J Clin Neurol.  2016 Jan;12(1):1-13. 10.3988/jcn.2016.12.1.1.

The Diagnosis and Treatment of Autoimmune Encephalitis

• Lancaster

• KMID: 2364914
• DOI: http://doi.org/10.3988/jcn.2016.12.1.1

Abstract

Autoimmune encephalitis causes subacute deficits of memory and cognition, often followed by suppressed level of consciousness or coma. A careful history and examination may show early clues to particular autoimmune causes, such as neuromyotonia, hyperekplexia, psychosis, dystonia, or the presence of particular tumors. Ancillary testing with MRI and EEG may be helpful for excluding other causes, managing seizures, and, rarely, for identifying characteristic findings. Appropriate autoantibody testing can confirm specific diagnoses, although this is often done in parallel with exclusion of infectious and other causes. Autoimmune encephalitis may be divided into several groups of diseases: those with pathogenic antibodies to cell surface proteins, those with antibodies to intracellular synaptic proteins, T-cell diseases associated with antibodies to intracellular antigens, and those associated with other autoimmune disorders. Many forms of autoimmune encephalitis are paraneoplastic, and each of these conveys a distinct risk profile for various tumors. Tumor screening and, if necessary, treatment is essential to proper management. Most forms of autoimmune encephalitis respond to immune therapies, although powerful immune suppression for weeks or months may be needed in difficult cases. Autoimmune encephalitis may relapse, so follow-up care is important.

Keyword

autoimmune; antibody; paraneoplastic; encephalitis; anti-NMDAR encephalitis

MeSH Terms

Anti-N-Methyl-D-Aspartate Receptor Encephalitis
Antibodies
Cognition
Coma
Consciousness
Diagnosis*
Dystonia
Electroencephalography
Encephalitis*
Follow-Up Studies
Isaacs Syndrome
Magnetic Resonance Imaging
Mass Screening
Membrane Proteins
Memory
Psychotic Disorders
Recurrence
Seizures
Stiff-Person Syndrome
T-Lymphocytes
Antibodies
Membrane Proteins

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