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Epilepsia. 2017 Jul;58(7):1181-1189. doi: 10.1111/epi.13797. Epub 2017 May 26.

Predictive models in the diagnosis and treatment of autoimmune epilepsy.

Author information

1
Department of Neurology, Mayo Clinic, Rochester, Minnesota, U.S.A.
2
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, U.S.A.
3
Department of Immunology, Mayo Clinic, Rochester, Minnesota, U.S.A.
4
Department of Neurology, Mayo Clinic, Scottsdale, Arizona, U.S.A.
5
Department of Neurology, Mayo Clinic, Jacksonville, Florida, U.S.A.

Abstract

OBJECTIVE:

To validate predictive models for neural antibody positivity and immunotherapy response in epilepsy.

METHODS:

We conducted a retrospective study of epilepsy cases at Mayo Clinic (Rochester-MN; Scottsdale-AZ, and Jacksonville-FL) in whom autoimmune encephalopathy/epilepsy/dementia autoantibody testing profiles were requested (06/30/2014-06/30/2016). An Antibody Prevalence in Epilepsy (APE) score, based on clinical characteristics, was assigned to each patient. Among patients who received immunotherapy, a Response to Immunotherapy in Epilepsy (RITE) score was assigned. Favorable seizure outcome was defined as >50% reduction of seizure frequency at the first follow-up.

RESULTS:

Serum and cerebrospinal fluid (CSF) from 1,736 patients were sent to the Mayo Clinic Neuroimmunology Laboratory for neural autoantibody evaluation. Three hundred eighty-seven of these patients met the diagnostic criteria for epilepsy. Central nervous system (CNS)-specific antibodies were detected in 44 patients. Certain clinical features such as new-onset epilepsy, autonomic dysfunction, viral prodrome, faciobrachial dystonic seizures/oral dyskinesia, inflammatory CSF profile, and mesial temporal magnetic resonance imaging (MRI) abnormalities had a significant association with positive antibody results. A significantly higher proportion of antibody-positive patients had an APE score ≥4 (97.7% vs. 21.6%, p < 0.01). Sensitivity and specificity of an APE score ≥4 to predict presence of specific neural auto-antibody were 97.7% and 77.9%, respectively. In the subset of patients who received immunotherapy (77), autonomic dysfunction, faciobrachial dystonic seizures/oral dyskinesia, early initiation of immunotherapy, and presence of antibodies targeting plasma membrane proteins (cell-surface antigens) were associated with favorable seizure outcome. Sensitivity and specificity of a RITE score ≥7 to predict favorable seizure outcome were 87.5% and 83.8%, respectively.

SIGNIFICANCE:

APE and RITE scores can aid diagnosis, treatment, and prognostication of autoimmune epilepsy. A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.

KEYWORDS:

Autoimmune limbic encephalitis; Diagnosis; Epilepsy; Immunotherapy; Paraneoplastic limbic encephalitis; Predictive model

Comment in

PMID:
28555833
DOI:
10.1111/epi.13797
[Indexed for MEDLINE]
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