BROWN-VIALETTO-VANLAERE-SYNDROME - A CASE WITH ANTIGANGLIOSIDE GM1 ANTIBODIES

We report the case of a woman suffering from progressive bulbopontine paralysis in whose the first symptom, bilateral hypoacousia, began in childhood. This clinical picture is that of the Brown-Vialetto-Van Lae re (BVVL) syndrome. Anti-ganglioside GM1 antibodies were moderately el evated in this patient intravenous immunoglobulins produced little ben efit. The main clinical characteristics of 29 BVVL patients reported i n literature are reviewed, and the pathological significance of anti-G M1 antibodies is discussed in the context of this disorder.