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
