Diagnosis and Management of Autoimmune Dementia

Elia Sechi, Eoin Flanagan

Research output: Contribution to journalReview article

Abstract

Purpose of review: To describe the clinical, laboratory, and MRI features that characterize cognitive decline in the setting of central nervous system (CNS) autoimmunity, and provide an overview of current treatment modalities. Recent findings: The field of autoimmune neurology is rapidly expanding due to the increasing number of newly discovered autoantibodies directed against specific CNS targets. The clinical syndromes associated with these autoantibodies are heterogeneous but frequently share common, recognizable clinical, and MRI characteristics. While the detection of certain autoantibodies strongly suggest the presence of an underlying malignancy (onconeural autoantibodies), a large proportion of cases remain idiopathic. Cognitive decline and encephalopathy are common manifestations of CNS autoimmunity, and can mimic neurodegenerative disorders. Recent findings suggest that the frequency of autoimmune encephalitis in the population is higher than previously thought, and potentially rivals that of infectious encephalitis. Moreover, emerging clinical scenarios that may predispose to CNS autoimmunity are increasingly been recognized. These include autoimmune dementia/encephalitis post-herpes simplex virus encephalitis, post-transplant and in association with immune checkpoint inhibitor treatment of cancer. Early recognition of autoimmune cognitive impairment is important given the potential for reversibility and disability prevention with appropriate treatment. Summary: Autoimmune cognitive impairment is treatable and may arise in a number of different clinical settings, with important treatment implications. Several clinical and para-clinical clues may help to differentiate these disorders from dementia of other etiologies.

Original languageEnglish (US)
Article number11
JournalCurrent Treatment Options in Neurology
Volume21
Issue number3
DOIs
StatePublished - Mar 1 2019

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Autoantibodies
Dementia
Central Nervous System
Autoimmunity
Herpes Simplex Encephalitis
Brain Diseases
Simplexvirus
Therapeutics
Neurology
Neurodegenerative Diseases
Neoplasms
Transplants
Cognitive Dysfunction
Population
Hashimoto's encephalitis

Keywords

  • Autoimmune cognitive impairment
  • Central nervous system autoimmunity
  • Immune check point inhibitors
  • Limbic encephalitis/encephalopathy
  • Neural autoantibodies

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Diagnosis and Management of Autoimmune Dementia. / Sechi, Elia; Flanagan, Eoin.

In: Current Treatment Options in Neurology, Vol. 21, No. 3, 11, 01.03.2019.

Research output: Contribution to journalReview article

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