Successful Treatment of Incomplete Susac Syndrome with Simultaneous Corticosteroids and Plasmapheresis Followed by Rituximab

Abstract

We present a case report of a patient with incomplete Susac syndrome. He had cognitive impairment, corpus callosum lesions, and vestibulocochlear dysfunction on brainstem auditory evoked responses. He was treated with methylprednisolone and plasmapheresis, improved, and then, also received rituximab. His improvement has been lasting as of this writing. This case shares our experience with a successful treatment of this rare condition that is incompletely understood and lacks well-established treatment guidelines.

Case report: Susac syndrome—two ends of the spectrum, single center case reports and review of the literature

“…Plasmapheresis is used less commonly in SuS. Limited evidence available in literature points to reasonable effectiveness of this treatment modality (27,44). In this patient, it led to stabilization, even though only four cycles were performed due to the patient developing asymptomatic coagulopathy.…”

“…In patients with severe and extremely severe CNS-predominant SuS, early addition of rituximab (as an initiation 2 × 1 g with a gap of 14 days, then repeated after 4-6 and 12 months) is strongly recommended (9,12,42). The clinical experience confirms that it is efficient, although only few cases were reported (8,14,27,(42)(43)(44)(45)(46). Equally, ocrelizumab as an alternative may be considered despite the lack of clinical experience (9).…”

Successful treatment of a complete Susac syndrome in a 16‐year‐old boy: A case report

“… 21 Empirical treatment including high‐dose corticosteroids is recommended as initial treatment of SuS. 22 The use of corticosteroids is an appropriate treatment plan for SuS because the disease is caused by inflammatory processes affecting the brain, retina, and ears. 10 , 22 There are other medications, such as azathioprine, mycophenolate mofetil, cyclophosphamide, and rituximab, that are recommended for autoimmune diseases.…”

“… 22 The use of corticosteroids is an appropriate treatment plan for SuS because the disease is caused by inflammatory processes affecting the brain, retina, and ears. 10 , 22 There are other medications, such as azathioprine, mycophenolate mofetil, cyclophosphamide, and rituximab, that are recommended for autoimmune diseases. 23 IV immunoglobulin is also the backbone of treatment when used simultaneously with corticosteroids.…”

“…The plasma exchange has also been implemented as an ancillary therapy in patients resistant to corticosteroids. 22 It can be used with other treatments such as corticosteroids and rituximab. 22 In one case report, a patient with incomplete SuS was treated with methylprednisolone, plasmapheresis, and rituximab, and sustained improvement occurred.…”