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Semin Neurol 2013; 33(04): 357-364
DOI: 10.1055/s-0033-1359318
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Paraneoplastic and Autoimmune Encephalopathies

Orna O'Toole
1   Mayo Clinic Rochester Alumni, Rochester, Minnesota
2   Department of Neurology, Mercy University Hospital, Grenville Place, Cork, Ireland
,
Stacey Clardy
3   Department of Neuroimmunology, Mayo Clinic, Rochester, Minnesota
,
Amy May Lin Quek
1   Mayo Clinic Rochester Alumni, Rochester, Minnesota
4   Department of Medicine, National University Health System, Singapore
› Author Affiliations
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Publication History

Publication Date:
14 November 2013 (online)

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Abstract

Immune-mediated encephalitis is an increasingly recognized cause of neurologic dysfunction including behavioral change, psychosis, movement disorders, seizures, autonomic instability, and coma. Associated antineuronal antibodies are of two main subtypes, those targeting neuronal cell surface antigens, which are pathogenic, and nonpathogenic antibodies targeting intracellular antigens. Antibody identification aids in screening for underlying cancers and prediction of outcome. Cancer is found most commonly with antibodies targeting intracellular neural components. Certain cancers, such as small-cell lung carcinoma, and breast and ovarian cancer are particularly immunogenic. When cancer is detected, oncologic treatment should be followed with immunotherapy. Nonpathogenic antibody disorders respond poorly to treatment, whereas pathogenic antibodies predict a favorable response to immune treatment. If no cancer is identified, then ongoing surveillance is recommended for 5 years after detection of most antineuronal antibodies.

Keywords

encephalopathy - limbic encephalitis - autoimmune dementia - autoimmune encephalitis - paraneoplastic - onconeural