Susac syndrome: neurological update (clinical features, long-term observational follow-up and management of sixteen patients).
Author
Bose, SmritiPapathanasiou, Athanasios
Karkhanis, Sameep
Appleton, Jason P
King, Dominic
Batra, Ruchika
Mollan, Susan P
Jacob, Saiju
Publication date
2023-08-22Subject
Neurology
Metadata
Show full item recordAbstract
Abstract Susac syndrome is a likely autoimmune microangiopathy affecting the brain, retina and inner ear. Due to the rarity of this condition, diagnosis and treatment can be challenging. Diagnosis is based on the presence of the clinical triad of central nervous system dysfunction, branch retinal artery occlusions and sensorineural hearing loss. Typical MRI findings of callosal and peri-callosal lesions may assist in diagnosis. Clinical course can be monophasic, polycyclic or chronic continuous. It is important to look out for red flags to attain an accurate diagnosis and follow a therapeutic algorithm based on severity of the disease and response to treatment. Patients are treated with steroids and immunosuppressive agents with a variable response. Early aggressive treatment especially in severe cases, may help in preventing relapses and morbidity/disability. This study highlights important diagnostic features and proposes a treatment algorithm based on clinical experience from management of 16 patients from 2 neuroscience centres in the UK since 2007, who were followed up over a long period of 3-15 years. Keywords: Branch retinal artery occlusion; Encephalopathy; Sensorineural hearing loss; Susac syndrome; Visual loss.Citation
Bose, S., Papathanasiou, A., Karkhanis, S., Appleton, J. P., King, D., Batra, R., Mollan, S. P., & Jacob, S. (2023). Susac syndrome: neurological update (clinical features, long-term observational follow-up and management of sixteen patients). Journal of neurology, 10.1007/s00415-023-11891-z. Advance online publication. https://doi.org/10.1007/s00415-023-11891-zType
ArticleAdditional Links
https://link.springer.com/journal/415PMID
37608221Journal
Journal of NeurologyPublisher
Springerae974a485f413a2113503eed53cd6c53
10.1007/s00415-023-11891-z