Autoimmune encephalitis with GABAA receptor antibodies in a 10-year-old girl

Recent studies have indicated that some cases of nonparaneoplastic autoimmune encephalitis in children can be caused by a systemic autoimmune disorder that generates autoantibodies to cell membrane proteins. We describe the clinical features of a 10-year-old girl with autoimmune encephalitis with autoantibodies against the GABAA receptor in whom type 1 diabetes mellitus developed during the course of the disease. The diagnosis was based on the progressive course of disease, pleocytosis in the cerebrospinal fluid (CSF), inflammatory changes in the brain and autoantibodies against the GABAA receptor detected in serum (absent in CSF). The treatment of encephalitis included intravenous immunoglobulins, intravenous methylprednisolone, oral prednisolone, cycles of plasmapheresis; this led to temporary remission. Finally, rituximab was applied as a second-line therapy with positive results.