Severe Bickerstaff's encephalitis treated with Rituximab: serum and CSF GQ1b antibodies

Bickerstaff's encephalitis is a syndrome of ophthalmoplegia, ataxia and impaired consciousness commonly associated with serum GQ1b antibodies. We describe a patient with seropositive Bickerstaff's encephalitis who did not respond either to plasma exchange or intravenous immunoglobulin but recovered following adjunct treatment with the anti-CD 20 monoclonal antibody, Rituximab. There was a concomitant reduction in serum GQ1b antibodies associated with improvement. Interestingly, GQ1b antibody was also detected in CSF. These findings have potentially significant clinical and immunopathological implications.