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Published on: April 25, 2025
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Diagnostic Criteria and Treatment Algorithm for Susac Syndrome
1Department of Neurology and Ophthalmology, Rockwood Clinic Neuro-Ophthalmology, Spokane, Washington.
Summary
Susac syndrome (SS) diagnosis can be challenging due to incomplete clinical or imaging findings. Arteriolar wall hyperfluorescence and central callosal lesions are key diagnostic indicators, guiding prompt and aggressive treatment.
Area of Science:
- Ophthalmology
- Neurology
- Rheumatology
Background:
- Susac syndrome (SS) classically presents with a triad of retinal artery occlusion, sensorineural hearing loss, and encephalopathy.
- Diagnosis can be complicated by incomplete clinical or neuroimaging presentations.
- A standardized treatment protocol for SS is currently lacking.
Purpose of the Study:
- To review the diagnostic criteria for Susac syndrome.
- To highlight key imaging findings that confirm the diagnosis.
- To outline current treatment options for Susac syndrome.
Main Methods:
- Literature review of medical publications on Susac syndrome.
- Analysis of clinical and neuroimaging diagnostic criteria.
- Summary of reported treatment modalities.
Main Results:
- Definitive diagnosis requires the presence of the clinical or neuroimaging triad.
- Arteriolar wall hyperfluorescence (AWH) and central callosal lesions are pathognomonic imaging findings.
- Gass plaques in retinal arterioles suggest SS but are not pathognomonic.
Conclusions:
- AWH and central callosal lesions confirm SS diagnosis in ambiguous cases.
- Prompt and aggressive treatment is crucial for Susac syndrome patients.
- Treatment may involve corticosteroids, IV immunoglobulin, mycophenolate mofetil, rituximab, or cyclophosphamide for refractory cases.
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