We aimed to describe unique clinical and diagnostic features of various autoimmune BE disorders based on prior literature.

Brainstem encephalitis (BE) is a rare, severe, and rapidly progressive inflammation of the brainstem, associated with infectious and autoimmune causes. It presents with cranial neuropathies and ataxia.

We used relevant keywords to review the literature for BE, focusing on conditions including MS, NMOSD, BBE, CLIPPERS, GFAP Astrocytopathy, ADEM, paraneoplastic syndromes, and Behçet disease. Clinical pathology and diagnostic features were summarized.

Etiology: A significant proportion of BE is due to autoimmune causes, in contrast to predominantly infectious causes of cerebral encephalitis.

Bickerstaff Brainstem Encephalitis (BBE) refers to Post-infectious demyelination associated with anti-GQ1b antibodies, affecting brainstem and cranial nerves.

Paraneoplastic Disease: Brainstem encephalitis due to tumors is linked to antibodies like Anti-Hu, Ma2, Ri, KLHL11, and LUZP4. Presents with cranial neuropathies, gait ataxia, and dysarthria.

CLIPPERS shows punctate brainstem lesions responsive to steroids.

GFAP Astrocytopathy presents with brainstem inflammation, confirmed by GFAP-IgG antibodies in CSF.

MOG Antibody-Associated Disease (MOGAD) has MOG-IgG antibodies that target myelin oligodendrocyte glycoprotein, leading to demyelination. Clinical findings include optic neuritis and brainstem involvement.

ADEM affects children, causing diffuse brainstem symptoms after infection.

Susac syndrome features encephalopathy, retinal artery occlusions, and hearing loss, with MRI showing brainstem involvement.

Autoimmune BE involves distinct clinical features and antibodies across various conditions. Early diagnosis and prompt immunosuppressive treatment are key to improving outcomes. Further research is needed to enhance diagnostic accuracy and treatment efficacy.