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Semin Neurol 2018; 38(03): 303-315
DOI: 10.1055/s-0038-1660480
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Autoimmune Dementia

Justin M. Long
1   Department of Neurology, Washington University School of Medicine, St. Louis, Missouri
,
Gregory S. Day
1   Department of Neurology, Washington University School of Medicine, St. Louis, Missouri
2   Charles F. and Joanne Knight Alzheimer Disease Research Center, Washington University School of Medicine, St. Louis, Missouri
› Author Affiliations
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Publication History

Publication Date:
16 July 2018 (online)

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Abstract

Dementia refers to an acquired syndrome of intraindividual cognitive decline that ultimately interferes with an individual's ability to manage their usual duties at work or home. As experience with the diagnosis and management of patients with autoimmune and paraneoplastic encephalitis (AE) has expanded, it has become increasingly apparent that dementia may arise as a subacute or chronic complication of immune-mediated injury to the central nervous system. Progressive memory and thinking problems may represent the first (or only) sign of an underlying autoimmune or paraneoplastic disease. Accordingly, there is a need to routinely consider the diagnosis of AE in patients with dementia, and to evaluate patients recovering from AE for clinically meaningful cognitive impairment. We review and summarize the available evidence concerning the diagnosis and care of AE patients with associated cognitive impairment, herein referred to as autoimmune dementia (AiD). Relevant information is used to propose a novel diagnostic framework that may be applied to improve recognition, and facilitate the expedited evaluation and treatment of patients with AiD.

Keywords

autoimmune encephalitis - encephalopathy - dementia - neurodegenerative illness
 

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