Rituximab improved the clinical condition, but anti-GAD was reduced considerably after one year of treatment (400 U/ml). Previous reports suggested a beneficial response to immunosuppressive therapy in patients with severe disease unresponsive to benzodiazepines and/or baclofen. Lymphocyte B depletion may be beneficial in refractory forms of the disease with intensive antibody production. In this case, also, respiratory failure responded well to rituximab. Here, GAD1 is linked to respiratory failure.