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April 2017

Antibody Prevalence in Epilepsy (APE) Score—Evolution in Autoimmune Epilepsy Practice

Author Affiliations
  • 1Departments of Laboratory Medicine and Pathology and Neurology, Mayo Clinic, Rochester, Minnesota
JAMA Neurol. 2017;74(4):384-385. doi:10.1001/jamaneurol.2016.5479

Since 2004, neural antigen-specific autoimmune central nervous system (CNS) disorders characterized by seizures and other symptoms have been recognized.1-3 CNS autoimmunity was initially conceptualized in the context of global cerebral dysfunction (encephalopathy), but it is now clear that some patients with CNS autoimmunity present with focal seizures alone or with seizures as the most prominent clinical manifestation.4-6 Autoimmune epilepsy is important to diagnose because affected patients usually have seizures that are refractory to standard antiepileptic therapy but are often responsive to immune therapies (1 or more of corticosteroids, intravenous immune globulin, plasma exchange, or immune suppressants).7 In some patients, the trigger for CNS autoimmunity is occult cancer (paraneoplastic), but in most patients, the cause is unknown.8 The majority of adult-onset epilepsies are of focal origin (localization-related), about half of which are of unknown cause, yet autoimmune diagnoses and immune therapy responses are comparatively rare.9 Thus, factors predictive of an autoimmune diagnosis could help in the selection of patients who are most appropriate for neural antibody testing and trials of treatment.