Treatment of Susac syndrome with gamma globulin and corticosteroids

Anti-endothelial serum antibodies in a patient with Susac's syndrome

“…The finding of auto-antibodies to endothelium in our patient is particularly intriguing in the light of previous studies demonstrating vascular pathology in brain biopsies from patients with SS such as endothelial thickening and necrosis, deposition of complement components, and perivascular lymphocytic infiltration [3,4], and in the light of occasional reports on a beneficial effect of plasma exchange [8,9], IVIG [8][9][10], and of rituximab, a B cell depleting agent [11], in SS.…”

Susac syndrome: clinical characteristics and treatment in 29 new cases

“…There are no randomized trials on treatment efficacy to date. Anecdotal reports have suggested a therapeutic benefit from azathioprine, cyclophosphamide, mycophenolate mofetil, infliximab, nimodipine, antiplatelet agents, intravenous immunoglobulin (IVIg), and/or plasma exchange (PLEX) in some patients [5][6][7][8][9][10][11][12][13][14][15][16][17][18].…”

Brain histopathology in three cases of Susac's syndrome: implications for lesion pathogenesis and treatment: Figure 1

“…Microinfarctions were seen frequently, with one case showing luminal occlusion due to reactive and hypertrophied endothelial cells. Previous reports have shown that endothelial cell proliferation and basement membrane thickening are features of SS,3 4 and thickening of precapillary arteriolar adventitia and loss of internal elastica have also been observed 3–5…”

“…Sparse perivascular lymphocytic inflammation has been inconsistently identified in some case reports,2 4 6 but this lymphoid population has not been previously immunophenotyped. CD8+ T cells predominated in cases 1 and 2, while CD4 and CD8 T cells were found equally in case 3.…”