Semin Neurol 2019; 39(02): 283-292
DOI: 10.1055/s-0039-1678583
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Autoimmune Encephalitides and Rapidly Progressive Dementias

Sarah F. Wesley
1  Department of Neurology, Yale School of Medicine, New Haven, Connecticut
Damien Ferguson
2  Academic Unit of Neurology, University of Dublin, Trinity College, Dublin 2, Ireland
› Author Affiliations
Further Information

Publication History

Publication Date:
29 March 2019 (online)


Rapidly progressive dementia (RPD) or cognitive decline is a common presenting complaint in neurology. While primary dementia is often a concern, other forms of reversible dementia must be thoroughly considered. This article focuses on the growing field of autoimmune encephalitis (AE) as it pertains to the differential diagnostic considerations in a work-up for RPD. Understanding clues in the history and examination is the first step in identifying patients with a potential autoimmune cause for RPD. While testing for infectious and toxic-metabolic etiologies is commonly preformed, it is necessary to consider early ancillary testing for AE in appropriate cases of RPD. Autoantibody testing in the spinal fluid and serum, brain imaging, and electroencephalography all form the first line of investigations for AE. Treatment options and strategies depend on the AE subtype and a number of individual patient considerations.


Sarah F. Wesley is a former editorial staff member for Neurology Resident and Fellow Section. She is currently a guest reviewer for Neurology without compensation. She also serves as a guest reviewer for Clinical Therapeutics and has received compensation.

Damien Ferguson has received travel grants from Biogen Idec for attendance at international conferences. He is currently enrolled in a PhD program funded by the Health Research Board of Ireland.