rare autoimmune disorder affecting the brain, retina, and inner ear.
Recent diagnostic criteria and treatment guidelines have been updated.
Understanding clinical manifestations and risk factors is crucial for patient management.

Purpose of the Study:

To examine clinical manifestations and disease activity in Susac syndrome.
To identify risk factors for disability, dependency, and return to work.
To evaluate treatment effectiveness and prognosis in SuS patients.

Main Methods:

Retrospective multicenter study of 20 Susac syndrome patients with ≥2 years follow-up.
Assessment of clinical and paraclinical activities, severity at onset and end of follow-up.
Evaluation of cognitive function (MoCA), disability (mRS), and employment status.

Main Results:

Mean age at onset 38.9 years; 45% presented with the complete triad.
Severe cerebral involvement at onset correlated with increased exacerbations, disability, and dependency.
Cognitive function improved in 75%; 50% resumed employment, 25% did not.

Conclusions:

Current treatments do not fully prevent relapses in Susac syndrome.
Severe brain onset, male sex, and elevated CSF protein are risk factors for poor prognosis.
High-frequency hearing loss does not rule out Susac syndrome diagnosis.