Autoimmune Encephalitis With Multiple Autoantibodies: A Diagnostic and Therapeutic Challenge

The Neurologist
Albert E KimGregory S Day

Abstract

Indications for autoantibody testing in patients with rapid-onset cognitive impairment have expanded in step with the growing number of disease-associated autoantibodies and clinical syndromes. Although increased access to autoantibody testing has broadened our understanding of the spectrum of autoimmune encephalitis (AE), it has also produced new challenges associated with deciphering the contributions of disease-associated autoantibodies in patients with atypical clinical features and/or multiple autoantibodies. These challenges are illustrated through presentation of a patient with AE associated with autoantibodies against intracellular and cell-surface neuronal antigens. The implications of detection of multiple autoantibodies are considered in the context of relevant literature, and used to frame a diagnostic and therapeutic approach. A previously well 67-year-old man presented with encephalopathy and psychosis, impaired visual fixation, and ataxia, emerging over 3 months. Hu, CRMP-5, and NMDAR autoantibodies were identified in the cerebrospinal fluid. No malignancy was discovered despite extensive investigations. An aggressive course of immunotherapy temporarily stabilized his course; however, the patient succumbed to his...Continue Reading

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Citations

Feb 12, 2019·Journal of Neurology·Margaret S BlattnerGregory S Day
May 30, 2019·International Journal of Molecular Sciences·Ritsuko Ohtani-Kaneko
Jun 12, 2020·Current Neurology and Neuroscience Reports·Margaret S Blattner, Gregory S Day
Jan 6, 2021·Annals of Clinical and Translational Neurology·Yaqing ShuXinhua Yu
Jun 17, 2021·Neurology. Neuroimmunology and Neuroinflammation·Justin R AbbatemarcoStacey L Clardy

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