Autoimmune encephalopathy

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

Autoimmune encephalopathy represents a complex category of disease with diverse immunologic associations, clinical manifestations, and therapeutic outcomes. Three main subgroups include autoimmune encephalopathies without cancer but with neural nonspecific serologic evidence of autoimmunity (encompassing Hashimoto's encephalopathy) that is the main focus of this review, paraneoplastic encephalopathies, and central nervous system (CNS) vasculitis (primary or secondary). Diagnosis of autoimmune encephalopathy can be suspected based on the clinical course, serologic evidence of autoimmunity, severe but nonspecific slowing on electroencephalography, and evidence of intrathecal inflammation in the cerebrospinal fluid. Rarely, there will be evidence of meningeal enhancement or increased fluid attenuated inversion-recovery signal in symptomatic regions on magnetic resonance imaging, but diagnosis may require brain biopsy demonstration of perivascular lymphocytic infiltrates. Nonspecific autoimmune encephalopathies are generally much more therapeutically responsive than paraneoplastic and vasculitic encephalopathies, so that high-dose corticosteroids may produce dramatic improvement within as little as a few days, although exceptional patients can require months of therapy. Paraneoplastic syndromes typically require tumor removal and often prove fatal. CNS vasculitides may respond to steroid therapy, but often require a second agent such as cyclophosphamide.

Original languageEnglish (US)
Pages (from-to)144-157
Number of pages14
JournalSeminars in Neurology
Volume31
Issue number2
DOIs
StatePublished - 2011

Fingerprint

Brain Diseases
Central Nervous System Vasculitis
Autoimmunity
Paraneoplastic Syndromes
Cyclophosphamide
Cerebrospinal Fluid
Electroencephalography
Neoplasms
Adrenal Cortex Hormones
Therapeutics
Steroids
Magnetic Resonance Imaging
Inflammation
Biopsy
Brain

Keywords

  • Autoimmune
  • dementia
  • encephalopathy
  • steroid-responsive
  • vasculitis

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

Autoimmune encephalopathy. / Flanagan, Eoin; Caselli, Richard John.

In: Seminars in Neurology, Vol. 31, No. 2, 2011, p. 144-157.

Research output: Contribution to journalArticle

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