Autoimmune Brainstem Encephalitis: An Illustrative Case and a Review of the Literature

Abstract

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

• brainstem encephalitis
• multiple sclerosis
• neuromyelitis optica spectrum disorder
• autoimmune glial fibrillary acidic protein astrocytopathy
• acute disseminated encephalomyelitis
• Bickerstaff brainstem encephalitis