Autoimmune maladies of the brainstem

There are numerous autoimmune diseases that present with brainstem involvement including multiple sclerosis, neuromyelitis optica spectrum disorders, myelin oligodendrocyte glycoprotein antibody-associated disease, acute disseminated encephalomyelitis, Bickerstaff brainstem encephalitis, chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), autoimmune encephalitis/encephalopathy, systemic lupus erythematosus, Sjögren's syndrome, neuro-sarcoidosis, and neuro-Behçet's disease. Symptoms of these diseases depend on the mechanisms, site and extent of the lesions. MRI images and autoantibodies in the blood are useful in diagnosing these diseases. Immunotherapy is the common treatment, though the response to treatment varies from disease to disease. In recent years, biologics have been applied for maintenance therapy.