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.
Highlights
Brainstem (BS) lesions have been linked to an extensive variety of pathologies such as infections, tumors, and autoimmune disorders [1]
Inflammatory BS lesions can be classified into two main categories, either primary inflammatory diseases of the central nervous system (CNS)—in the setting of what is known as autoimmune brainstem encephalitis (BSE)—or
magnetic resonance imaging (MRI) studies are recommended to rule out potential malignant evolution, since cases of early-stage CNS lymphoma presenting as CLIPPERS have been described [57]
Summary
Brainstem (BS) lesions have been linked to an extensive variety of pathologies such as infections, tumors, and autoimmune disorders [1]. Inflammatory BS lesions can be classified into two main categories, either primary inflammatory diseases of the central nervous system (CNS)—in the setting of what is known as autoimmune brainstem encephalitis (BSE)—or. CNS affection secondary to systemic diseases, where neurological symptoms are usually associated with other manifestations of the disease. In the latter case, CNS involvement usually occurs in the setting of an established systemic disease, and the diagnosis is usually clear-cut. In some cases, these manifestations may precede other known symptoms of the disease rendering the diagnosis more challenging. She denied any fall, bowel or urinary disturbances, recent infection, or fever. BSE with bellar exam, significant bilateral dysmetria wasofnoted on finger-to-nose testing. Severely ataxic, and the patient was unable to ambulate without assistance
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