Autoimmune brainstem encephalitis (BSE) is a rare neurological condition with a wide range of underlying etiologies. It can be subdivided into two broad groups: a primary inflammatory disease of the central nervous system (CNS) or a brainstem disorder secondary to systemic diseases where the CNS is only one of many affected organs. Symptoms range from mild to life-threatening manifestations. Most cases respond well to immunotherapy. Therefore, broad and in-depth knowledge of the various inflammatory disorders that target the brainstem is essential for guiding the diagnostic approach and assisting in early initiation of appropriate therapy. We herein report on a case of BSE and provide an overview of the various causes of autoimmune BSE with an emphasis on the clinical manifestations and diagnostic approach.
Keywords: Bickerstaff brainstem encephalitis; CLIPPERS; acute disseminated encephalomyelitis; autoimmune glial fibrillary acidic protein astrocytopathy; brainstem encephalitis; connective tissue disease; multiple sclerosis; neuromyelitis optica spectrum disorder; paraneoplastic syndromes.